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This lecture covers the most common sources of facial pain, including trigeminal neuralgia, TMJ, Sjogren's, Eagles syndrome, glossopharyngeal neuralgia and other..This is based on a lecture on facial pain, given at University of Maryland School of Dental Surgery
Citation preview
Facial Pain from Various Sources-Diagnoses and Differential
Diagnoses
Course 4
Nelson Hendler MD MS
Former Assistant Professor of Neurosurgery
Johns Hopkins University School of Medicine
Former Associate Professor of Physiology
University of Maryland School of Dental Surgery
Past president-American Academy of Pain Management
wwwDiagnoseMyPaincom
Sensory Nerve Chart
for nerves which
cause pain to the
face Not only do
many of the 12 cranial
nerve (nerve which
come directly off of
the brain) have
painful conditions but
the nerve roots (nerve
which come off of the
spinal cord) also can
cause face pain The
C2-3 root may refer
pain to the angle of
the jaw or to an area
behind the eye The
occipital nerve may
give pain like a stripe
up the back of the
head
What is temporomandibular (TMJ) joint syndrome
bull Damage to the ligaments andor disc of the joint between the skull and the head of the jaw
bull The temporomandibular (TMJ) joint is a very unusual joint It is formed by the head of the condoyle of the jaw (mandible) inserting into a recess in the skull right beneath the temporal bone
bull The jaw bone is held to the skull by this joint and the ligaments that hold the jaw to the skull are the strong lateral temporomandibular ligaments and two weaker medial ligaments(Guralnick W Kaban L and Merrill R Temporomandibular joint afflictions New England Journal of Medicine Vol 299 No 3 pp 120-129 July 20 1978)
What is temporomandibular (TMJ) joint syndrome
bull The nerves that supply the joint are the auriculotemporal and massetric branches of the third division of the trigeminal nerve (Guralnick W Kaban L and Merrill R Temporomandibular joint afflictions New England Journal of Medicine Vol 299 No 3 pp 120-129 July 20 1978)
bull Irritation of these nerves produces pain in the jaw cheek andor temple
How do you diagnosis temporomandibular (TMJ) joint syndrome
bull Typical symptoms are bull facial pain in the cheek bull in the area of the joint bull in the jaw line bull ringing in the ear bull dizziness bull Pain in the temple and bull clicks in the jaw on opening or closing the jaw
(Gelb H TMJ Syndrome The tell-tale click Behavioral Medicine March 1978)
How do you diagnosis temporomandibular (TMJ) joint syndrome
bull TMJ is often diagnosed as something else such as Menierrersquossyndrome or trigeminal neuralgia (CommentaryThe Great Imposter Diseases of the Temporomandibular (TMJ) joint JAMA Vol 235 No 22 May 31 1976)
bull Sometimes MRIs with the jaws open and shut or even better cine MRIs (MRI movies) will reveal a displaced meniscus between the jaw and the skull
bull Injection of frac14 cc of 05 bupivicaine in the thesternocleidomastoid muscle may give temporary relief thereby confirming the diagnosis (Travell J Temporomandibular Joint Pain Referred from Muscles of the Head and Neck Journal of Prosthetic Dentistry Volume 10 No 4 1960 pp 745 ndash 763 and Travell JG Guest Editorial A Conjoint Attack on the
Causes of Craniomandibular Pain Journal of CraniomandibularPractice March ndash May 1984 2109 ndash 110)
How do you treat temporomandibular(TMJ) joint syndrome
bull Muscles relaxants and anti-inflammatory drugs such as iboprophen or naproxen are helpful (Guralnick W Kaban L and Merrill R Temporomandibular joint afflictions New England Journal of Medicine Vol 299 No 3 pp 120-129 July 20 1978)
bull The use of a Kil-Brux night guard may help which is a bite guard which fits over teeth on one side and with a smooth side on the other side so the opposing teeth can slide over the smooth surface
bull The dentist should never grind down the teeth which alters the bite and repositions the jaw
bull Only in rare instances is surgery recommended to repair the disc or ligaments and rarely does it improve the condition
Temporal Bone
JAW
BONE
SKULL
Normal TMJ
anatomy The
condyle is the part
of the jaw bone
which inserts into
the recess in the
skull Ligaments
hold the jaw bone
into the recess or
fossa (another name
for recess) The
fossa is an
indentation in the
skull bone which
accepts the condyle
of the jaw bone
Think of ligaments
like stretched rubber
bands holding the
condyle into the
fossa
The disc is like a
cushion and a shock
absorber The disc is
sort of like a water
filled balloon which
allows the condyle to
roll around in the
fossa while being
held in place by
ligaments The jaw
can slide forward or
back side to side or
pivot-a 3 way joint
In TMJ either the
disc or ligaments are
torn and this allow
the condyle to move
more than it should
Sometime the disc
moves too and gets
trapped between the
front of the fossa and
the condyle when
the jaw is opened
This causes the click
heard with TMJ
which is sort of like
snapping your
fingers
Condyle
Disc
Fossa
ligaments
What is trigeminal neuralgia bull There are two main etiologies to the pain of trigeminal
neuralgia bull In both types the pain can be constant or intermittent and
follows the path of the sensory branches of the trigeminal nerve into the forehead along the cheek bone or in the jaw line or any combination of the three (Empting L Hendler N Kolodny L Kraus H Tips on hard to manage pain syndromes Patient Care page 26-46 April 30 1990)
bull One etiology is a congenital compression of the trigeminal nerve by the middle cerebral artery in the skull This was well researched by Peter Janetta MD and his team at Uni of Pttisburgh
bull (Sekula RF Marchan EM Fletcher LH Casey KF Jannetta PJ Microvasculardecompression for trigeminal neuralgia in elderly patients J Neuro Neurosurg2008 Apr108(4)689-91)
SENSORY DISTRIBUTION
OF THE TRIGEMINAL
NERVE V IS FOR ROMAN
NUMERAL FIVE THERE
ARE THREE SENSORY
BRANCHES V1 or the
ophthalmic branch V2 or
the maxillary branch AND
V3 or the mandibular
branch Notice all the
sensory sensation is in the
jaw cheek and forehead
nose and eye
What is trigeminal neuralgia
bull A second form of trigeminal neuralgia is a herpetic-like viral irritation of the trigeminal nerve (Empting L Hendler N Kolodny L Kraus H Tips on hard to manage pain syndromes Patient Care page 26-46 April 30 1990)
bull Interestingly the pain of trigeminal neuralgia from both sources is clinically the same
bull This is an example of ldquoconvergencerdquo which is a single clinical manifestation due to several etiologies
How do you diagnose trigeminal neuralgia
bull The pain of both types of trigeminal neuralgia can be constant or intermittent and follows the path of the sensory branches of the trigeminal nerve into the forehead along the cheek bone or in the jaw line or any combination of the three (Empting L Hendler N Kolodny L Kraus H Tips on hard to manage pain syndromes Patient Care page 26-46 April 30 1990)
bull The pain is sensitive to light touch and may occur in burst of pain rather than being constant This occurs in both types of trigeminal neuralgia
How do you diagnose trigeminal neuralgia
bull In order to differentiate between the two forms of trigeminal neuralgia an MRI of the brain with vascular enhancement is useful
bull If there is no vessel compression it is highly likely the cause of the pain is post-herpetic
bull In addition to an MRI with vascular enhancement an MRI of the brain with thin-sections (2 mm slices) through the trigeminal nerve and an MRA (Magnet resonance angiography) may be helpful in differentiating the post-viral etiology from the compression of the nerve by the blood vessel
What is the treatment of trigeminal neuralgia
bull Since there are two main etiologies to the pain of trigeminal neuralgia there are two types of treatment
bull If the etiology is compression of the trigeminal nerve by the middle cerebral artery in the skull then the treatment is intra-cranial decompression of the nerve by inserting a sponge between the artery and the nerve (Sekula RF Marchan EM Fletcher LH Casey KF Jannetta PJ Microvascular decompression for trigeminal neuralgia in elderly patients J NeuroNeurosurg2008 Apr108(4)689-91)
What is the treatment of trigeminal neuralgia
bull If the etiology is due to a herpetic-like viral irritation of the trigeminal nerve (Empting L Hendler N Kolodny L Kraus H Tips on hard to manage pain syndromes Patient Care page 26-46 April 30 1990) then the treatment is the use of medications
bull Some people respond to low dose anti-depressants but the most useful medication is an anti-convulsant such as gaba-pentin (Neurontin) or Tegretol
bull Anti-viral drug such as acyclovir will also help
bull Rarely radio-frequency lesions of the retro-gausserian ganglion can be employed if all else fails
What is Eagles syndrome
bull Eagles Syndrome is characterized by an a specific orofacial pain secondary to calcification of the stylohyoid ligament or elongated styoid process In about 4 of general population an elongated styloid process occurs while only about 4 of these patients are symptomatic The stylo-hyoid bone is considered abnormal if it is greater than 25 mm in length (Casale M Rinaldi V Quattrocchi C Bressi F Vincenzi B Santini D Tonini G Salvinelli F Atypical chronic head and neck pain dont forget Eagles syndrome Eur Rev Med Pharmacol Sci 2008 Mar-Apr12(2)131-3)
3D-CT of a very long
stylo-hyoid
bonehellipmuch longer
than 25 mm Follow
the numbers 123
and 4 on both sides of
the 3D-CT and the is
the stylo-hyoid bone
which is about 39 mm
in this 3D-CT The
bone marked H is the
hyoid bone In this
3D-CT the stylo-hyoid
bone is so long that is
spontaneously broke
between 2 and 3 but it
is still symptomatic
and causes pain with
swollowing Removal
of the bone from the
break to 4 on the 3D-
CT will relieve
symptoms
What are the symptoms of Eagles syndrome and how do you diagnosis itbull The patient has a sensation of a foreign body localized at
the tonsillar fossa associated with a dull intermittent painbull Pain is usually worse with swallowingbull A bony projection is usually palpable with bimanual trans-
oral explorationbull Basically you put your finger in the mouth of the patient
down their throat and push towards the outside where the stylohyoid bone is If this reproduces the pain this confirms the diagnosis
bull A lateral radiograph and a computed tomography scan of head and neck will show an elongated styloid process of at least 25 mm (Casale M Rinaldi V Quattrocchi C Bressi F Vincenzi B Santini D Tonini G Salvinelli FAtypical chronic head and neck pain dont forget Eagles syndrome Eur Rev Med Pharmacol Sci 2008 Mar-Apr12(2)131-3)
How do you treat Eagles syndromebull Medical treatment represents the first choice
bull Use non-steroidal anti- inflammatory drugs
bull If this is not effective then surgical resection of the styloid process may help (cutting off the tip)
bull In the case of persistence or ingravescence of the complaint surgery may be the only option
bull Symptoms may overlap with glossopharyngealneuritis (presented later)
bull (Casale M Rinaldi V Quattrocchi C Bressi F Vincenzi B Santini D Tonini G
Salvinelli F Atypical chronic head and neck pain dont forget Eagles syndrome EurRev Med Pharmacol Sci 2008 Mar-Apr12(2)131-3)
What is Sjogrenrsquos Syndrome bull Sjogren syndrome is an autoimmune disorder in
which the glands that produce tears and saliva are destroyed causing dry mouth and dry eyes
bull Symptoms may include itching eyes feeling that something is in the eye difficulty swallowing loss of sense of taste thick saliva mouth sores many cavities in the teeth and pain in the face usually in the trigeminal or facial nerve
bull (Kruszka P OBrian RJ Diagnosis and management of Sjogren syndrome Am Fam Physician 200979465-470)
What are symptoms of Sjogrenrsquos Syndromebull Can be mistaken for trigeminal neuralgia
bull When there is unexplained pain in the face take a careful history Ask about dry mouth itchy eyes lots of cavities trouble swallowing SICCA complex (1)
bull Trigeminal sensory neuropathy can occur with Sjogrenrsquos and may be characterized by progressive sensory complaints on the face They could start on one side and subsequently become bilateral (2)
bull (1) Wu AJ Optimizing dry mouth treatment for individuals with Sjoumlgrens syndrome Rheum Dis Clin North Am 2008 Nov34(4)1001-1010
bull (2) Steven Mandel MD Clinical Professor of Neurology Jefferson Medical College)
Laboratory Tests to Diagnose Sjogrenrsquos
bull SSA and SSB ANA sed rate rheumatoid factor (RF) Schirmer test corneal fluorescein staining conjunctival lissamine green staining tear-film breakup time and lip biopsy are useful methods of diagnosing Sjogrenrsquos but not always reliable
bull Yang Z Liang Y Zhong R Is identification of anti-SSA andor -SSB antibodies necessary in serum samples referred for antinuclear antibodies testing J Clin Lab Anal 2012 Nov26(6)447-51 Br J Ophthalmol 2012 Dec96(12)1498-503 doi with aqueous-deficient dry eye
bull Liew MS Zhang M Kim E Akpek EK Prevalence and predictors of Sjogrens syndrome in a prospective cohort of patients with aqueous-deficient dry eye Br J Ophthalmol 2012 Dec96(12)1498-503
Treatment of Sjogrenrsquosbull Conventional immunosuppressive therapies
such as corticosteroids have been used in some patients with variable benefit
bull Current therapy for dryness is principally symptomatic
bull The greatest experience in pSS is with rituximab an anti-CD20 (expressed on a subset of B-cells) monoclonal antibody
(Bowman S Barone F Biologic treatments in Sjoumlgrens syndrome Presse Med 2012 Sep41(9 Pt 2)e495-509)
Symptoms of Glossopharyngeal Neuritis
bull Glossopharyngeal neuralgia is classically described as ldquostabbingrdquo or ldquoelectrical shock-likerdquo in nature Episodes are typically brief lasting a couple of seconds up to a few minutes and can be quite debilitating They can occur in the tongue throat ear and tonsils on one side of the head but in rare cases may be bilateral Painful attacks may occur spontaneously but are more often associated with a specific stimulus such as chewing speaking coughing swallowing or laughing The attacks can occur frequently throughout the day or once every couple of weeks and they can also disturb sleep
bull httpwwwcolumbianeurosurgeryorgconditionsglossopharyngeal-neuralgia
Causes of Glossopharyngeal Neuralgiabull Neuralgia is typically caused by compression of the
glossopharyngeal nerve
bull Compression of the nerve by an adjacent artery andor vein can occur near the brainstem
bull Compression of the nerve more distally in the neck by an elongated styloid process (a bone in the neck) can cause this pain syndrome as well and this is called Eagle syndrome
bull Glossopharyngeal neuralgia can be caused by a tumor or infection in the region of ninth cranial nerve or by multiple sclerosis
bull httpwwwcolumbianeurosurgeryorgconditionsglossopharyngeal-neuralgia
Differential diagnosis of Glossopharyngeal Neuritis (IX)
bull The diagnosis is made on clinical grounds alone
bull no specific test can be done to prove the diagnosis in any given patient Episodic ear andor throat pain often triggered by touching the palate or tonsil is strongly suggestive of the disorder
bull High resolution MRI or CT imaging of the brainstem may reveal vascular compression tumors or demyelinating lesions involving the ninth cranial nerve
bull High resolution CT scanning of the neck can reveal an elongated styloid process suggestive of Eagle syndrome
bull Pain that distinctly involves the ear rear 13 of the tongue andor throat is the classic pain distribution seen in glossopharyngeal neuralgia (ninth cranial nerve or IX N)
httpwwwcolumbianeurosurgeryorgconditionsglossopharyngeal-neuralgia
Treatment of Glossopharyngeal Neuritisbull Most treatment for Glossopharyngeal Neuralgia is
pharmacological Anticonvulsant medications including oxcarbazepine carbamazepine gabapentin and pregabalin Some antidepressants can also be helpful either alone or in combination with the seizure medications If medications fail surgical intervention is indicated
bull The first-choice treatment microvascular decompression (MVD) as it has the highest initial and long-term success rates Patients unable to tolerate such an operation may consider one of the many less invasive options including Gamma Knife radiosurgery
bull Eagles syndrome TMJ and Trigeminal neuralgia can co-exist with glossopharyngeal neuritis and mask the diagnosis or be mistaken for it
httpwwwcolumbianeurosurgeryorgconditionsglossopharyngeal-neuralgia
Sensory Nerve Chart
for nerves which
cause pain to the
face Not only do
many of the 12 cranial
nerve (nerve which
come directly off of
the brain) have
painful conditions but
the nerve roots (nerve
which come off of the
spinal cord) also can
cause face pain The
C2-3 root may refer
pain to the angle of
the jaw or to an area
behind the eye The
occipital nerve may
give pain like a stripe
up the back of the
head
What is temporomandibular (TMJ) joint syndrome
bull Damage to the ligaments andor disc of the joint between the skull and the head of the jaw
bull The temporomandibular (TMJ) joint is a very unusual joint It is formed by the head of the condoyle of the jaw (mandible) inserting into a recess in the skull right beneath the temporal bone
bull The jaw bone is held to the skull by this joint and the ligaments that hold the jaw to the skull are the strong lateral temporomandibular ligaments and two weaker medial ligaments(Guralnick W Kaban L and Merrill R Temporomandibular joint afflictions New England Journal of Medicine Vol 299 No 3 pp 120-129 July 20 1978)
What is temporomandibular (TMJ) joint syndrome
bull The nerves that supply the joint are the auriculotemporal and massetric branches of the third division of the trigeminal nerve (Guralnick W Kaban L and Merrill R Temporomandibular joint afflictions New England Journal of Medicine Vol 299 No 3 pp 120-129 July 20 1978)
bull Irritation of these nerves produces pain in the jaw cheek andor temple
How do you diagnosis temporomandibular (TMJ) joint syndrome
bull Typical symptoms are bull facial pain in the cheek bull in the area of the joint bull in the jaw line bull ringing in the ear bull dizziness bull Pain in the temple and bull clicks in the jaw on opening or closing the jaw
(Gelb H TMJ Syndrome The tell-tale click Behavioral Medicine March 1978)
How do you diagnosis temporomandibular (TMJ) joint syndrome
bull TMJ is often diagnosed as something else such as Menierrersquossyndrome or trigeminal neuralgia (CommentaryThe Great Imposter Diseases of the Temporomandibular (TMJ) joint JAMA Vol 235 No 22 May 31 1976)
bull Sometimes MRIs with the jaws open and shut or even better cine MRIs (MRI movies) will reveal a displaced meniscus between the jaw and the skull
bull Injection of frac14 cc of 05 bupivicaine in the thesternocleidomastoid muscle may give temporary relief thereby confirming the diagnosis (Travell J Temporomandibular Joint Pain Referred from Muscles of the Head and Neck Journal of Prosthetic Dentistry Volume 10 No 4 1960 pp 745 ndash 763 and Travell JG Guest Editorial A Conjoint Attack on the
Causes of Craniomandibular Pain Journal of CraniomandibularPractice March ndash May 1984 2109 ndash 110)
How do you treat temporomandibular(TMJ) joint syndrome
bull Muscles relaxants and anti-inflammatory drugs such as iboprophen or naproxen are helpful (Guralnick W Kaban L and Merrill R Temporomandibular joint afflictions New England Journal of Medicine Vol 299 No 3 pp 120-129 July 20 1978)
bull The use of a Kil-Brux night guard may help which is a bite guard which fits over teeth on one side and with a smooth side on the other side so the opposing teeth can slide over the smooth surface
bull The dentist should never grind down the teeth which alters the bite and repositions the jaw
bull Only in rare instances is surgery recommended to repair the disc or ligaments and rarely does it improve the condition
Temporal Bone
JAW
BONE
SKULL
Normal TMJ
anatomy The
condyle is the part
of the jaw bone
which inserts into
the recess in the
skull Ligaments
hold the jaw bone
into the recess or
fossa (another name
for recess) The
fossa is an
indentation in the
skull bone which
accepts the condyle
of the jaw bone
Think of ligaments
like stretched rubber
bands holding the
condyle into the
fossa
The disc is like a
cushion and a shock
absorber The disc is
sort of like a water
filled balloon which
allows the condyle to
roll around in the
fossa while being
held in place by
ligaments The jaw
can slide forward or
back side to side or
pivot-a 3 way joint
In TMJ either the
disc or ligaments are
torn and this allow
the condyle to move
more than it should
Sometime the disc
moves too and gets
trapped between the
front of the fossa and
the condyle when
the jaw is opened
This causes the click
heard with TMJ
which is sort of like
snapping your
fingers
Condyle
Disc
Fossa
ligaments
What is trigeminal neuralgia bull There are two main etiologies to the pain of trigeminal
neuralgia bull In both types the pain can be constant or intermittent and
follows the path of the sensory branches of the trigeminal nerve into the forehead along the cheek bone or in the jaw line or any combination of the three (Empting L Hendler N Kolodny L Kraus H Tips on hard to manage pain syndromes Patient Care page 26-46 April 30 1990)
bull One etiology is a congenital compression of the trigeminal nerve by the middle cerebral artery in the skull This was well researched by Peter Janetta MD and his team at Uni of Pttisburgh
bull (Sekula RF Marchan EM Fletcher LH Casey KF Jannetta PJ Microvasculardecompression for trigeminal neuralgia in elderly patients J Neuro Neurosurg2008 Apr108(4)689-91)
SENSORY DISTRIBUTION
OF THE TRIGEMINAL
NERVE V IS FOR ROMAN
NUMERAL FIVE THERE
ARE THREE SENSORY
BRANCHES V1 or the
ophthalmic branch V2 or
the maxillary branch AND
V3 or the mandibular
branch Notice all the
sensory sensation is in the
jaw cheek and forehead
nose and eye
What is trigeminal neuralgia
bull A second form of trigeminal neuralgia is a herpetic-like viral irritation of the trigeminal nerve (Empting L Hendler N Kolodny L Kraus H Tips on hard to manage pain syndromes Patient Care page 26-46 April 30 1990)
bull Interestingly the pain of trigeminal neuralgia from both sources is clinically the same
bull This is an example of ldquoconvergencerdquo which is a single clinical manifestation due to several etiologies
How do you diagnose trigeminal neuralgia
bull The pain of both types of trigeminal neuralgia can be constant or intermittent and follows the path of the sensory branches of the trigeminal nerve into the forehead along the cheek bone or in the jaw line or any combination of the three (Empting L Hendler N Kolodny L Kraus H Tips on hard to manage pain syndromes Patient Care page 26-46 April 30 1990)
bull The pain is sensitive to light touch and may occur in burst of pain rather than being constant This occurs in both types of trigeminal neuralgia
How do you diagnose trigeminal neuralgia
bull In order to differentiate between the two forms of trigeminal neuralgia an MRI of the brain with vascular enhancement is useful
bull If there is no vessel compression it is highly likely the cause of the pain is post-herpetic
bull In addition to an MRI with vascular enhancement an MRI of the brain with thin-sections (2 mm slices) through the trigeminal nerve and an MRA (Magnet resonance angiography) may be helpful in differentiating the post-viral etiology from the compression of the nerve by the blood vessel
What is the treatment of trigeminal neuralgia
bull Since there are two main etiologies to the pain of trigeminal neuralgia there are two types of treatment
bull If the etiology is compression of the trigeminal nerve by the middle cerebral artery in the skull then the treatment is intra-cranial decompression of the nerve by inserting a sponge between the artery and the nerve (Sekula RF Marchan EM Fletcher LH Casey KF Jannetta PJ Microvascular decompression for trigeminal neuralgia in elderly patients J NeuroNeurosurg2008 Apr108(4)689-91)
What is the treatment of trigeminal neuralgia
bull If the etiology is due to a herpetic-like viral irritation of the trigeminal nerve (Empting L Hendler N Kolodny L Kraus H Tips on hard to manage pain syndromes Patient Care page 26-46 April 30 1990) then the treatment is the use of medications
bull Some people respond to low dose anti-depressants but the most useful medication is an anti-convulsant such as gaba-pentin (Neurontin) or Tegretol
bull Anti-viral drug such as acyclovir will also help
bull Rarely radio-frequency lesions of the retro-gausserian ganglion can be employed if all else fails
What is Eagles syndrome
bull Eagles Syndrome is characterized by an a specific orofacial pain secondary to calcification of the stylohyoid ligament or elongated styoid process In about 4 of general population an elongated styloid process occurs while only about 4 of these patients are symptomatic The stylo-hyoid bone is considered abnormal if it is greater than 25 mm in length (Casale M Rinaldi V Quattrocchi C Bressi F Vincenzi B Santini D Tonini G Salvinelli F Atypical chronic head and neck pain dont forget Eagles syndrome Eur Rev Med Pharmacol Sci 2008 Mar-Apr12(2)131-3)
3D-CT of a very long
stylo-hyoid
bonehellipmuch longer
than 25 mm Follow
the numbers 123
and 4 on both sides of
the 3D-CT and the is
the stylo-hyoid bone
which is about 39 mm
in this 3D-CT The
bone marked H is the
hyoid bone In this
3D-CT the stylo-hyoid
bone is so long that is
spontaneously broke
between 2 and 3 but it
is still symptomatic
and causes pain with
swollowing Removal
of the bone from the
break to 4 on the 3D-
CT will relieve
symptoms
What are the symptoms of Eagles syndrome and how do you diagnosis itbull The patient has a sensation of a foreign body localized at
the tonsillar fossa associated with a dull intermittent painbull Pain is usually worse with swallowingbull A bony projection is usually palpable with bimanual trans-
oral explorationbull Basically you put your finger in the mouth of the patient
down their throat and push towards the outside where the stylohyoid bone is If this reproduces the pain this confirms the diagnosis
bull A lateral radiograph and a computed tomography scan of head and neck will show an elongated styloid process of at least 25 mm (Casale M Rinaldi V Quattrocchi C Bressi F Vincenzi B Santini D Tonini G Salvinelli FAtypical chronic head and neck pain dont forget Eagles syndrome Eur Rev Med Pharmacol Sci 2008 Mar-Apr12(2)131-3)
How do you treat Eagles syndromebull Medical treatment represents the first choice
bull Use non-steroidal anti- inflammatory drugs
bull If this is not effective then surgical resection of the styloid process may help (cutting off the tip)
bull In the case of persistence or ingravescence of the complaint surgery may be the only option
bull Symptoms may overlap with glossopharyngealneuritis (presented later)
bull (Casale M Rinaldi V Quattrocchi C Bressi F Vincenzi B Santini D Tonini G
Salvinelli F Atypical chronic head and neck pain dont forget Eagles syndrome EurRev Med Pharmacol Sci 2008 Mar-Apr12(2)131-3)
What is Sjogrenrsquos Syndrome bull Sjogren syndrome is an autoimmune disorder in
which the glands that produce tears and saliva are destroyed causing dry mouth and dry eyes
bull Symptoms may include itching eyes feeling that something is in the eye difficulty swallowing loss of sense of taste thick saliva mouth sores many cavities in the teeth and pain in the face usually in the trigeminal or facial nerve
bull (Kruszka P OBrian RJ Diagnosis and management of Sjogren syndrome Am Fam Physician 200979465-470)
What are symptoms of Sjogrenrsquos Syndromebull Can be mistaken for trigeminal neuralgia
bull When there is unexplained pain in the face take a careful history Ask about dry mouth itchy eyes lots of cavities trouble swallowing SICCA complex (1)
bull Trigeminal sensory neuropathy can occur with Sjogrenrsquos and may be characterized by progressive sensory complaints on the face They could start on one side and subsequently become bilateral (2)
bull (1) Wu AJ Optimizing dry mouth treatment for individuals with Sjoumlgrens syndrome Rheum Dis Clin North Am 2008 Nov34(4)1001-1010
bull (2) Steven Mandel MD Clinical Professor of Neurology Jefferson Medical College)
Laboratory Tests to Diagnose Sjogrenrsquos
bull SSA and SSB ANA sed rate rheumatoid factor (RF) Schirmer test corneal fluorescein staining conjunctival lissamine green staining tear-film breakup time and lip biopsy are useful methods of diagnosing Sjogrenrsquos but not always reliable
bull Yang Z Liang Y Zhong R Is identification of anti-SSA andor -SSB antibodies necessary in serum samples referred for antinuclear antibodies testing J Clin Lab Anal 2012 Nov26(6)447-51 Br J Ophthalmol 2012 Dec96(12)1498-503 doi with aqueous-deficient dry eye
bull Liew MS Zhang M Kim E Akpek EK Prevalence and predictors of Sjogrens syndrome in a prospective cohort of patients with aqueous-deficient dry eye Br J Ophthalmol 2012 Dec96(12)1498-503
Treatment of Sjogrenrsquosbull Conventional immunosuppressive therapies
such as corticosteroids have been used in some patients with variable benefit
bull Current therapy for dryness is principally symptomatic
bull The greatest experience in pSS is with rituximab an anti-CD20 (expressed on a subset of B-cells) monoclonal antibody
(Bowman S Barone F Biologic treatments in Sjoumlgrens syndrome Presse Med 2012 Sep41(9 Pt 2)e495-509)
Symptoms of Glossopharyngeal Neuritis
bull Glossopharyngeal neuralgia is classically described as ldquostabbingrdquo or ldquoelectrical shock-likerdquo in nature Episodes are typically brief lasting a couple of seconds up to a few minutes and can be quite debilitating They can occur in the tongue throat ear and tonsils on one side of the head but in rare cases may be bilateral Painful attacks may occur spontaneously but are more often associated with a specific stimulus such as chewing speaking coughing swallowing or laughing The attacks can occur frequently throughout the day or once every couple of weeks and they can also disturb sleep
bull httpwwwcolumbianeurosurgeryorgconditionsglossopharyngeal-neuralgia
Causes of Glossopharyngeal Neuralgiabull Neuralgia is typically caused by compression of the
glossopharyngeal nerve
bull Compression of the nerve by an adjacent artery andor vein can occur near the brainstem
bull Compression of the nerve more distally in the neck by an elongated styloid process (a bone in the neck) can cause this pain syndrome as well and this is called Eagle syndrome
bull Glossopharyngeal neuralgia can be caused by a tumor or infection in the region of ninth cranial nerve or by multiple sclerosis
bull httpwwwcolumbianeurosurgeryorgconditionsglossopharyngeal-neuralgia
Differential diagnosis of Glossopharyngeal Neuritis (IX)
bull The diagnosis is made on clinical grounds alone
bull no specific test can be done to prove the diagnosis in any given patient Episodic ear andor throat pain often triggered by touching the palate or tonsil is strongly suggestive of the disorder
bull High resolution MRI or CT imaging of the brainstem may reveal vascular compression tumors or demyelinating lesions involving the ninth cranial nerve
bull High resolution CT scanning of the neck can reveal an elongated styloid process suggestive of Eagle syndrome
bull Pain that distinctly involves the ear rear 13 of the tongue andor throat is the classic pain distribution seen in glossopharyngeal neuralgia (ninth cranial nerve or IX N)
httpwwwcolumbianeurosurgeryorgconditionsglossopharyngeal-neuralgia
Treatment of Glossopharyngeal Neuritisbull Most treatment for Glossopharyngeal Neuralgia is
pharmacological Anticonvulsant medications including oxcarbazepine carbamazepine gabapentin and pregabalin Some antidepressants can also be helpful either alone or in combination with the seizure medications If medications fail surgical intervention is indicated
bull The first-choice treatment microvascular decompression (MVD) as it has the highest initial and long-term success rates Patients unable to tolerate such an operation may consider one of the many less invasive options including Gamma Knife radiosurgery
bull Eagles syndrome TMJ and Trigeminal neuralgia can co-exist with glossopharyngeal neuritis and mask the diagnosis or be mistaken for it
httpwwwcolumbianeurosurgeryorgconditionsglossopharyngeal-neuralgia
What is temporomandibular (TMJ) joint syndrome
bull Damage to the ligaments andor disc of the joint between the skull and the head of the jaw
bull The temporomandibular (TMJ) joint is a very unusual joint It is formed by the head of the condoyle of the jaw (mandible) inserting into a recess in the skull right beneath the temporal bone
bull The jaw bone is held to the skull by this joint and the ligaments that hold the jaw to the skull are the strong lateral temporomandibular ligaments and two weaker medial ligaments(Guralnick W Kaban L and Merrill R Temporomandibular joint afflictions New England Journal of Medicine Vol 299 No 3 pp 120-129 July 20 1978)
What is temporomandibular (TMJ) joint syndrome
bull The nerves that supply the joint are the auriculotemporal and massetric branches of the third division of the trigeminal nerve (Guralnick W Kaban L and Merrill R Temporomandibular joint afflictions New England Journal of Medicine Vol 299 No 3 pp 120-129 July 20 1978)
bull Irritation of these nerves produces pain in the jaw cheek andor temple
How do you diagnosis temporomandibular (TMJ) joint syndrome
bull Typical symptoms are bull facial pain in the cheek bull in the area of the joint bull in the jaw line bull ringing in the ear bull dizziness bull Pain in the temple and bull clicks in the jaw on opening or closing the jaw
(Gelb H TMJ Syndrome The tell-tale click Behavioral Medicine March 1978)
How do you diagnosis temporomandibular (TMJ) joint syndrome
bull TMJ is often diagnosed as something else such as Menierrersquossyndrome or trigeminal neuralgia (CommentaryThe Great Imposter Diseases of the Temporomandibular (TMJ) joint JAMA Vol 235 No 22 May 31 1976)
bull Sometimes MRIs with the jaws open and shut or even better cine MRIs (MRI movies) will reveal a displaced meniscus between the jaw and the skull
bull Injection of frac14 cc of 05 bupivicaine in the thesternocleidomastoid muscle may give temporary relief thereby confirming the diagnosis (Travell J Temporomandibular Joint Pain Referred from Muscles of the Head and Neck Journal of Prosthetic Dentistry Volume 10 No 4 1960 pp 745 ndash 763 and Travell JG Guest Editorial A Conjoint Attack on the
Causes of Craniomandibular Pain Journal of CraniomandibularPractice March ndash May 1984 2109 ndash 110)
How do you treat temporomandibular(TMJ) joint syndrome
bull Muscles relaxants and anti-inflammatory drugs such as iboprophen or naproxen are helpful (Guralnick W Kaban L and Merrill R Temporomandibular joint afflictions New England Journal of Medicine Vol 299 No 3 pp 120-129 July 20 1978)
bull The use of a Kil-Brux night guard may help which is a bite guard which fits over teeth on one side and with a smooth side on the other side so the opposing teeth can slide over the smooth surface
bull The dentist should never grind down the teeth which alters the bite and repositions the jaw
bull Only in rare instances is surgery recommended to repair the disc or ligaments and rarely does it improve the condition
Temporal Bone
JAW
BONE
SKULL
Normal TMJ
anatomy The
condyle is the part
of the jaw bone
which inserts into
the recess in the
skull Ligaments
hold the jaw bone
into the recess or
fossa (another name
for recess) The
fossa is an
indentation in the
skull bone which
accepts the condyle
of the jaw bone
Think of ligaments
like stretched rubber
bands holding the
condyle into the
fossa
The disc is like a
cushion and a shock
absorber The disc is
sort of like a water
filled balloon which
allows the condyle to
roll around in the
fossa while being
held in place by
ligaments The jaw
can slide forward or
back side to side or
pivot-a 3 way joint
In TMJ either the
disc or ligaments are
torn and this allow
the condyle to move
more than it should
Sometime the disc
moves too and gets
trapped between the
front of the fossa and
the condyle when
the jaw is opened
This causes the click
heard with TMJ
which is sort of like
snapping your
fingers
Condyle
Disc
Fossa
ligaments
What is trigeminal neuralgia bull There are two main etiologies to the pain of trigeminal
neuralgia bull In both types the pain can be constant or intermittent and
follows the path of the sensory branches of the trigeminal nerve into the forehead along the cheek bone or in the jaw line or any combination of the three (Empting L Hendler N Kolodny L Kraus H Tips on hard to manage pain syndromes Patient Care page 26-46 April 30 1990)
bull One etiology is a congenital compression of the trigeminal nerve by the middle cerebral artery in the skull This was well researched by Peter Janetta MD and his team at Uni of Pttisburgh
bull (Sekula RF Marchan EM Fletcher LH Casey KF Jannetta PJ Microvasculardecompression for trigeminal neuralgia in elderly patients J Neuro Neurosurg2008 Apr108(4)689-91)
SENSORY DISTRIBUTION
OF THE TRIGEMINAL
NERVE V IS FOR ROMAN
NUMERAL FIVE THERE
ARE THREE SENSORY
BRANCHES V1 or the
ophthalmic branch V2 or
the maxillary branch AND
V3 or the mandibular
branch Notice all the
sensory sensation is in the
jaw cheek and forehead
nose and eye
What is trigeminal neuralgia
bull A second form of trigeminal neuralgia is a herpetic-like viral irritation of the trigeminal nerve (Empting L Hendler N Kolodny L Kraus H Tips on hard to manage pain syndromes Patient Care page 26-46 April 30 1990)
bull Interestingly the pain of trigeminal neuralgia from both sources is clinically the same
bull This is an example of ldquoconvergencerdquo which is a single clinical manifestation due to several etiologies
How do you diagnose trigeminal neuralgia
bull The pain of both types of trigeminal neuralgia can be constant or intermittent and follows the path of the sensory branches of the trigeminal nerve into the forehead along the cheek bone or in the jaw line or any combination of the three (Empting L Hendler N Kolodny L Kraus H Tips on hard to manage pain syndromes Patient Care page 26-46 April 30 1990)
bull The pain is sensitive to light touch and may occur in burst of pain rather than being constant This occurs in both types of trigeminal neuralgia
How do you diagnose trigeminal neuralgia
bull In order to differentiate between the two forms of trigeminal neuralgia an MRI of the brain with vascular enhancement is useful
bull If there is no vessel compression it is highly likely the cause of the pain is post-herpetic
bull In addition to an MRI with vascular enhancement an MRI of the brain with thin-sections (2 mm slices) through the trigeminal nerve and an MRA (Magnet resonance angiography) may be helpful in differentiating the post-viral etiology from the compression of the nerve by the blood vessel
What is the treatment of trigeminal neuralgia
bull Since there are two main etiologies to the pain of trigeminal neuralgia there are two types of treatment
bull If the etiology is compression of the trigeminal nerve by the middle cerebral artery in the skull then the treatment is intra-cranial decompression of the nerve by inserting a sponge between the artery and the nerve (Sekula RF Marchan EM Fletcher LH Casey KF Jannetta PJ Microvascular decompression for trigeminal neuralgia in elderly patients J NeuroNeurosurg2008 Apr108(4)689-91)
What is the treatment of trigeminal neuralgia
bull If the etiology is due to a herpetic-like viral irritation of the trigeminal nerve (Empting L Hendler N Kolodny L Kraus H Tips on hard to manage pain syndromes Patient Care page 26-46 April 30 1990) then the treatment is the use of medications
bull Some people respond to low dose anti-depressants but the most useful medication is an anti-convulsant such as gaba-pentin (Neurontin) or Tegretol
bull Anti-viral drug such as acyclovir will also help
bull Rarely radio-frequency lesions of the retro-gausserian ganglion can be employed if all else fails
What is Eagles syndrome
bull Eagles Syndrome is characterized by an a specific orofacial pain secondary to calcification of the stylohyoid ligament or elongated styoid process In about 4 of general population an elongated styloid process occurs while only about 4 of these patients are symptomatic The stylo-hyoid bone is considered abnormal if it is greater than 25 mm in length (Casale M Rinaldi V Quattrocchi C Bressi F Vincenzi B Santini D Tonini G Salvinelli F Atypical chronic head and neck pain dont forget Eagles syndrome Eur Rev Med Pharmacol Sci 2008 Mar-Apr12(2)131-3)
3D-CT of a very long
stylo-hyoid
bonehellipmuch longer
than 25 mm Follow
the numbers 123
and 4 on both sides of
the 3D-CT and the is
the stylo-hyoid bone
which is about 39 mm
in this 3D-CT The
bone marked H is the
hyoid bone In this
3D-CT the stylo-hyoid
bone is so long that is
spontaneously broke
between 2 and 3 but it
is still symptomatic
and causes pain with
swollowing Removal
of the bone from the
break to 4 on the 3D-
CT will relieve
symptoms
What are the symptoms of Eagles syndrome and how do you diagnosis itbull The patient has a sensation of a foreign body localized at
the tonsillar fossa associated with a dull intermittent painbull Pain is usually worse with swallowingbull A bony projection is usually palpable with bimanual trans-
oral explorationbull Basically you put your finger in the mouth of the patient
down their throat and push towards the outside where the stylohyoid bone is If this reproduces the pain this confirms the diagnosis
bull A lateral radiograph and a computed tomography scan of head and neck will show an elongated styloid process of at least 25 mm (Casale M Rinaldi V Quattrocchi C Bressi F Vincenzi B Santini D Tonini G Salvinelli FAtypical chronic head and neck pain dont forget Eagles syndrome Eur Rev Med Pharmacol Sci 2008 Mar-Apr12(2)131-3)
How do you treat Eagles syndromebull Medical treatment represents the first choice
bull Use non-steroidal anti- inflammatory drugs
bull If this is not effective then surgical resection of the styloid process may help (cutting off the tip)
bull In the case of persistence or ingravescence of the complaint surgery may be the only option
bull Symptoms may overlap with glossopharyngealneuritis (presented later)
bull (Casale M Rinaldi V Quattrocchi C Bressi F Vincenzi B Santini D Tonini G
Salvinelli F Atypical chronic head and neck pain dont forget Eagles syndrome EurRev Med Pharmacol Sci 2008 Mar-Apr12(2)131-3)
What is Sjogrenrsquos Syndrome bull Sjogren syndrome is an autoimmune disorder in
which the glands that produce tears and saliva are destroyed causing dry mouth and dry eyes
bull Symptoms may include itching eyes feeling that something is in the eye difficulty swallowing loss of sense of taste thick saliva mouth sores many cavities in the teeth and pain in the face usually in the trigeminal or facial nerve
bull (Kruszka P OBrian RJ Diagnosis and management of Sjogren syndrome Am Fam Physician 200979465-470)
What are symptoms of Sjogrenrsquos Syndromebull Can be mistaken for trigeminal neuralgia
bull When there is unexplained pain in the face take a careful history Ask about dry mouth itchy eyes lots of cavities trouble swallowing SICCA complex (1)
bull Trigeminal sensory neuropathy can occur with Sjogrenrsquos and may be characterized by progressive sensory complaints on the face They could start on one side and subsequently become bilateral (2)
bull (1) Wu AJ Optimizing dry mouth treatment for individuals with Sjoumlgrens syndrome Rheum Dis Clin North Am 2008 Nov34(4)1001-1010
bull (2) Steven Mandel MD Clinical Professor of Neurology Jefferson Medical College)
Laboratory Tests to Diagnose Sjogrenrsquos
bull SSA and SSB ANA sed rate rheumatoid factor (RF) Schirmer test corneal fluorescein staining conjunctival lissamine green staining tear-film breakup time and lip biopsy are useful methods of diagnosing Sjogrenrsquos but not always reliable
bull Yang Z Liang Y Zhong R Is identification of anti-SSA andor -SSB antibodies necessary in serum samples referred for antinuclear antibodies testing J Clin Lab Anal 2012 Nov26(6)447-51 Br J Ophthalmol 2012 Dec96(12)1498-503 doi with aqueous-deficient dry eye
bull Liew MS Zhang M Kim E Akpek EK Prevalence and predictors of Sjogrens syndrome in a prospective cohort of patients with aqueous-deficient dry eye Br J Ophthalmol 2012 Dec96(12)1498-503
Treatment of Sjogrenrsquosbull Conventional immunosuppressive therapies
such as corticosteroids have been used in some patients with variable benefit
bull Current therapy for dryness is principally symptomatic
bull The greatest experience in pSS is with rituximab an anti-CD20 (expressed on a subset of B-cells) monoclonal antibody
(Bowman S Barone F Biologic treatments in Sjoumlgrens syndrome Presse Med 2012 Sep41(9 Pt 2)e495-509)
Symptoms of Glossopharyngeal Neuritis
bull Glossopharyngeal neuralgia is classically described as ldquostabbingrdquo or ldquoelectrical shock-likerdquo in nature Episodes are typically brief lasting a couple of seconds up to a few minutes and can be quite debilitating They can occur in the tongue throat ear and tonsils on one side of the head but in rare cases may be bilateral Painful attacks may occur spontaneously but are more often associated with a specific stimulus such as chewing speaking coughing swallowing or laughing The attacks can occur frequently throughout the day or once every couple of weeks and they can also disturb sleep
bull httpwwwcolumbianeurosurgeryorgconditionsglossopharyngeal-neuralgia
Causes of Glossopharyngeal Neuralgiabull Neuralgia is typically caused by compression of the
glossopharyngeal nerve
bull Compression of the nerve by an adjacent artery andor vein can occur near the brainstem
bull Compression of the nerve more distally in the neck by an elongated styloid process (a bone in the neck) can cause this pain syndrome as well and this is called Eagle syndrome
bull Glossopharyngeal neuralgia can be caused by a tumor or infection in the region of ninth cranial nerve or by multiple sclerosis
bull httpwwwcolumbianeurosurgeryorgconditionsglossopharyngeal-neuralgia
Differential diagnosis of Glossopharyngeal Neuritis (IX)
bull The diagnosis is made on clinical grounds alone
bull no specific test can be done to prove the diagnosis in any given patient Episodic ear andor throat pain often triggered by touching the palate or tonsil is strongly suggestive of the disorder
bull High resolution MRI or CT imaging of the brainstem may reveal vascular compression tumors or demyelinating lesions involving the ninth cranial nerve
bull High resolution CT scanning of the neck can reveal an elongated styloid process suggestive of Eagle syndrome
bull Pain that distinctly involves the ear rear 13 of the tongue andor throat is the classic pain distribution seen in glossopharyngeal neuralgia (ninth cranial nerve or IX N)
httpwwwcolumbianeurosurgeryorgconditionsglossopharyngeal-neuralgia
Treatment of Glossopharyngeal Neuritisbull Most treatment for Glossopharyngeal Neuralgia is
pharmacological Anticonvulsant medications including oxcarbazepine carbamazepine gabapentin and pregabalin Some antidepressants can also be helpful either alone or in combination with the seizure medications If medications fail surgical intervention is indicated
bull The first-choice treatment microvascular decompression (MVD) as it has the highest initial and long-term success rates Patients unable to tolerate such an operation may consider one of the many less invasive options including Gamma Knife radiosurgery
bull Eagles syndrome TMJ and Trigeminal neuralgia can co-exist with glossopharyngeal neuritis and mask the diagnosis or be mistaken for it
httpwwwcolumbianeurosurgeryorgconditionsglossopharyngeal-neuralgia
What is temporomandibular (TMJ) joint syndrome
bull The nerves that supply the joint are the auriculotemporal and massetric branches of the third division of the trigeminal nerve (Guralnick W Kaban L and Merrill R Temporomandibular joint afflictions New England Journal of Medicine Vol 299 No 3 pp 120-129 July 20 1978)
bull Irritation of these nerves produces pain in the jaw cheek andor temple
How do you diagnosis temporomandibular (TMJ) joint syndrome
bull Typical symptoms are bull facial pain in the cheek bull in the area of the joint bull in the jaw line bull ringing in the ear bull dizziness bull Pain in the temple and bull clicks in the jaw on opening or closing the jaw
(Gelb H TMJ Syndrome The tell-tale click Behavioral Medicine March 1978)
How do you diagnosis temporomandibular (TMJ) joint syndrome
bull TMJ is often diagnosed as something else such as Menierrersquossyndrome or trigeminal neuralgia (CommentaryThe Great Imposter Diseases of the Temporomandibular (TMJ) joint JAMA Vol 235 No 22 May 31 1976)
bull Sometimes MRIs with the jaws open and shut or even better cine MRIs (MRI movies) will reveal a displaced meniscus between the jaw and the skull
bull Injection of frac14 cc of 05 bupivicaine in the thesternocleidomastoid muscle may give temporary relief thereby confirming the diagnosis (Travell J Temporomandibular Joint Pain Referred from Muscles of the Head and Neck Journal of Prosthetic Dentistry Volume 10 No 4 1960 pp 745 ndash 763 and Travell JG Guest Editorial A Conjoint Attack on the
Causes of Craniomandibular Pain Journal of CraniomandibularPractice March ndash May 1984 2109 ndash 110)
How do you treat temporomandibular(TMJ) joint syndrome
bull Muscles relaxants and anti-inflammatory drugs such as iboprophen or naproxen are helpful (Guralnick W Kaban L and Merrill R Temporomandibular joint afflictions New England Journal of Medicine Vol 299 No 3 pp 120-129 July 20 1978)
bull The use of a Kil-Brux night guard may help which is a bite guard which fits over teeth on one side and with a smooth side on the other side so the opposing teeth can slide over the smooth surface
bull The dentist should never grind down the teeth which alters the bite and repositions the jaw
bull Only in rare instances is surgery recommended to repair the disc or ligaments and rarely does it improve the condition
Temporal Bone
JAW
BONE
SKULL
Normal TMJ
anatomy The
condyle is the part
of the jaw bone
which inserts into
the recess in the
skull Ligaments
hold the jaw bone
into the recess or
fossa (another name
for recess) The
fossa is an
indentation in the
skull bone which
accepts the condyle
of the jaw bone
Think of ligaments
like stretched rubber
bands holding the
condyle into the
fossa
The disc is like a
cushion and a shock
absorber The disc is
sort of like a water
filled balloon which
allows the condyle to
roll around in the
fossa while being
held in place by
ligaments The jaw
can slide forward or
back side to side or
pivot-a 3 way joint
In TMJ either the
disc or ligaments are
torn and this allow
the condyle to move
more than it should
Sometime the disc
moves too and gets
trapped between the
front of the fossa and
the condyle when
the jaw is opened
This causes the click
heard with TMJ
which is sort of like
snapping your
fingers
Condyle
Disc
Fossa
ligaments
What is trigeminal neuralgia bull There are two main etiologies to the pain of trigeminal
neuralgia bull In both types the pain can be constant or intermittent and
follows the path of the sensory branches of the trigeminal nerve into the forehead along the cheek bone or in the jaw line or any combination of the three (Empting L Hendler N Kolodny L Kraus H Tips on hard to manage pain syndromes Patient Care page 26-46 April 30 1990)
bull One etiology is a congenital compression of the trigeminal nerve by the middle cerebral artery in the skull This was well researched by Peter Janetta MD and his team at Uni of Pttisburgh
bull (Sekula RF Marchan EM Fletcher LH Casey KF Jannetta PJ Microvasculardecompression for trigeminal neuralgia in elderly patients J Neuro Neurosurg2008 Apr108(4)689-91)
SENSORY DISTRIBUTION
OF THE TRIGEMINAL
NERVE V IS FOR ROMAN
NUMERAL FIVE THERE
ARE THREE SENSORY
BRANCHES V1 or the
ophthalmic branch V2 or
the maxillary branch AND
V3 or the mandibular
branch Notice all the
sensory sensation is in the
jaw cheek and forehead
nose and eye
What is trigeminal neuralgia
bull A second form of trigeminal neuralgia is a herpetic-like viral irritation of the trigeminal nerve (Empting L Hendler N Kolodny L Kraus H Tips on hard to manage pain syndromes Patient Care page 26-46 April 30 1990)
bull Interestingly the pain of trigeminal neuralgia from both sources is clinically the same
bull This is an example of ldquoconvergencerdquo which is a single clinical manifestation due to several etiologies
How do you diagnose trigeminal neuralgia
bull The pain of both types of trigeminal neuralgia can be constant or intermittent and follows the path of the sensory branches of the trigeminal nerve into the forehead along the cheek bone or in the jaw line or any combination of the three (Empting L Hendler N Kolodny L Kraus H Tips on hard to manage pain syndromes Patient Care page 26-46 April 30 1990)
bull The pain is sensitive to light touch and may occur in burst of pain rather than being constant This occurs in both types of trigeminal neuralgia
How do you diagnose trigeminal neuralgia
bull In order to differentiate between the two forms of trigeminal neuralgia an MRI of the brain with vascular enhancement is useful
bull If there is no vessel compression it is highly likely the cause of the pain is post-herpetic
bull In addition to an MRI with vascular enhancement an MRI of the brain with thin-sections (2 mm slices) through the trigeminal nerve and an MRA (Magnet resonance angiography) may be helpful in differentiating the post-viral etiology from the compression of the nerve by the blood vessel
What is the treatment of trigeminal neuralgia
bull Since there are two main etiologies to the pain of trigeminal neuralgia there are two types of treatment
bull If the etiology is compression of the trigeminal nerve by the middle cerebral artery in the skull then the treatment is intra-cranial decompression of the nerve by inserting a sponge between the artery and the nerve (Sekula RF Marchan EM Fletcher LH Casey KF Jannetta PJ Microvascular decompression for trigeminal neuralgia in elderly patients J NeuroNeurosurg2008 Apr108(4)689-91)
What is the treatment of trigeminal neuralgia
bull If the etiology is due to a herpetic-like viral irritation of the trigeminal nerve (Empting L Hendler N Kolodny L Kraus H Tips on hard to manage pain syndromes Patient Care page 26-46 April 30 1990) then the treatment is the use of medications
bull Some people respond to low dose anti-depressants but the most useful medication is an anti-convulsant such as gaba-pentin (Neurontin) or Tegretol
bull Anti-viral drug such as acyclovir will also help
bull Rarely radio-frequency lesions of the retro-gausserian ganglion can be employed if all else fails
What is Eagles syndrome
bull Eagles Syndrome is characterized by an a specific orofacial pain secondary to calcification of the stylohyoid ligament or elongated styoid process In about 4 of general population an elongated styloid process occurs while only about 4 of these patients are symptomatic The stylo-hyoid bone is considered abnormal if it is greater than 25 mm in length (Casale M Rinaldi V Quattrocchi C Bressi F Vincenzi B Santini D Tonini G Salvinelli F Atypical chronic head and neck pain dont forget Eagles syndrome Eur Rev Med Pharmacol Sci 2008 Mar-Apr12(2)131-3)
3D-CT of a very long
stylo-hyoid
bonehellipmuch longer
than 25 mm Follow
the numbers 123
and 4 on both sides of
the 3D-CT and the is
the stylo-hyoid bone
which is about 39 mm
in this 3D-CT The
bone marked H is the
hyoid bone In this
3D-CT the stylo-hyoid
bone is so long that is
spontaneously broke
between 2 and 3 but it
is still symptomatic
and causes pain with
swollowing Removal
of the bone from the
break to 4 on the 3D-
CT will relieve
symptoms
What are the symptoms of Eagles syndrome and how do you diagnosis itbull The patient has a sensation of a foreign body localized at
the tonsillar fossa associated with a dull intermittent painbull Pain is usually worse with swallowingbull A bony projection is usually palpable with bimanual trans-
oral explorationbull Basically you put your finger in the mouth of the patient
down their throat and push towards the outside where the stylohyoid bone is If this reproduces the pain this confirms the diagnosis
bull A lateral radiograph and a computed tomography scan of head and neck will show an elongated styloid process of at least 25 mm (Casale M Rinaldi V Quattrocchi C Bressi F Vincenzi B Santini D Tonini G Salvinelli FAtypical chronic head and neck pain dont forget Eagles syndrome Eur Rev Med Pharmacol Sci 2008 Mar-Apr12(2)131-3)
How do you treat Eagles syndromebull Medical treatment represents the first choice
bull Use non-steroidal anti- inflammatory drugs
bull If this is not effective then surgical resection of the styloid process may help (cutting off the tip)
bull In the case of persistence or ingravescence of the complaint surgery may be the only option
bull Symptoms may overlap with glossopharyngealneuritis (presented later)
bull (Casale M Rinaldi V Quattrocchi C Bressi F Vincenzi B Santini D Tonini G
Salvinelli F Atypical chronic head and neck pain dont forget Eagles syndrome EurRev Med Pharmacol Sci 2008 Mar-Apr12(2)131-3)
What is Sjogrenrsquos Syndrome bull Sjogren syndrome is an autoimmune disorder in
which the glands that produce tears and saliva are destroyed causing dry mouth and dry eyes
bull Symptoms may include itching eyes feeling that something is in the eye difficulty swallowing loss of sense of taste thick saliva mouth sores many cavities in the teeth and pain in the face usually in the trigeminal or facial nerve
bull (Kruszka P OBrian RJ Diagnosis and management of Sjogren syndrome Am Fam Physician 200979465-470)
What are symptoms of Sjogrenrsquos Syndromebull Can be mistaken for trigeminal neuralgia
bull When there is unexplained pain in the face take a careful history Ask about dry mouth itchy eyes lots of cavities trouble swallowing SICCA complex (1)
bull Trigeminal sensory neuropathy can occur with Sjogrenrsquos and may be characterized by progressive sensory complaints on the face They could start on one side and subsequently become bilateral (2)
bull (1) Wu AJ Optimizing dry mouth treatment for individuals with Sjoumlgrens syndrome Rheum Dis Clin North Am 2008 Nov34(4)1001-1010
bull (2) Steven Mandel MD Clinical Professor of Neurology Jefferson Medical College)
Laboratory Tests to Diagnose Sjogrenrsquos
bull SSA and SSB ANA sed rate rheumatoid factor (RF) Schirmer test corneal fluorescein staining conjunctival lissamine green staining tear-film breakup time and lip biopsy are useful methods of diagnosing Sjogrenrsquos but not always reliable
bull Yang Z Liang Y Zhong R Is identification of anti-SSA andor -SSB antibodies necessary in serum samples referred for antinuclear antibodies testing J Clin Lab Anal 2012 Nov26(6)447-51 Br J Ophthalmol 2012 Dec96(12)1498-503 doi with aqueous-deficient dry eye
bull Liew MS Zhang M Kim E Akpek EK Prevalence and predictors of Sjogrens syndrome in a prospective cohort of patients with aqueous-deficient dry eye Br J Ophthalmol 2012 Dec96(12)1498-503
Treatment of Sjogrenrsquosbull Conventional immunosuppressive therapies
such as corticosteroids have been used in some patients with variable benefit
bull Current therapy for dryness is principally symptomatic
bull The greatest experience in pSS is with rituximab an anti-CD20 (expressed on a subset of B-cells) monoclonal antibody
(Bowman S Barone F Biologic treatments in Sjoumlgrens syndrome Presse Med 2012 Sep41(9 Pt 2)e495-509)
Symptoms of Glossopharyngeal Neuritis
bull Glossopharyngeal neuralgia is classically described as ldquostabbingrdquo or ldquoelectrical shock-likerdquo in nature Episodes are typically brief lasting a couple of seconds up to a few minutes and can be quite debilitating They can occur in the tongue throat ear and tonsils on one side of the head but in rare cases may be bilateral Painful attacks may occur spontaneously but are more often associated with a specific stimulus such as chewing speaking coughing swallowing or laughing The attacks can occur frequently throughout the day or once every couple of weeks and they can also disturb sleep
bull httpwwwcolumbianeurosurgeryorgconditionsglossopharyngeal-neuralgia
Causes of Glossopharyngeal Neuralgiabull Neuralgia is typically caused by compression of the
glossopharyngeal nerve
bull Compression of the nerve by an adjacent artery andor vein can occur near the brainstem
bull Compression of the nerve more distally in the neck by an elongated styloid process (a bone in the neck) can cause this pain syndrome as well and this is called Eagle syndrome
bull Glossopharyngeal neuralgia can be caused by a tumor or infection in the region of ninth cranial nerve or by multiple sclerosis
bull httpwwwcolumbianeurosurgeryorgconditionsglossopharyngeal-neuralgia
Differential diagnosis of Glossopharyngeal Neuritis (IX)
bull The diagnosis is made on clinical grounds alone
bull no specific test can be done to prove the diagnosis in any given patient Episodic ear andor throat pain often triggered by touching the palate or tonsil is strongly suggestive of the disorder
bull High resolution MRI or CT imaging of the brainstem may reveal vascular compression tumors or demyelinating lesions involving the ninth cranial nerve
bull High resolution CT scanning of the neck can reveal an elongated styloid process suggestive of Eagle syndrome
bull Pain that distinctly involves the ear rear 13 of the tongue andor throat is the classic pain distribution seen in glossopharyngeal neuralgia (ninth cranial nerve or IX N)
httpwwwcolumbianeurosurgeryorgconditionsglossopharyngeal-neuralgia
Treatment of Glossopharyngeal Neuritisbull Most treatment for Glossopharyngeal Neuralgia is
pharmacological Anticonvulsant medications including oxcarbazepine carbamazepine gabapentin and pregabalin Some antidepressants can also be helpful either alone or in combination with the seizure medications If medications fail surgical intervention is indicated
bull The first-choice treatment microvascular decompression (MVD) as it has the highest initial and long-term success rates Patients unable to tolerate such an operation may consider one of the many less invasive options including Gamma Knife radiosurgery
bull Eagles syndrome TMJ and Trigeminal neuralgia can co-exist with glossopharyngeal neuritis and mask the diagnosis or be mistaken for it
httpwwwcolumbianeurosurgeryorgconditionsglossopharyngeal-neuralgia
How do you diagnosis temporomandibular (TMJ) joint syndrome
bull Typical symptoms are bull facial pain in the cheek bull in the area of the joint bull in the jaw line bull ringing in the ear bull dizziness bull Pain in the temple and bull clicks in the jaw on opening or closing the jaw
(Gelb H TMJ Syndrome The tell-tale click Behavioral Medicine March 1978)
How do you diagnosis temporomandibular (TMJ) joint syndrome
bull TMJ is often diagnosed as something else such as Menierrersquossyndrome or trigeminal neuralgia (CommentaryThe Great Imposter Diseases of the Temporomandibular (TMJ) joint JAMA Vol 235 No 22 May 31 1976)
bull Sometimes MRIs with the jaws open and shut or even better cine MRIs (MRI movies) will reveal a displaced meniscus between the jaw and the skull
bull Injection of frac14 cc of 05 bupivicaine in the thesternocleidomastoid muscle may give temporary relief thereby confirming the diagnosis (Travell J Temporomandibular Joint Pain Referred from Muscles of the Head and Neck Journal of Prosthetic Dentistry Volume 10 No 4 1960 pp 745 ndash 763 and Travell JG Guest Editorial A Conjoint Attack on the
Causes of Craniomandibular Pain Journal of CraniomandibularPractice March ndash May 1984 2109 ndash 110)
How do you treat temporomandibular(TMJ) joint syndrome
bull Muscles relaxants and anti-inflammatory drugs such as iboprophen or naproxen are helpful (Guralnick W Kaban L and Merrill R Temporomandibular joint afflictions New England Journal of Medicine Vol 299 No 3 pp 120-129 July 20 1978)
bull The use of a Kil-Brux night guard may help which is a bite guard which fits over teeth on one side and with a smooth side on the other side so the opposing teeth can slide over the smooth surface
bull The dentist should never grind down the teeth which alters the bite and repositions the jaw
bull Only in rare instances is surgery recommended to repair the disc or ligaments and rarely does it improve the condition
Temporal Bone
JAW
BONE
SKULL
Normal TMJ
anatomy The
condyle is the part
of the jaw bone
which inserts into
the recess in the
skull Ligaments
hold the jaw bone
into the recess or
fossa (another name
for recess) The
fossa is an
indentation in the
skull bone which
accepts the condyle
of the jaw bone
Think of ligaments
like stretched rubber
bands holding the
condyle into the
fossa
The disc is like a
cushion and a shock
absorber The disc is
sort of like a water
filled balloon which
allows the condyle to
roll around in the
fossa while being
held in place by
ligaments The jaw
can slide forward or
back side to side or
pivot-a 3 way joint
In TMJ either the
disc or ligaments are
torn and this allow
the condyle to move
more than it should
Sometime the disc
moves too and gets
trapped between the
front of the fossa and
the condyle when
the jaw is opened
This causes the click
heard with TMJ
which is sort of like
snapping your
fingers
Condyle
Disc
Fossa
ligaments
What is trigeminal neuralgia bull There are two main etiologies to the pain of trigeminal
neuralgia bull In both types the pain can be constant or intermittent and
follows the path of the sensory branches of the trigeminal nerve into the forehead along the cheek bone or in the jaw line or any combination of the three (Empting L Hendler N Kolodny L Kraus H Tips on hard to manage pain syndromes Patient Care page 26-46 April 30 1990)
bull One etiology is a congenital compression of the trigeminal nerve by the middle cerebral artery in the skull This was well researched by Peter Janetta MD and his team at Uni of Pttisburgh
bull (Sekula RF Marchan EM Fletcher LH Casey KF Jannetta PJ Microvasculardecompression for trigeminal neuralgia in elderly patients J Neuro Neurosurg2008 Apr108(4)689-91)
SENSORY DISTRIBUTION
OF THE TRIGEMINAL
NERVE V IS FOR ROMAN
NUMERAL FIVE THERE
ARE THREE SENSORY
BRANCHES V1 or the
ophthalmic branch V2 or
the maxillary branch AND
V3 or the mandibular
branch Notice all the
sensory sensation is in the
jaw cheek and forehead
nose and eye
What is trigeminal neuralgia
bull A second form of trigeminal neuralgia is a herpetic-like viral irritation of the trigeminal nerve (Empting L Hendler N Kolodny L Kraus H Tips on hard to manage pain syndromes Patient Care page 26-46 April 30 1990)
bull Interestingly the pain of trigeminal neuralgia from both sources is clinically the same
bull This is an example of ldquoconvergencerdquo which is a single clinical manifestation due to several etiologies
How do you diagnose trigeminal neuralgia
bull The pain of both types of trigeminal neuralgia can be constant or intermittent and follows the path of the sensory branches of the trigeminal nerve into the forehead along the cheek bone or in the jaw line or any combination of the three (Empting L Hendler N Kolodny L Kraus H Tips on hard to manage pain syndromes Patient Care page 26-46 April 30 1990)
bull The pain is sensitive to light touch and may occur in burst of pain rather than being constant This occurs in both types of trigeminal neuralgia
How do you diagnose trigeminal neuralgia
bull In order to differentiate between the two forms of trigeminal neuralgia an MRI of the brain with vascular enhancement is useful
bull If there is no vessel compression it is highly likely the cause of the pain is post-herpetic
bull In addition to an MRI with vascular enhancement an MRI of the brain with thin-sections (2 mm slices) through the trigeminal nerve and an MRA (Magnet resonance angiography) may be helpful in differentiating the post-viral etiology from the compression of the nerve by the blood vessel
What is the treatment of trigeminal neuralgia
bull Since there are two main etiologies to the pain of trigeminal neuralgia there are two types of treatment
bull If the etiology is compression of the trigeminal nerve by the middle cerebral artery in the skull then the treatment is intra-cranial decompression of the nerve by inserting a sponge between the artery and the nerve (Sekula RF Marchan EM Fletcher LH Casey KF Jannetta PJ Microvascular decompression for trigeminal neuralgia in elderly patients J NeuroNeurosurg2008 Apr108(4)689-91)
What is the treatment of trigeminal neuralgia
bull If the etiology is due to a herpetic-like viral irritation of the trigeminal nerve (Empting L Hendler N Kolodny L Kraus H Tips on hard to manage pain syndromes Patient Care page 26-46 April 30 1990) then the treatment is the use of medications
bull Some people respond to low dose anti-depressants but the most useful medication is an anti-convulsant such as gaba-pentin (Neurontin) or Tegretol
bull Anti-viral drug such as acyclovir will also help
bull Rarely radio-frequency lesions of the retro-gausserian ganglion can be employed if all else fails
What is Eagles syndrome
bull Eagles Syndrome is characterized by an a specific orofacial pain secondary to calcification of the stylohyoid ligament or elongated styoid process In about 4 of general population an elongated styloid process occurs while only about 4 of these patients are symptomatic The stylo-hyoid bone is considered abnormal if it is greater than 25 mm in length (Casale M Rinaldi V Quattrocchi C Bressi F Vincenzi B Santini D Tonini G Salvinelli F Atypical chronic head and neck pain dont forget Eagles syndrome Eur Rev Med Pharmacol Sci 2008 Mar-Apr12(2)131-3)
3D-CT of a very long
stylo-hyoid
bonehellipmuch longer
than 25 mm Follow
the numbers 123
and 4 on both sides of
the 3D-CT and the is
the stylo-hyoid bone
which is about 39 mm
in this 3D-CT The
bone marked H is the
hyoid bone In this
3D-CT the stylo-hyoid
bone is so long that is
spontaneously broke
between 2 and 3 but it
is still symptomatic
and causes pain with
swollowing Removal
of the bone from the
break to 4 on the 3D-
CT will relieve
symptoms
What are the symptoms of Eagles syndrome and how do you diagnosis itbull The patient has a sensation of a foreign body localized at
the tonsillar fossa associated with a dull intermittent painbull Pain is usually worse with swallowingbull A bony projection is usually palpable with bimanual trans-
oral explorationbull Basically you put your finger in the mouth of the patient
down their throat and push towards the outside where the stylohyoid bone is If this reproduces the pain this confirms the diagnosis
bull A lateral radiograph and a computed tomography scan of head and neck will show an elongated styloid process of at least 25 mm (Casale M Rinaldi V Quattrocchi C Bressi F Vincenzi B Santini D Tonini G Salvinelli FAtypical chronic head and neck pain dont forget Eagles syndrome Eur Rev Med Pharmacol Sci 2008 Mar-Apr12(2)131-3)
How do you treat Eagles syndromebull Medical treatment represents the first choice
bull Use non-steroidal anti- inflammatory drugs
bull If this is not effective then surgical resection of the styloid process may help (cutting off the tip)
bull In the case of persistence or ingravescence of the complaint surgery may be the only option
bull Symptoms may overlap with glossopharyngealneuritis (presented later)
bull (Casale M Rinaldi V Quattrocchi C Bressi F Vincenzi B Santini D Tonini G
Salvinelli F Atypical chronic head and neck pain dont forget Eagles syndrome EurRev Med Pharmacol Sci 2008 Mar-Apr12(2)131-3)
What is Sjogrenrsquos Syndrome bull Sjogren syndrome is an autoimmune disorder in
which the glands that produce tears and saliva are destroyed causing dry mouth and dry eyes
bull Symptoms may include itching eyes feeling that something is in the eye difficulty swallowing loss of sense of taste thick saliva mouth sores many cavities in the teeth and pain in the face usually in the trigeminal or facial nerve
bull (Kruszka P OBrian RJ Diagnosis and management of Sjogren syndrome Am Fam Physician 200979465-470)
What are symptoms of Sjogrenrsquos Syndromebull Can be mistaken for trigeminal neuralgia
bull When there is unexplained pain in the face take a careful history Ask about dry mouth itchy eyes lots of cavities trouble swallowing SICCA complex (1)
bull Trigeminal sensory neuropathy can occur with Sjogrenrsquos and may be characterized by progressive sensory complaints on the face They could start on one side and subsequently become bilateral (2)
bull (1) Wu AJ Optimizing dry mouth treatment for individuals with Sjoumlgrens syndrome Rheum Dis Clin North Am 2008 Nov34(4)1001-1010
bull (2) Steven Mandel MD Clinical Professor of Neurology Jefferson Medical College)
Laboratory Tests to Diagnose Sjogrenrsquos
bull SSA and SSB ANA sed rate rheumatoid factor (RF) Schirmer test corneal fluorescein staining conjunctival lissamine green staining tear-film breakup time and lip biopsy are useful methods of diagnosing Sjogrenrsquos but not always reliable
bull Yang Z Liang Y Zhong R Is identification of anti-SSA andor -SSB antibodies necessary in serum samples referred for antinuclear antibodies testing J Clin Lab Anal 2012 Nov26(6)447-51 Br J Ophthalmol 2012 Dec96(12)1498-503 doi with aqueous-deficient dry eye
bull Liew MS Zhang M Kim E Akpek EK Prevalence and predictors of Sjogrens syndrome in a prospective cohort of patients with aqueous-deficient dry eye Br J Ophthalmol 2012 Dec96(12)1498-503
Treatment of Sjogrenrsquosbull Conventional immunosuppressive therapies
such as corticosteroids have been used in some patients with variable benefit
bull Current therapy for dryness is principally symptomatic
bull The greatest experience in pSS is with rituximab an anti-CD20 (expressed on a subset of B-cells) monoclonal antibody
(Bowman S Barone F Biologic treatments in Sjoumlgrens syndrome Presse Med 2012 Sep41(9 Pt 2)e495-509)
Symptoms of Glossopharyngeal Neuritis
bull Glossopharyngeal neuralgia is classically described as ldquostabbingrdquo or ldquoelectrical shock-likerdquo in nature Episodes are typically brief lasting a couple of seconds up to a few minutes and can be quite debilitating They can occur in the tongue throat ear and tonsils on one side of the head but in rare cases may be bilateral Painful attacks may occur spontaneously but are more often associated with a specific stimulus such as chewing speaking coughing swallowing or laughing The attacks can occur frequently throughout the day or once every couple of weeks and they can also disturb sleep
bull httpwwwcolumbianeurosurgeryorgconditionsglossopharyngeal-neuralgia
Causes of Glossopharyngeal Neuralgiabull Neuralgia is typically caused by compression of the
glossopharyngeal nerve
bull Compression of the nerve by an adjacent artery andor vein can occur near the brainstem
bull Compression of the nerve more distally in the neck by an elongated styloid process (a bone in the neck) can cause this pain syndrome as well and this is called Eagle syndrome
bull Glossopharyngeal neuralgia can be caused by a tumor or infection in the region of ninth cranial nerve or by multiple sclerosis
bull httpwwwcolumbianeurosurgeryorgconditionsglossopharyngeal-neuralgia
Differential diagnosis of Glossopharyngeal Neuritis (IX)
bull The diagnosis is made on clinical grounds alone
bull no specific test can be done to prove the diagnosis in any given patient Episodic ear andor throat pain often triggered by touching the palate or tonsil is strongly suggestive of the disorder
bull High resolution MRI or CT imaging of the brainstem may reveal vascular compression tumors or demyelinating lesions involving the ninth cranial nerve
bull High resolution CT scanning of the neck can reveal an elongated styloid process suggestive of Eagle syndrome
bull Pain that distinctly involves the ear rear 13 of the tongue andor throat is the classic pain distribution seen in glossopharyngeal neuralgia (ninth cranial nerve or IX N)
httpwwwcolumbianeurosurgeryorgconditionsglossopharyngeal-neuralgia
Treatment of Glossopharyngeal Neuritisbull Most treatment for Glossopharyngeal Neuralgia is
pharmacological Anticonvulsant medications including oxcarbazepine carbamazepine gabapentin and pregabalin Some antidepressants can also be helpful either alone or in combination with the seizure medications If medications fail surgical intervention is indicated
bull The first-choice treatment microvascular decompression (MVD) as it has the highest initial and long-term success rates Patients unable to tolerate such an operation may consider one of the many less invasive options including Gamma Knife radiosurgery
bull Eagles syndrome TMJ and Trigeminal neuralgia can co-exist with glossopharyngeal neuritis and mask the diagnosis or be mistaken for it
httpwwwcolumbianeurosurgeryorgconditionsglossopharyngeal-neuralgia
How do you diagnosis temporomandibular (TMJ) joint syndrome
bull TMJ is often diagnosed as something else such as Menierrersquossyndrome or trigeminal neuralgia (CommentaryThe Great Imposter Diseases of the Temporomandibular (TMJ) joint JAMA Vol 235 No 22 May 31 1976)
bull Sometimes MRIs with the jaws open and shut or even better cine MRIs (MRI movies) will reveal a displaced meniscus between the jaw and the skull
bull Injection of frac14 cc of 05 bupivicaine in the thesternocleidomastoid muscle may give temporary relief thereby confirming the diagnosis (Travell J Temporomandibular Joint Pain Referred from Muscles of the Head and Neck Journal of Prosthetic Dentistry Volume 10 No 4 1960 pp 745 ndash 763 and Travell JG Guest Editorial A Conjoint Attack on the
Causes of Craniomandibular Pain Journal of CraniomandibularPractice March ndash May 1984 2109 ndash 110)
How do you treat temporomandibular(TMJ) joint syndrome
bull Muscles relaxants and anti-inflammatory drugs such as iboprophen or naproxen are helpful (Guralnick W Kaban L and Merrill R Temporomandibular joint afflictions New England Journal of Medicine Vol 299 No 3 pp 120-129 July 20 1978)
bull The use of a Kil-Brux night guard may help which is a bite guard which fits over teeth on one side and with a smooth side on the other side so the opposing teeth can slide over the smooth surface
bull The dentist should never grind down the teeth which alters the bite and repositions the jaw
bull Only in rare instances is surgery recommended to repair the disc or ligaments and rarely does it improve the condition
Temporal Bone
JAW
BONE
SKULL
Normal TMJ
anatomy The
condyle is the part
of the jaw bone
which inserts into
the recess in the
skull Ligaments
hold the jaw bone
into the recess or
fossa (another name
for recess) The
fossa is an
indentation in the
skull bone which
accepts the condyle
of the jaw bone
Think of ligaments
like stretched rubber
bands holding the
condyle into the
fossa
The disc is like a
cushion and a shock
absorber The disc is
sort of like a water
filled balloon which
allows the condyle to
roll around in the
fossa while being
held in place by
ligaments The jaw
can slide forward or
back side to side or
pivot-a 3 way joint
In TMJ either the
disc or ligaments are
torn and this allow
the condyle to move
more than it should
Sometime the disc
moves too and gets
trapped between the
front of the fossa and
the condyle when
the jaw is opened
This causes the click
heard with TMJ
which is sort of like
snapping your
fingers
Condyle
Disc
Fossa
ligaments
What is trigeminal neuralgia bull There are two main etiologies to the pain of trigeminal
neuralgia bull In both types the pain can be constant or intermittent and
follows the path of the sensory branches of the trigeminal nerve into the forehead along the cheek bone or in the jaw line or any combination of the three (Empting L Hendler N Kolodny L Kraus H Tips on hard to manage pain syndromes Patient Care page 26-46 April 30 1990)
bull One etiology is a congenital compression of the trigeminal nerve by the middle cerebral artery in the skull This was well researched by Peter Janetta MD and his team at Uni of Pttisburgh
bull (Sekula RF Marchan EM Fletcher LH Casey KF Jannetta PJ Microvasculardecompression for trigeminal neuralgia in elderly patients J Neuro Neurosurg2008 Apr108(4)689-91)
SENSORY DISTRIBUTION
OF THE TRIGEMINAL
NERVE V IS FOR ROMAN
NUMERAL FIVE THERE
ARE THREE SENSORY
BRANCHES V1 or the
ophthalmic branch V2 or
the maxillary branch AND
V3 or the mandibular
branch Notice all the
sensory sensation is in the
jaw cheek and forehead
nose and eye
What is trigeminal neuralgia
bull A second form of trigeminal neuralgia is a herpetic-like viral irritation of the trigeminal nerve (Empting L Hendler N Kolodny L Kraus H Tips on hard to manage pain syndromes Patient Care page 26-46 April 30 1990)
bull Interestingly the pain of trigeminal neuralgia from both sources is clinically the same
bull This is an example of ldquoconvergencerdquo which is a single clinical manifestation due to several etiologies
How do you diagnose trigeminal neuralgia
bull The pain of both types of trigeminal neuralgia can be constant or intermittent and follows the path of the sensory branches of the trigeminal nerve into the forehead along the cheek bone or in the jaw line or any combination of the three (Empting L Hendler N Kolodny L Kraus H Tips on hard to manage pain syndromes Patient Care page 26-46 April 30 1990)
bull The pain is sensitive to light touch and may occur in burst of pain rather than being constant This occurs in both types of trigeminal neuralgia
How do you diagnose trigeminal neuralgia
bull In order to differentiate between the two forms of trigeminal neuralgia an MRI of the brain with vascular enhancement is useful
bull If there is no vessel compression it is highly likely the cause of the pain is post-herpetic
bull In addition to an MRI with vascular enhancement an MRI of the brain with thin-sections (2 mm slices) through the trigeminal nerve and an MRA (Magnet resonance angiography) may be helpful in differentiating the post-viral etiology from the compression of the nerve by the blood vessel
What is the treatment of trigeminal neuralgia
bull Since there are two main etiologies to the pain of trigeminal neuralgia there are two types of treatment
bull If the etiology is compression of the trigeminal nerve by the middle cerebral artery in the skull then the treatment is intra-cranial decompression of the nerve by inserting a sponge between the artery and the nerve (Sekula RF Marchan EM Fletcher LH Casey KF Jannetta PJ Microvascular decompression for trigeminal neuralgia in elderly patients J NeuroNeurosurg2008 Apr108(4)689-91)
What is the treatment of trigeminal neuralgia
bull If the etiology is due to a herpetic-like viral irritation of the trigeminal nerve (Empting L Hendler N Kolodny L Kraus H Tips on hard to manage pain syndromes Patient Care page 26-46 April 30 1990) then the treatment is the use of medications
bull Some people respond to low dose anti-depressants but the most useful medication is an anti-convulsant such as gaba-pentin (Neurontin) or Tegretol
bull Anti-viral drug such as acyclovir will also help
bull Rarely radio-frequency lesions of the retro-gausserian ganglion can be employed if all else fails
What is Eagles syndrome
bull Eagles Syndrome is characterized by an a specific orofacial pain secondary to calcification of the stylohyoid ligament or elongated styoid process In about 4 of general population an elongated styloid process occurs while only about 4 of these patients are symptomatic The stylo-hyoid bone is considered abnormal if it is greater than 25 mm in length (Casale M Rinaldi V Quattrocchi C Bressi F Vincenzi B Santini D Tonini G Salvinelli F Atypical chronic head and neck pain dont forget Eagles syndrome Eur Rev Med Pharmacol Sci 2008 Mar-Apr12(2)131-3)
3D-CT of a very long
stylo-hyoid
bonehellipmuch longer
than 25 mm Follow
the numbers 123
and 4 on both sides of
the 3D-CT and the is
the stylo-hyoid bone
which is about 39 mm
in this 3D-CT The
bone marked H is the
hyoid bone In this
3D-CT the stylo-hyoid
bone is so long that is
spontaneously broke
between 2 and 3 but it
is still symptomatic
and causes pain with
swollowing Removal
of the bone from the
break to 4 on the 3D-
CT will relieve
symptoms
What are the symptoms of Eagles syndrome and how do you diagnosis itbull The patient has a sensation of a foreign body localized at
the tonsillar fossa associated with a dull intermittent painbull Pain is usually worse with swallowingbull A bony projection is usually palpable with bimanual trans-
oral explorationbull Basically you put your finger in the mouth of the patient
down their throat and push towards the outside where the stylohyoid bone is If this reproduces the pain this confirms the diagnosis
bull A lateral radiograph and a computed tomography scan of head and neck will show an elongated styloid process of at least 25 mm (Casale M Rinaldi V Quattrocchi C Bressi F Vincenzi B Santini D Tonini G Salvinelli FAtypical chronic head and neck pain dont forget Eagles syndrome Eur Rev Med Pharmacol Sci 2008 Mar-Apr12(2)131-3)
How do you treat Eagles syndromebull Medical treatment represents the first choice
bull Use non-steroidal anti- inflammatory drugs
bull If this is not effective then surgical resection of the styloid process may help (cutting off the tip)
bull In the case of persistence or ingravescence of the complaint surgery may be the only option
bull Symptoms may overlap with glossopharyngealneuritis (presented later)
bull (Casale M Rinaldi V Quattrocchi C Bressi F Vincenzi B Santini D Tonini G
Salvinelli F Atypical chronic head and neck pain dont forget Eagles syndrome EurRev Med Pharmacol Sci 2008 Mar-Apr12(2)131-3)
What is Sjogrenrsquos Syndrome bull Sjogren syndrome is an autoimmune disorder in
which the glands that produce tears and saliva are destroyed causing dry mouth and dry eyes
bull Symptoms may include itching eyes feeling that something is in the eye difficulty swallowing loss of sense of taste thick saliva mouth sores many cavities in the teeth and pain in the face usually in the trigeminal or facial nerve
bull (Kruszka P OBrian RJ Diagnosis and management of Sjogren syndrome Am Fam Physician 200979465-470)
What are symptoms of Sjogrenrsquos Syndromebull Can be mistaken for trigeminal neuralgia
bull When there is unexplained pain in the face take a careful history Ask about dry mouth itchy eyes lots of cavities trouble swallowing SICCA complex (1)
bull Trigeminal sensory neuropathy can occur with Sjogrenrsquos and may be characterized by progressive sensory complaints on the face They could start on one side and subsequently become bilateral (2)
bull (1) Wu AJ Optimizing dry mouth treatment for individuals with Sjoumlgrens syndrome Rheum Dis Clin North Am 2008 Nov34(4)1001-1010
bull (2) Steven Mandel MD Clinical Professor of Neurology Jefferson Medical College)
Laboratory Tests to Diagnose Sjogrenrsquos
bull SSA and SSB ANA sed rate rheumatoid factor (RF) Schirmer test corneal fluorescein staining conjunctival lissamine green staining tear-film breakup time and lip biopsy are useful methods of diagnosing Sjogrenrsquos but not always reliable
bull Yang Z Liang Y Zhong R Is identification of anti-SSA andor -SSB antibodies necessary in serum samples referred for antinuclear antibodies testing J Clin Lab Anal 2012 Nov26(6)447-51 Br J Ophthalmol 2012 Dec96(12)1498-503 doi with aqueous-deficient dry eye
bull Liew MS Zhang M Kim E Akpek EK Prevalence and predictors of Sjogrens syndrome in a prospective cohort of patients with aqueous-deficient dry eye Br J Ophthalmol 2012 Dec96(12)1498-503
Treatment of Sjogrenrsquosbull Conventional immunosuppressive therapies
such as corticosteroids have been used in some patients with variable benefit
bull Current therapy for dryness is principally symptomatic
bull The greatest experience in pSS is with rituximab an anti-CD20 (expressed on a subset of B-cells) monoclonal antibody
(Bowman S Barone F Biologic treatments in Sjoumlgrens syndrome Presse Med 2012 Sep41(9 Pt 2)e495-509)
Symptoms of Glossopharyngeal Neuritis
bull Glossopharyngeal neuralgia is classically described as ldquostabbingrdquo or ldquoelectrical shock-likerdquo in nature Episodes are typically brief lasting a couple of seconds up to a few minutes and can be quite debilitating They can occur in the tongue throat ear and tonsils on one side of the head but in rare cases may be bilateral Painful attacks may occur spontaneously but are more often associated with a specific stimulus such as chewing speaking coughing swallowing or laughing The attacks can occur frequently throughout the day or once every couple of weeks and they can also disturb sleep
bull httpwwwcolumbianeurosurgeryorgconditionsglossopharyngeal-neuralgia
Causes of Glossopharyngeal Neuralgiabull Neuralgia is typically caused by compression of the
glossopharyngeal nerve
bull Compression of the nerve by an adjacent artery andor vein can occur near the brainstem
bull Compression of the nerve more distally in the neck by an elongated styloid process (a bone in the neck) can cause this pain syndrome as well and this is called Eagle syndrome
bull Glossopharyngeal neuralgia can be caused by a tumor or infection in the region of ninth cranial nerve or by multiple sclerosis
bull httpwwwcolumbianeurosurgeryorgconditionsglossopharyngeal-neuralgia
Differential diagnosis of Glossopharyngeal Neuritis (IX)
bull The diagnosis is made on clinical grounds alone
bull no specific test can be done to prove the diagnosis in any given patient Episodic ear andor throat pain often triggered by touching the palate or tonsil is strongly suggestive of the disorder
bull High resolution MRI or CT imaging of the brainstem may reveal vascular compression tumors or demyelinating lesions involving the ninth cranial nerve
bull High resolution CT scanning of the neck can reveal an elongated styloid process suggestive of Eagle syndrome
bull Pain that distinctly involves the ear rear 13 of the tongue andor throat is the classic pain distribution seen in glossopharyngeal neuralgia (ninth cranial nerve or IX N)
httpwwwcolumbianeurosurgeryorgconditionsglossopharyngeal-neuralgia
Treatment of Glossopharyngeal Neuritisbull Most treatment for Glossopharyngeal Neuralgia is
pharmacological Anticonvulsant medications including oxcarbazepine carbamazepine gabapentin and pregabalin Some antidepressants can also be helpful either alone or in combination with the seizure medications If medications fail surgical intervention is indicated
bull The first-choice treatment microvascular decompression (MVD) as it has the highest initial and long-term success rates Patients unable to tolerate such an operation may consider one of the many less invasive options including Gamma Knife radiosurgery
bull Eagles syndrome TMJ and Trigeminal neuralgia can co-exist with glossopharyngeal neuritis and mask the diagnosis or be mistaken for it
httpwwwcolumbianeurosurgeryorgconditionsglossopharyngeal-neuralgia
How do you treat temporomandibular(TMJ) joint syndrome
bull Muscles relaxants and anti-inflammatory drugs such as iboprophen or naproxen are helpful (Guralnick W Kaban L and Merrill R Temporomandibular joint afflictions New England Journal of Medicine Vol 299 No 3 pp 120-129 July 20 1978)
bull The use of a Kil-Brux night guard may help which is a bite guard which fits over teeth on one side and with a smooth side on the other side so the opposing teeth can slide over the smooth surface
bull The dentist should never grind down the teeth which alters the bite and repositions the jaw
bull Only in rare instances is surgery recommended to repair the disc or ligaments and rarely does it improve the condition
Temporal Bone
JAW
BONE
SKULL
Normal TMJ
anatomy The
condyle is the part
of the jaw bone
which inserts into
the recess in the
skull Ligaments
hold the jaw bone
into the recess or
fossa (another name
for recess) The
fossa is an
indentation in the
skull bone which
accepts the condyle
of the jaw bone
Think of ligaments
like stretched rubber
bands holding the
condyle into the
fossa
The disc is like a
cushion and a shock
absorber The disc is
sort of like a water
filled balloon which
allows the condyle to
roll around in the
fossa while being
held in place by
ligaments The jaw
can slide forward or
back side to side or
pivot-a 3 way joint
In TMJ either the
disc or ligaments are
torn and this allow
the condyle to move
more than it should
Sometime the disc
moves too and gets
trapped between the
front of the fossa and
the condyle when
the jaw is opened
This causes the click
heard with TMJ
which is sort of like
snapping your
fingers
Condyle
Disc
Fossa
ligaments
What is trigeminal neuralgia bull There are two main etiologies to the pain of trigeminal
neuralgia bull In both types the pain can be constant or intermittent and
follows the path of the sensory branches of the trigeminal nerve into the forehead along the cheek bone or in the jaw line or any combination of the three (Empting L Hendler N Kolodny L Kraus H Tips on hard to manage pain syndromes Patient Care page 26-46 April 30 1990)
bull One etiology is a congenital compression of the trigeminal nerve by the middle cerebral artery in the skull This was well researched by Peter Janetta MD and his team at Uni of Pttisburgh
bull (Sekula RF Marchan EM Fletcher LH Casey KF Jannetta PJ Microvasculardecompression for trigeminal neuralgia in elderly patients J Neuro Neurosurg2008 Apr108(4)689-91)
SENSORY DISTRIBUTION
OF THE TRIGEMINAL
NERVE V IS FOR ROMAN
NUMERAL FIVE THERE
ARE THREE SENSORY
BRANCHES V1 or the
ophthalmic branch V2 or
the maxillary branch AND
V3 or the mandibular
branch Notice all the
sensory sensation is in the
jaw cheek and forehead
nose and eye
What is trigeminal neuralgia
bull A second form of trigeminal neuralgia is a herpetic-like viral irritation of the trigeminal nerve (Empting L Hendler N Kolodny L Kraus H Tips on hard to manage pain syndromes Patient Care page 26-46 April 30 1990)
bull Interestingly the pain of trigeminal neuralgia from both sources is clinically the same
bull This is an example of ldquoconvergencerdquo which is a single clinical manifestation due to several etiologies
How do you diagnose trigeminal neuralgia
bull The pain of both types of trigeminal neuralgia can be constant or intermittent and follows the path of the sensory branches of the trigeminal nerve into the forehead along the cheek bone or in the jaw line or any combination of the three (Empting L Hendler N Kolodny L Kraus H Tips on hard to manage pain syndromes Patient Care page 26-46 April 30 1990)
bull The pain is sensitive to light touch and may occur in burst of pain rather than being constant This occurs in both types of trigeminal neuralgia
How do you diagnose trigeminal neuralgia
bull In order to differentiate between the two forms of trigeminal neuralgia an MRI of the brain with vascular enhancement is useful
bull If there is no vessel compression it is highly likely the cause of the pain is post-herpetic
bull In addition to an MRI with vascular enhancement an MRI of the brain with thin-sections (2 mm slices) through the trigeminal nerve and an MRA (Magnet resonance angiography) may be helpful in differentiating the post-viral etiology from the compression of the nerve by the blood vessel
What is the treatment of trigeminal neuralgia
bull Since there are two main etiologies to the pain of trigeminal neuralgia there are two types of treatment
bull If the etiology is compression of the trigeminal nerve by the middle cerebral artery in the skull then the treatment is intra-cranial decompression of the nerve by inserting a sponge between the artery and the nerve (Sekula RF Marchan EM Fletcher LH Casey KF Jannetta PJ Microvascular decompression for trigeminal neuralgia in elderly patients J NeuroNeurosurg2008 Apr108(4)689-91)
What is the treatment of trigeminal neuralgia
bull If the etiology is due to a herpetic-like viral irritation of the trigeminal nerve (Empting L Hendler N Kolodny L Kraus H Tips on hard to manage pain syndromes Patient Care page 26-46 April 30 1990) then the treatment is the use of medications
bull Some people respond to low dose anti-depressants but the most useful medication is an anti-convulsant such as gaba-pentin (Neurontin) or Tegretol
bull Anti-viral drug such as acyclovir will also help
bull Rarely radio-frequency lesions of the retro-gausserian ganglion can be employed if all else fails
What is Eagles syndrome
bull Eagles Syndrome is characterized by an a specific orofacial pain secondary to calcification of the stylohyoid ligament or elongated styoid process In about 4 of general population an elongated styloid process occurs while only about 4 of these patients are symptomatic The stylo-hyoid bone is considered abnormal if it is greater than 25 mm in length (Casale M Rinaldi V Quattrocchi C Bressi F Vincenzi B Santini D Tonini G Salvinelli F Atypical chronic head and neck pain dont forget Eagles syndrome Eur Rev Med Pharmacol Sci 2008 Mar-Apr12(2)131-3)
3D-CT of a very long
stylo-hyoid
bonehellipmuch longer
than 25 mm Follow
the numbers 123
and 4 on both sides of
the 3D-CT and the is
the stylo-hyoid bone
which is about 39 mm
in this 3D-CT The
bone marked H is the
hyoid bone In this
3D-CT the stylo-hyoid
bone is so long that is
spontaneously broke
between 2 and 3 but it
is still symptomatic
and causes pain with
swollowing Removal
of the bone from the
break to 4 on the 3D-
CT will relieve
symptoms
What are the symptoms of Eagles syndrome and how do you diagnosis itbull The patient has a sensation of a foreign body localized at
the tonsillar fossa associated with a dull intermittent painbull Pain is usually worse with swallowingbull A bony projection is usually palpable with bimanual trans-
oral explorationbull Basically you put your finger in the mouth of the patient
down their throat and push towards the outside where the stylohyoid bone is If this reproduces the pain this confirms the diagnosis
bull A lateral radiograph and a computed tomography scan of head and neck will show an elongated styloid process of at least 25 mm (Casale M Rinaldi V Quattrocchi C Bressi F Vincenzi B Santini D Tonini G Salvinelli FAtypical chronic head and neck pain dont forget Eagles syndrome Eur Rev Med Pharmacol Sci 2008 Mar-Apr12(2)131-3)
How do you treat Eagles syndromebull Medical treatment represents the first choice
bull Use non-steroidal anti- inflammatory drugs
bull If this is not effective then surgical resection of the styloid process may help (cutting off the tip)
bull In the case of persistence or ingravescence of the complaint surgery may be the only option
bull Symptoms may overlap with glossopharyngealneuritis (presented later)
bull (Casale M Rinaldi V Quattrocchi C Bressi F Vincenzi B Santini D Tonini G
Salvinelli F Atypical chronic head and neck pain dont forget Eagles syndrome EurRev Med Pharmacol Sci 2008 Mar-Apr12(2)131-3)
What is Sjogrenrsquos Syndrome bull Sjogren syndrome is an autoimmune disorder in
which the glands that produce tears and saliva are destroyed causing dry mouth and dry eyes
bull Symptoms may include itching eyes feeling that something is in the eye difficulty swallowing loss of sense of taste thick saliva mouth sores many cavities in the teeth and pain in the face usually in the trigeminal or facial nerve
bull (Kruszka P OBrian RJ Diagnosis and management of Sjogren syndrome Am Fam Physician 200979465-470)
What are symptoms of Sjogrenrsquos Syndromebull Can be mistaken for trigeminal neuralgia
bull When there is unexplained pain in the face take a careful history Ask about dry mouth itchy eyes lots of cavities trouble swallowing SICCA complex (1)
bull Trigeminal sensory neuropathy can occur with Sjogrenrsquos and may be characterized by progressive sensory complaints on the face They could start on one side and subsequently become bilateral (2)
bull (1) Wu AJ Optimizing dry mouth treatment for individuals with Sjoumlgrens syndrome Rheum Dis Clin North Am 2008 Nov34(4)1001-1010
bull (2) Steven Mandel MD Clinical Professor of Neurology Jefferson Medical College)
Laboratory Tests to Diagnose Sjogrenrsquos
bull SSA and SSB ANA sed rate rheumatoid factor (RF) Schirmer test corneal fluorescein staining conjunctival lissamine green staining tear-film breakup time and lip biopsy are useful methods of diagnosing Sjogrenrsquos but not always reliable
bull Yang Z Liang Y Zhong R Is identification of anti-SSA andor -SSB antibodies necessary in serum samples referred for antinuclear antibodies testing J Clin Lab Anal 2012 Nov26(6)447-51 Br J Ophthalmol 2012 Dec96(12)1498-503 doi with aqueous-deficient dry eye
bull Liew MS Zhang M Kim E Akpek EK Prevalence and predictors of Sjogrens syndrome in a prospective cohort of patients with aqueous-deficient dry eye Br J Ophthalmol 2012 Dec96(12)1498-503
Treatment of Sjogrenrsquosbull Conventional immunosuppressive therapies
such as corticosteroids have been used in some patients with variable benefit
bull Current therapy for dryness is principally symptomatic
bull The greatest experience in pSS is with rituximab an anti-CD20 (expressed on a subset of B-cells) monoclonal antibody
(Bowman S Barone F Biologic treatments in Sjoumlgrens syndrome Presse Med 2012 Sep41(9 Pt 2)e495-509)
Symptoms of Glossopharyngeal Neuritis
bull Glossopharyngeal neuralgia is classically described as ldquostabbingrdquo or ldquoelectrical shock-likerdquo in nature Episodes are typically brief lasting a couple of seconds up to a few minutes and can be quite debilitating They can occur in the tongue throat ear and tonsils on one side of the head but in rare cases may be bilateral Painful attacks may occur spontaneously but are more often associated with a specific stimulus such as chewing speaking coughing swallowing or laughing The attacks can occur frequently throughout the day or once every couple of weeks and they can also disturb sleep
bull httpwwwcolumbianeurosurgeryorgconditionsglossopharyngeal-neuralgia
Causes of Glossopharyngeal Neuralgiabull Neuralgia is typically caused by compression of the
glossopharyngeal nerve
bull Compression of the nerve by an adjacent artery andor vein can occur near the brainstem
bull Compression of the nerve more distally in the neck by an elongated styloid process (a bone in the neck) can cause this pain syndrome as well and this is called Eagle syndrome
bull Glossopharyngeal neuralgia can be caused by a tumor or infection in the region of ninth cranial nerve or by multiple sclerosis
bull httpwwwcolumbianeurosurgeryorgconditionsglossopharyngeal-neuralgia
Differential diagnosis of Glossopharyngeal Neuritis (IX)
bull The diagnosis is made on clinical grounds alone
bull no specific test can be done to prove the diagnosis in any given patient Episodic ear andor throat pain often triggered by touching the palate or tonsil is strongly suggestive of the disorder
bull High resolution MRI or CT imaging of the brainstem may reveal vascular compression tumors or demyelinating lesions involving the ninth cranial nerve
bull High resolution CT scanning of the neck can reveal an elongated styloid process suggestive of Eagle syndrome
bull Pain that distinctly involves the ear rear 13 of the tongue andor throat is the classic pain distribution seen in glossopharyngeal neuralgia (ninth cranial nerve or IX N)
httpwwwcolumbianeurosurgeryorgconditionsglossopharyngeal-neuralgia
Treatment of Glossopharyngeal Neuritisbull Most treatment for Glossopharyngeal Neuralgia is
pharmacological Anticonvulsant medications including oxcarbazepine carbamazepine gabapentin and pregabalin Some antidepressants can also be helpful either alone or in combination with the seizure medications If medications fail surgical intervention is indicated
bull The first-choice treatment microvascular decompression (MVD) as it has the highest initial and long-term success rates Patients unable to tolerate such an operation may consider one of the many less invasive options including Gamma Knife radiosurgery
bull Eagles syndrome TMJ and Trigeminal neuralgia can co-exist with glossopharyngeal neuritis and mask the diagnosis or be mistaken for it
httpwwwcolumbianeurosurgeryorgconditionsglossopharyngeal-neuralgia
Temporal Bone
JAW
BONE
SKULL
Normal TMJ
anatomy The
condyle is the part
of the jaw bone
which inserts into
the recess in the
skull Ligaments
hold the jaw bone
into the recess or
fossa (another name
for recess) The
fossa is an
indentation in the
skull bone which
accepts the condyle
of the jaw bone
Think of ligaments
like stretched rubber
bands holding the
condyle into the
fossa
The disc is like a
cushion and a shock
absorber The disc is
sort of like a water
filled balloon which
allows the condyle to
roll around in the
fossa while being
held in place by
ligaments The jaw
can slide forward or
back side to side or
pivot-a 3 way joint
In TMJ either the
disc or ligaments are
torn and this allow
the condyle to move
more than it should
Sometime the disc
moves too and gets
trapped between the
front of the fossa and
the condyle when
the jaw is opened
This causes the click
heard with TMJ
which is sort of like
snapping your
fingers
Condyle
Disc
Fossa
ligaments
What is trigeminal neuralgia bull There are two main etiologies to the pain of trigeminal
neuralgia bull In both types the pain can be constant or intermittent and
follows the path of the sensory branches of the trigeminal nerve into the forehead along the cheek bone or in the jaw line or any combination of the three (Empting L Hendler N Kolodny L Kraus H Tips on hard to manage pain syndromes Patient Care page 26-46 April 30 1990)
bull One etiology is a congenital compression of the trigeminal nerve by the middle cerebral artery in the skull This was well researched by Peter Janetta MD and his team at Uni of Pttisburgh
bull (Sekula RF Marchan EM Fletcher LH Casey KF Jannetta PJ Microvasculardecompression for trigeminal neuralgia in elderly patients J Neuro Neurosurg2008 Apr108(4)689-91)
SENSORY DISTRIBUTION
OF THE TRIGEMINAL
NERVE V IS FOR ROMAN
NUMERAL FIVE THERE
ARE THREE SENSORY
BRANCHES V1 or the
ophthalmic branch V2 or
the maxillary branch AND
V3 or the mandibular
branch Notice all the
sensory sensation is in the
jaw cheek and forehead
nose and eye
What is trigeminal neuralgia
bull A second form of trigeminal neuralgia is a herpetic-like viral irritation of the trigeminal nerve (Empting L Hendler N Kolodny L Kraus H Tips on hard to manage pain syndromes Patient Care page 26-46 April 30 1990)
bull Interestingly the pain of trigeminal neuralgia from both sources is clinically the same
bull This is an example of ldquoconvergencerdquo which is a single clinical manifestation due to several etiologies
How do you diagnose trigeminal neuralgia
bull The pain of both types of trigeminal neuralgia can be constant or intermittent and follows the path of the sensory branches of the trigeminal nerve into the forehead along the cheek bone or in the jaw line or any combination of the three (Empting L Hendler N Kolodny L Kraus H Tips on hard to manage pain syndromes Patient Care page 26-46 April 30 1990)
bull The pain is sensitive to light touch and may occur in burst of pain rather than being constant This occurs in both types of trigeminal neuralgia
How do you diagnose trigeminal neuralgia
bull In order to differentiate between the two forms of trigeminal neuralgia an MRI of the brain with vascular enhancement is useful
bull If there is no vessel compression it is highly likely the cause of the pain is post-herpetic
bull In addition to an MRI with vascular enhancement an MRI of the brain with thin-sections (2 mm slices) through the trigeminal nerve and an MRA (Magnet resonance angiography) may be helpful in differentiating the post-viral etiology from the compression of the nerve by the blood vessel
What is the treatment of trigeminal neuralgia
bull Since there are two main etiologies to the pain of trigeminal neuralgia there are two types of treatment
bull If the etiology is compression of the trigeminal nerve by the middle cerebral artery in the skull then the treatment is intra-cranial decompression of the nerve by inserting a sponge between the artery and the nerve (Sekula RF Marchan EM Fletcher LH Casey KF Jannetta PJ Microvascular decompression for trigeminal neuralgia in elderly patients J NeuroNeurosurg2008 Apr108(4)689-91)
What is the treatment of trigeminal neuralgia
bull If the etiology is due to a herpetic-like viral irritation of the trigeminal nerve (Empting L Hendler N Kolodny L Kraus H Tips on hard to manage pain syndromes Patient Care page 26-46 April 30 1990) then the treatment is the use of medications
bull Some people respond to low dose anti-depressants but the most useful medication is an anti-convulsant such as gaba-pentin (Neurontin) or Tegretol
bull Anti-viral drug such as acyclovir will also help
bull Rarely radio-frequency lesions of the retro-gausserian ganglion can be employed if all else fails
What is Eagles syndrome
bull Eagles Syndrome is characterized by an a specific orofacial pain secondary to calcification of the stylohyoid ligament or elongated styoid process In about 4 of general population an elongated styloid process occurs while only about 4 of these patients are symptomatic The stylo-hyoid bone is considered abnormal if it is greater than 25 mm in length (Casale M Rinaldi V Quattrocchi C Bressi F Vincenzi B Santini D Tonini G Salvinelli F Atypical chronic head and neck pain dont forget Eagles syndrome Eur Rev Med Pharmacol Sci 2008 Mar-Apr12(2)131-3)
3D-CT of a very long
stylo-hyoid
bonehellipmuch longer
than 25 mm Follow
the numbers 123
and 4 on both sides of
the 3D-CT and the is
the stylo-hyoid bone
which is about 39 mm
in this 3D-CT The
bone marked H is the
hyoid bone In this
3D-CT the stylo-hyoid
bone is so long that is
spontaneously broke
between 2 and 3 but it
is still symptomatic
and causes pain with
swollowing Removal
of the bone from the
break to 4 on the 3D-
CT will relieve
symptoms
What are the symptoms of Eagles syndrome and how do you diagnosis itbull The patient has a sensation of a foreign body localized at
the tonsillar fossa associated with a dull intermittent painbull Pain is usually worse with swallowingbull A bony projection is usually palpable with bimanual trans-
oral explorationbull Basically you put your finger in the mouth of the patient
down their throat and push towards the outside where the stylohyoid bone is If this reproduces the pain this confirms the diagnosis
bull A lateral radiograph and a computed tomography scan of head and neck will show an elongated styloid process of at least 25 mm (Casale M Rinaldi V Quattrocchi C Bressi F Vincenzi B Santini D Tonini G Salvinelli FAtypical chronic head and neck pain dont forget Eagles syndrome Eur Rev Med Pharmacol Sci 2008 Mar-Apr12(2)131-3)
How do you treat Eagles syndromebull Medical treatment represents the first choice
bull Use non-steroidal anti- inflammatory drugs
bull If this is not effective then surgical resection of the styloid process may help (cutting off the tip)
bull In the case of persistence or ingravescence of the complaint surgery may be the only option
bull Symptoms may overlap with glossopharyngealneuritis (presented later)
bull (Casale M Rinaldi V Quattrocchi C Bressi F Vincenzi B Santini D Tonini G
Salvinelli F Atypical chronic head and neck pain dont forget Eagles syndrome EurRev Med Pharmacol Sci 2008 Mar-Apr12(2)131-3)
What is Sjogrenrsquos Syndrome bull Sjogren syndrome is an autoimmune disorder in
which the glands that produce tears and saliva are destroyed causing dry mouth and dry eyes
bull Symptoms may include itching eyes feeling that something is in the eye difficulty swallowing loss of sense of taste thick saliva mouth sores many cavities in the teeth and pain in the face usually in the trigeminal or facial nerve
bull (Kruszka P OBrian RJ Diagnosis and management of Sjogren syndrome Am Fam Physician 200979465-470)
What are symptoms of Sjogrenrsquos Syndromebull Can be mistaken for trigeminal neuralgia
bull When there is unexplained pain in the face take a careful history Ask about dry mouth itchy eyes lots of cavities trouble swallowing SICCA complex (1)
bull Trigeminal sensory neuropathy can occur with Sjogrenrsquos and may be characterized by progressive sensory complaints on the face They could start on one side and subsequently become bilateral (2)
bull (1) Wu AJ Optimizing dry mouth treatment for individuals with Sjoumlgrens syndrome Rheum Dis Clin North Am 2008 Nov34(4)1001-1010
bull (2) Steven Mandel MD Clinical Professor of Neurology Jefferson Medical College)
Laboratory Tests to Diagnose Sjogrenrsquos
bull SSA and SSB ANA sed rate rheumatoid factor (RF) Schirmer test corneal fluorescein staining conjunctival lissamine green staining tear-film breakup time and lip biopsy are useful methods of diagnosing Sjogrenrsquos but not always reliable
bull Yang Z Liang Y Zhong R Is identification of anti-SSA andor -SSB antibodies necessary in serum samples referred for antinuclear antibodies testing J Clin Lab Anal 2012 Nov26(6)447-51 Br J Ophthalmol 2012 Dec96(12)1498-503 doi with aqueous-deficient dry eye
bull Liew MS Zhang M Kim E Akpek EK Prevalence and predictors of Sjogrens syndrome in a prospective cohort of patients with aqueous-deficient dry eye Br J Ophthalmol 2012 Dec96(12)1498-503
Treatment of Sjogrenrsquosbull Conventional immunosuppressive therapies
such as corticosteroids have been used in some patients with variable benefit
bull Current therapy for dryness is principally symptomatic
bull The greatest experience in pSS is with rituximab an anti-CD20 (expressed on a subset of B-cells) monoclonal antibody
(Bowman S Barone F Biologic treatments in Sjoumlgrens syndrome Presse Med 2012 Sep41(9 Pt 2)e495-509)
Symptoms of Glossopharyngeal Neuritis
bull Glossopharyngeal neuralgia is classically described as ldquostabbingrdquo or ldquoelectrical shock-likerdquo in nature Episodes are typically brief lasting a couple of seconds up to a few minutes and can be quite debilitating They can occur in the tongue throat ear and tonsils on one side of the head but in rare cases may be bilateral Painful attacks may occur spontaneously but are more often associated with a specific stimulus such as chewing speaking coughing swallowing or laughing The attacks can occur frequently throughout the day or once every couple of weeks and they can also disturb sleep
bull httpwwwcolumbianeurosurgeryorgconditionsglossopharyngeal-neuralgia
Causes of Glossopharyngeal Neuralgiabull Neuralgia is typically caused by compression of the
glossopharyngeal nerve
bull Compression of the nerve by an adjacent artery andor vein can occur near the brainstem
bull Compression of the nerve more distally in the neck by an elongated styloid process (a bone in the neck) can cause this pain syndrome as well and this is called Eagle syndrome
bull Glossopharyngeal neuralgia can be caused by a tumor or infection in the region of ninth cranial nerve or by multiple sclerosis
bull httpwwwcolumbianeurosurgeryorgconditionsglossopharyngeal-neuralgia
Differential diagnosis of Glossopharyngeal Neuritis (IX)
bull The diagnosis is made on clinical grounds alone
bull no specific test can be done to prove the diagnosis in any given patient Episodic ear andor throat pain often triggered by touching the palate or tonsil is strongly suggestive of the disorder
bull High resolution MRI or CT imaging of the brainstem may reveal vascular compression tumors or demyelinating lesions involving the ninth cranial nerve
bull High resolution CT scanning of the neck can reveal an elongated styloid process suggestive of Eagle syndrome
bull Pain that distinctly involves the ear rear 13 of the tongue andor throat is the classic pain distribution seen in glossopharyngeal neuralgia (ninth cranial nerve or IX N)
httpwwwcolumbianeurosurgeryorgconditionsglossopharyngeal-neuralgia
Treatment of Glossopharyngeal Neuritisbull Most treatment for Glossopharyngeal Neuralgia is
pharmacological Anticonvulsant medications including oxcarbazepine carbamazepine gabapentin and pregabalin Some antidepressants can also be helpful either alone or in combination with the seizure medications If medications fail surgical intervention is indicated
bull The first-choice treatment microvascular decompression (MVD) as it has the highest initial and long-term success rates Patients unable to tolerate such an operation may consider one of the many less invasive options including Gamma Knife radiosurgery
bull Eagles syndrome TMJ and Trigeminal neuralgia can co-exist with glossopharyngeal neuritis and mask the diagnosis or be mistaken for it
httpwwwcolumbianeurosurgeryorgconditionsglossopharyngeal-neuralgia
In TMJ either the
disc or ligaments are
torn and this allow
the condyle to move
more than it should
Sometime the disc
moves too and gets
trapped between the
front of the fossa and
the condyle when
the jaw is opened
This causes the click
heard with TMJ
which is sort of like
snapping your
fingers
Condyle
Disc
Fossa
ligaments
What is trigeminal neuralgia bull There are two main etiologies to the pain of trigeminal
neuralgia bull In both types the pain can be constant or intermittent and
follows the path of the sensory branches of the trigeminal nerve into the forehead along the cheek bone or in the jaw line or any combination of the three (Empting L Hendler N Kolodny L Kraus H Tips on hard to manage pain syndromes Patient Care page 26-46 April 30 1990)
bull One etiology is a congenital compression of the trigeminal nerve by the middle cerebral artery in the skull This was well researched by Peter Janetta MD and his team at Uni of Pttisburgh
bull (Sekula RF Marchan EM Fletcher LH Casey KF Jannetta PJ Microvasculardecompression for trigeminal neuralgia in elderly patients J Neuro Neurosurg2008 Apr108(4)689-91)
SENSORY DISTRIBUTION
OF THE TRIGEMINAL
NERVE V IS FOR ROMAN
NUMERAL FIVE THERE
ARE THREE SENSORY
BRANCHES V1 or the
ophthalmic branch V2 or
the maxillary branch AND
V3 or the mandibular
branch Notice all the
sensory sensation is in the
jaw cheek and forehead
nose and eye
What is trigeminal neuralgia
bull A second form of trigeminal neuralgia is a herpetic-like viral irritation of the trigeminal nerve (Empting L Hendler N Kolodny L Kraus H Tips on hard to manage pain syndromes Patient Care page 26-46 April 30 1990)
bull Interestingly the pain of trigeminal neuralgia from both sources is clinically the same
bull This is an example of ldquoconvergencerdquo which is a single clinical manifestation due to several etiologies
How do you diagnose trigeminal neuralgia
bull The pain of both types of trigeminal neuralgia can be constant or intermittent and follows the path of the sensory branches of the trigeminal nerve into the forehead along the cheek bone or in the jaw line or any combination of the three (Empting L Hendler N Kolodny L Kraus H Tips on hard to manage pain syndromes Patient Care page 26-46 April 30 1990)
bull The pain is sensitive to light touch and may occur in burst of pain rather than being constant This occurs in both types of trigeminal neuralgia
How do you diagnose trigeminal neuralgia
bull In order to differentiate between the two forms of trigeminal neuralgia an MRI of the brain with vascular enhancement is useful
bull If there is no vessel compression it is highly likely the cause of the pain is post-herpetic
bull In addition to an MRI with vascular enhancement an MRI of the brain with thin-sections (2 mm slices) through the trigeminal nerve and an MRA (Magnet resonance angiography) may be helpful in differentiating the post-viral etiology from the compression of the nerve by the blood vessel
What is the treatment of trigeminal neuralgia
bull Since there are two main etiologies to the pain of trigeminal neuralgia there are two types of treatment
bull If the etiology is compression of the trigeminal nerve by the middle cerebral artery in the skull then the treatment is intra-cranial decompression of the nerve by inserting a sponge between the artery and the nerve (Sekula RF Marchan EM Fletcher LH Casey KF Jannetta PJ Microvascular decompression for trigeminal neuralgia in elderly patients J NeuroNeurosurg2008 Apr108(4)689-91)
What is the treatment of trigeminal neuralgia
bull If the etiology is due to a herpetic-like viral irritation of the trigeminal nerve (Empting L Hendler N Kolodny L Kraus H Tips on hard to manage pain syndromes Patient Care page 26-46 April 30 1990) then the treatment is the use of medications
bull Some people respond to low dose anti-depressants but the most useful medication is an anti-convulsant such as gaba-pentin (Neurontin) or Tegretol
bull Anti-viral drug such as acyclovir will also help
bull Rarely radio-frequency lesions of the retro-gausserian ganglion can be employed if all else fails
What is Eagles syndrome
bull Eagles Syndrome is characterized by an a specific orofacial pain secondary to calcification of the stylohyoid ligament or elongated styoid process In about 4 of general population an elongated styloid process occurs while only about 4 of these patients are symptomatic The stylo-hyoid bone is considered abnormal if it is greater than 25 mm in length (Casale M Rinaldi V Quattrocchi C Bressi F Vincenzi B Santini D Tonini G Salvinelli F Atypical chronic head and neck pain dont forget Eagles syndrome Eur Rev Med Pharmacol Sci 2008 Mar-Apr12(2)131-3)
3D-CT of a very long
stylo-hyoid
bonehellipmuch longer
than 25 mm Follow
the numbers 123
and 4 on both sides of
the 3D-CT and the is
the stylo-hyoid bone
which is about 39 mm
in this 3D-CT The
bone marked H is the
hyoid bone In this
3D-CT the stylo-hyoid
bone is so long that is
spontaneously broke
between 2 and 3 but it
is still symptomatic
and causes pain with
swollowing Removal
of the bone from the
break to 4 on the 3D-
CT will relieve
symptoms
What are the symptoms of Eagles syndrome and how do you diagnosis itbull The patient has a sensation of a foreign body localized at
the tonsillar fossa associated with a dull intermittent painbull Pain is usually worse with swallowingbull A bony projection is usually palpable with bimanual trans-
oral explorationbull Basically you put your finger in the mouth of the patient
down their throat and push towards the outside where the stylohyoid bone is If this reproduces the pain this confirms the diagnosis
bull A lateral radiograph and a computed tomography scan of head and neck will show an elongated styloid process of at least 25 mm (Casale M Rinaldi V Quattrocchi C Bressi F Vincenzi B Santini D Tonini G Salvinelli FAtypical chronic head and neck pain dont forget Eagles syndrome Eur Rev Med Pharmacol Sci 2008 Mar-Apr12(2)131-3)
How do you treat Eagles syndromebull Medical treatment represents the first choice
bull Use non-steroidal anti- inflammatory drugs
bull If this is not effective then surgical resection of the styloid process may help (cutting off the tip)
bull In the case of persistence or ingravescence of the complaint surgery may be the only option
bull Symptoms may overlap with glossopharyngealneuritis (presented later)
bull (Casale M Rinaldi V Quattrocchi C Bressi F Vincenzi B Santini D Tonini G
Salvinelli F Atypical chronic head and neck pain dont forget Eagles syndrome EurRev Med Pharmacol Sci 2008 Mar-Apr12(2)131-3)
What is Sjogrenrsquos Syndrome bull Sjogren syndrome is an autoimmune disorder in
which the glands that produce tears and saliva are destroyed causing dry mouth and dry eyes
bull Symptoms may include itching eyes feeling that something is in the eye difficulty swallowing loss of sense of taste thick saliva mouth sores many cavities in the teeth and pain in the face usually in the trigeminal or facial nerve
bull (Kruszka P OBrian RJ Diagnosis and management of Sjogren syndrome Am Fam Physician 200979465-470)
What are symptoms of Sjogrenrsquos Syndromebull Can be mistaken for trigeminal neuralgia
bull When there is unexplained pain in the face take a careful history Ask about dry mouth itchy eyes lots of cavities trouble swallowing SICCA complex (1)
bull Trigeminal sensory neuropathy can occur with Sjogrenrsquos and may be characterized by progressive sensory complaints on the face They could start on one side and subsequently become bilateral (2)
bull (1) Wu AJ Optimizing dry mouth treatment for individuals with Sjoumlgrens syndrome Rheum Dis Clin North Am 2008 Nov34(4)1001-1010
bull (2) Steven Mandel MD Clinical Professor of Neurology Jefferson Medical College)
Laboratory Tests to Diagnose Sjogrenrsquos
bull SSA and SSB ANA sed rate rheumatoid factor (RF) Schirmer test corneal fluorescein staining conjunctival lissamine green staining tear-film breakup time and lip biopsy are useful methods of diagnosing Sjogrenrsquos but not always reliable
bull Yang Z Liang Y Zhong R Is identification of anti-SSA andor -SSB antibodies necessary in serum samples referred for antinuclear antibodies testing J Clin Lab Anal 2012 Nov26(6)447-51 Br J Ophthalmol 2012 Dec96(12)1498-503 doi with aqueous-deficient dry eye
bull Liew MS Zhang M Kim E Akpek EK Prevalence and predictors of Sjogrens syndrome in a prospective cohort of patients with aqueous-deficient dry eye Br J Ophthalmol 2012 Dec96(12)1498-503
Treatment of Sjogrenrsquosbull Conventional immunosuppressive therapies
such as corticosteroids have been used in some patients with variable benefit
bull Current therapy for dryness is principally symptomatic
bull The greatest experience in pSS is with rituximab an anti-CD20 (expressed on a subset of B-cells) monoclonal antibody
(Bowman S Barone F Biologic treatments in Sjoumlgrens syndrome Presse Med 2012 Sep41(9 Pt 2)e495-509)
Symptoms of Glossopharyngeal Neuritis
bull Glossopharyngeal neuralgia is classically described as ldquostabbingrdquo or ldquoelectrical shock-likerdquo in nature Episodes are typically brief lasting a couple of seconds up to a few minutes and can be quite debilitating They can occur in the tongue throat ear and tonsils on one side of the head but in rare cases may be bilateral Painful attacks may occur spontaneously but are more often associated with a specific stimulus such as chewing speaking coughing swallowing or laughing The attacks can occur frequently throughout the day or once every couple of weeks and they can also disturb sleep
bull httpwwwcolumbianeurosurgeryorgconditionsglossopharyngeal-neuralgia
Causes of Glossopharyngeal Neuralgiabull Neuralgia is typically caused by compression of the
glossopharyngeal nerve
bull Compression of the nerve by an adjacent artery andor vein can occur near the brainstem
bull Compression of the nerve more distally in the neck by an elongated styloid process (a bone in the neck) can cause this pain syndrome as well and this is called Eagle syndrome
bull Glossopharyngeal neuralgia can be caused by a tumor or infection in the region of ninth cranial nerve or by multiple sclerosis
bull httpwwwcolumbianeurosurgeryorgconditionsglossopharyngeal-neuralgia
Differential diagnosis of Glossopharyngeal Neuritis (IX)
bull The diagnosis is made on clinical grounds alone
bull no specific test can be done to prove the diagnosis in any given patient Episodic ear andor throat pain often triggered by touching the palate or tonsil is strongly suggestive of the disorder
bull High resolution MRI or CT imaging of the brainstem may reveal vascular compression tumors or demyelinating lesions involving the ninth cranial nerve
bull High resolution CT scanning of the neck can reveal an elongated styloid process suggestive of Eagle syndrome
bull Pain that distinctly involves the ear rear 13 of the tongue andor throat is the classic pain distribution seen in glossopharyngeal neuralgia (ninth cranial nerve or IX N)
httpwwwcolumbianeurosurgeryorgconditionsglossopharyngeal-neuralgia
Treatment of Glossopharyngeal Neuritisbull Most treatment for Glossopharyngeal Neuralgia is
pharmacological Anticonvulsant medications including oxcarbazepine carbamazepine gabapentin and pregabalin Some antidepressants can also be helpful either alone or in combination with the seizure medications If medications fail surgical intervention is indicated
bull The first-choice treatment microvascular decompression (MVD) as it has the highest initial and long-term success rates Patients unable to tolerate such an operation may consider one of the many less invasive options including Gamma Knife radiosurgery
bull Eagles syndrome TMJ and Trigeminal neuralgia can co-exist with glossopharyngeal neuritis and mask the diagnosis or be mistaken for it
httpwwwcolumbianeurosurgeryorgconditionsglossopharyngeal-neuralgia
What is trigeminal neuralgia bull There are two main etiologies to the pain of trigeminal
neuralgia bull In both types the pain can be constant or intermittent and
follows the path of the sensory branches of the trigeminal nerve into the forehead along the cheek bone or in the jaw line or any combination of the three (Empting L Hendler N Kolodny L Kraus H Tips on hard to manage pain syndromes Patient Care page 26-46 April 30 1990)
bull One etiology is a congenital compression of the trigeminal nerve by the middle cerebral artery in the skull This was well researched by Peter Janetta MD and his team at Uni of Pttisburgh
bull (Sekula RF Marchan EM Fletcher LH Casey KF Jannetta PJ Microvasculardecompression for trigeminal neuralgia in elderly patients J Neuro Neurosurg2008 Apr108(4)689-91)
SENSORY DISTRIBUTION
OF THE TRIGEMINAL
NERVE V IS FOR ROMAN
NUMERAL FIVE THERE
ARE THREE SENSORY
BRANCHES V1 or the
ophthalmic branch V2 or
the maxillary branch AND
V3 or the mandibular
branch Notice all the
sensory sensation is in the
jaw cheek and forehead
nose and eye
What is trigeminal neuralgia
bull A second form of trigeminal neuralgia is a herpetic-like viral irritation of the trigeminal nerve (Empting L Hendler N Kolodny L Kraus H Tips on hard to manage pain syndromes Patient Care page 26-46 April 30 1990)
bull Interestingly the pain of trigeminal neuralgia from both sources is clinically the same
bull This is an example of ldquoconvergencerdquo which is a single clinical manifestation due to several etiologies
How do you diagnose trigeminal neuralgia
bull The pain of both types of trigeminal neuralgia can be constant or intermittent and follows the path of the sensory branches of the trigeminal nerve into the forehead along the cheek bone or in the jaw line or any combination of the three (Empting L Hendler N Kolodny L Kraus H Tips on hard to manage pain syndromes Patient Care page 26-46 April 30 1990)
bull The pain is sensitive to light touch and may occur in burst of pain rather than being constant This occurs in both types of trigeminal neuralgia
How do you diagnose trigeminal neuralgia
bull In order to differentiate between the two forms of trigeminal neuralgia an MRI of the brain with vascular enhancement is useful
bull If there is no vessel compression it is highly likely the cause of the pain is post-herpetic
bull In addition to an MRI with vascular enhancement an MRI of the brain with thin-sections (2 mm slices) through the trigeminal nerve and an MRA (Magnet resonance angiography) may be helpful in differentiating the post-viral etiology from the compression of the nerve by the blood vessel
What is the treatment of trigeminal neuralgia
bull Since there are two main etiologies to the pain of trigeminal neuralgia there are two types of treatment
bull If the etiology is compression of the trigeminal nerve by the middle cerebral artery in the skull then the treatment is intra-cranial decompression of the nerve by inserting a sponge between the artery and the nerve (Sekula RF Marchan EM Fletcher LH Casey KF Jannetta PJ Microvascular decompression for trigeminal neuralgia in elderly patients J NeuroNeurosurg2008 Apr108(4)689-91)
What is the treatment of trigeminal neuralgia
bull If the etiology is due to a herpetic-like viral irritation of the trigeminal nerve (Empting L Hendler N Kolodny L Kraus H Tips on hard to manage pain syndromes Patient Care page 26-46 April 30 1990) then the treatment is the use of medications
bull Some people respond to low dose anti-depressants but the most useful medication is an anti-convulsant such as gaba-pentin (Neurontin) or Tegretol
bull Anti-viral drug such as acyclovir will also help
bull Rarely radio-frequency lesions of the retro-gausserian ganglion can be employed if all else fails
What is Eagles syndrome
bull Eagles Syndrome is characterized by an a specific orofacial pain secondary to calcification of the stylohyoid ligament or elongated styoid process In about 4 of general population an elongated styloid process occurs while only about 4 of these patients are symptomatic The stylo-hyoid bone is considered abnormal if it is greater than 25 mm in length (Casale M Rinaldi V Quattrocchi C Bressi F Vincenzi B Santini D Tonini G Salvinelli F Atypical chronic head and neck pain dont forget Eagles syndrome Eur Rev Med Pharmacol Sci 2008 Mar-Apr12(2)131-3)
3D-CT of a very long
stylo-hyoid
bonehellipmuch longer
than 25 mm Follow
the numbers 123
and 4 on both sides of
the 3D-CT and the is
the stylo-hyoid bone
which is about 39 mm
in this 3D-CT The
bone marked H is the
hyoid bone In this
3D-CT the stylo-hyoid
bone is so long that is
spontaneously broke
between 2 and 3 but it
is still symptomatic
and causes pain with
swollowing Removal
of the bone from the
break to 4 on the 3D-
CT will relieve
symptoms
What are the symptoms of Eagles syndrome and how do you diagnosis itbull The patient has a sensation of a foreign body localized at
the tonsillar fossa associated with a dull intermittent painbull Pain is usually worse with swallowingbull A bony projection is usually palpable with bimanual trans-
oral explorationbull Basically you put your finger in the mouth of the patient
down their throat and push towards the outside where the stylohyoid bone is If this reproduces the pain this confirms the diagnosis
bull A lateral radiograph and a computed tomography scan of head and neck will show an elongated styloid process of at least 25 mm (Casale M Rinaldi V Quattrocchi C Bressi F Vincenzi B Santini D Tonini G Salvinelli FAtypical chronic head and neck pain dont forget Eagles syndrome Eur Rev Med Pharmacol Sci 2008 Mar-Apr12(2)131-3)
How do you treat Eagles syndromebull Medical treatment represents the first choice
bull Use non-steroidal anti- inflammatory drugs
bull If this is not effective then surgical resection of the styloid process may help (cutting off the tip)
bull In the case of persistence or ingravescence of the complaint surgery may be the only option
bull Symptoms may overlap with glossopharyngealneuritis (presented later)
bull (Casale M Rinaldi V Quattrocchi C Bressi F Vincenzi B Santini D Tonini G
Salvinelli F Atypical chronic head and neck pain dont forget Eagles syndrome EurRev Med Pharmacol Sci 2008 Mar-Apr12(2)131-3)
What is Sjogrenrsquos Syndrome bull Sjogren syndrome is an autoimmune disorder in
which the glands that produce tears and saliva are destroyed causing dry mouth and dry eyes
bull Symptoms may include itching eyes feeling that something is in the eye difficulty swallowing loss of sense of taste thick saliva mouth sores many cavities in the teeth and pain in the face usually in the trigeminal or facial nerve
bull (Kruszka P OBrian RJ Diagnosis and management of Sjogren syndrome Am Fam Physician 200979465-470)
What are symptoms of Sjogrenrsquos Syndromebull Can be mistaken for trigeminal neuralgia
bull When there is unexplained pain in the face take a careful history Ask about dry mouth itchy eyes lots of cavities trouble swallowing SICCA complex (1)
bull Trigeminal sensory neuropathy can occur with Sjogrenrsquos and may be characterized by progressive sensory complaints on the face They could start on one side and subsequently become bilateral (2)
bull (1) Wu AJ Optimizing dry mouth treatment for individuals with Sjoumlgrens syndrome Rheum Dis Clin North Am 2008 Nov34(4)1001-1010
bull (2) Steven Mandel MD Clinical Professor of Neurology Jefferson Medical College)
Laboratory Tests to Diagnose Sjogrenrsquos
bull SSA and SSB ANA sed rate rheumatoid factor (RF) Schirmer test corneal fluorescein staining conjunctival lissamine green staining tear-film breakup time and lip biopsy are useful methods of diagnosing Sjogrenrsquos but not always reliable
bull Yang Z Liang Y Zhong R Is identification of anti-SSA andor -SSB antibodies necessary in serum samples referred for antinuclear antibodies testing J Clin Lab Anal 2012 Nov26(6)447-51 Br J Ophthalmol 2012 Dec96(12)1498-503 doi with aqueous-deficient dry eye
bull Liew MS Zhang M Kim E Akpek EK Prevalence and predictors of Sjogrens syndrome in a prospective cohort of patients with aqueous-deficient dry eye Br J Ophthalmol 2012 Dec96(12)1498-503
Treatment of Sjogrenrsquosbull Conventional immunosuppressive therapies
such as corticosteroids have been used in some patients with variable benefit
bull Current therapy for dryness is principally symptomatic
bull The greatest experience in pSS is with rituximab an anti-CD20 (expressed on a subset of B-cells) monoclonal antibody
(Bowman S Barone F Biologic treatments in Sjoumlgrens syndrome Presse Med 2012 Sep41(9 Pt 2)e495-509)
Symptoms of Glossopharyngeal Neuritis
bull Glossopharyngeal neuralgia is classically described as ldquostabbingrdquo or ldquoelectrical shock-likerdquo in nature Episodes are typically brief lasting a couple of seconds up to a few minutes and can be quite debilitating They can occur in the tongue throat ear and tonsils on one side of the head but in rare cases may be bilateral Painful attacks may occur spontaneously but are more often associated with a specific stimulus such as chewing speaking coughing swallowing or laughing The attacks can occur frequently throughout the day or once every couple of weeks and they can also disturb sleep
bull httpwwwcolumbianeurosurgeryorgconditionsglossopharyngeal-neuralgia
Causes of Glossopharyngeal Neuralgiabull Neuralgia is typically caused by compression of the
glossopharyngeal nerve
bull Compression of the nerve by an adjacent artery andor vein can occur near the brainstem
bull Compression of the nerve more distally in the neck by an elongated styloid process (a bone in the neck) can cause this pain syndrome as well and this is called Eagle syndrome
bull Glossopharyngeal neuralgia can be caused by a tumor or infection in the region of ninth cranial nerve or by multiple sclerosis
bull httpwwwcolumbianeurosurgeryorgconditionsglossopharyngeal-neuralgia
Differential diagnosis of Glossopharyngeal Neuritis (IX)
bull The diagnosis is made on clinical grounds alone
bull no specific test can be done to prove the diagnosis in any given patient Episodic ear andor throat pain often triggered by touching the palate or tonsil is strongly suggestive of the disorder
bull High resolution MRI or CT imaging of the brainstem may reveal vascular compression tumors or demyelinating lesions involving the ninth cranial nerve
bull High resolution CT scanning of the neck can reveal an elongated styloid process suggestive of Eagle syndrome
bull Pain that distinctly involves the ear rear 13 of the tongue andor throat is the classic pain distribution seen in glossopharyngeal neuralgia (ninth cranial nerve or IX N)
httpwwwcolumbianeurosurgeryorgconditionsglossopharyngeal-neuralgia
Treatment of Glossopharyngeal Neuritisbull Most treatment for Glossopharyngeal Neuralgia is
pharmacological Anticonvulsant medications including oxcarbazepine carbamazepine gabapentin and pregabalin Some antidepressants can also be helpful either alone or in combination with the seizure medications If medications fail surgical intervention is indicated
bull The first-choice treatment microvascular decompression (MVD) as it has the highest initial and long-term success rates Patients unable to tolerate such an operation may consider one of the many less invasive options including Gamma Knife radiosurgery
bull Eagles syndrome TMJ and Trigeminal neuralgia can co-exist with glossopharyngeal neuritis and mask the diagnosis or be mistaken for it
httpwwwcolumbianeurosurgeryorgconditionsglossopharyngeal-neuralgia
SENSORY DISTRIBUTION
OF THE TRIGEMINAL
NERVE V IS FOR ROMAN
NUMERAL FIVE THERE
ARE THREE SENSORY
BRANCHES V1 or the
ophthalmic branch V2 or
the maxillary branch AND
V3 or the mandibular
branch Notice all the
sensory sensation is in the
jaw cheek and forehead
nose and eye
What is trigeminal neuralgia
bull A second form of trigeminal neuralgia is a herpetic-like viral irritation of the trigeminal nerve (Empting L Hendler N Kolodny L Kraus H Tips on hard to manage pain syndromes Patient Care page 26-46 April 30 1990)
bull Interestingly the pain of trigeminal neuralgia from both sources is clinically the same
bull This is an example of ldquoconvergencerdquo which is a single clinical manifestation due to several etiologies
How do you diagnose trigeminal neuralgia
bull The pain of both types of trigeminal neuralgia can be constant or intermittent and follows the path of the sensory branches of the trigeminal nerve into the forehead along the cheek bone or in the jaw line or any combination of the three (Empting L Hendler N Kolodny L Kraus H Tips on hard to manage pain syndromes Patient Care page 26-46 April 30 1990)
bull The pain is sensitive to light touch and may occur in burst of pain rather than being constant This occurs in both types of trigeminal neuralgia
How do you diagnose trigeminal neuralgia
bull In order to differentiate between the two forms of trigeminal neuralgia an MRI of the brain with vascular enhancement is useful
bull If there is no vessel compression it is highly likely the cause of the pain is post-herpetic
bull In addition to an MRI with vascular enhancement an MRI of the brain with thin-sections (2 mm slices) through the trigeminal nerve and an MRA (Magnet resonance angiography) may be helpful in differentiating the post-viral etiology from the compression of the nerve by the blood vessel
What is the treatment of trigeminal neuralgia
bull Since there are two main etiologies to the pain of trigeminal neuralgia there are two types of treatment
bull If the etiology is compression of the trigeminal nerve by the middle cerebral artery in the skull then the treatment is intra-cranial decompression of the nerve by inserting a sponge between the artery and the nerve (Sekula RF Marchan EM Fletcher LH Casey KF Jannetta PJ Microvascular decompression for trigeminal neuralgia in elderly patients J NeuroNeurosurg2008 Apr108(4)689-91)
What is the treatment of trigeminal neuralgia
bull If the etiology is due to a herpetic-like viral irritation of the trigeminal nerve (Empting L Hendler N Kolodny L Kraus H Tips on hard to manage pain syndromes Patient Care page 26-46 April 30 1990) then the treatment is the use of medications
bull Some people respond to low dose anti-depressants but the most useful medication is an anti-convulsant such as gaba-pentin (Neurontin) or Tegretol
bull Anti-viral drug such as acyclovir will also help
bull Rarely radio-frequency lesions of the retro-gausserian ganglion can be employed if all else fails
What is Eagles syndrome
bull Eagles Syndrome is characterized by an a specific orofacial pain secondary to calcification of the stylohyoid ligament or elongated styoid process In about 4 of general population an elongated styloid process occurs while only about 4 of these patients are symptomatic The stylo-hyoid bone is considered abnormal if it is greater than 25 mm in length (Casale M Rinaldi V Quattrocchi C Bressi F Vincenzi B Santini D Tonini G Salvinelli F Atypical chronic head and neck pain dont forget Eagles syndrome Eur Rev Med Pharmacol Sci 2008 Mar-Apr12(2)131-3)
3D-CT of a very long
stylo-hyoid
bonehellipmuch longer
than 25 mm Follow
the numbers 123
and 4 on both sides of
the 3D-CT and the is
the stylo-hyoid bone
which is about 39 mm
in this 3D-CT The
bone marked H is the
hyoid bone In this
3D-CT the stylo-hyoid
bone is so long that is
spontaneously broke
between 2 and 3 but it
is still symptomatic
and causes pain with
swollowing Removal
of the bone from the
break to 4 on the 3D-
CT will relieve
symptoms
What are the symptoms of Eagles syndrome and how do you diagnosis itbull The patient has a sensation of a foreign body localized at
the tonsillar fossa associated with a dull intermittent painbull Pain is usually worse with swallowingbull A bony projection is usually palpable with bimanual trans-
oral explorationbull Basically you put your finger in the mouth of the patient
down their throat and push towards the outside where the stylohyoid bone is If this reproduces the pain this confirms the diagnosis
bull A lateral radiograph and a computed tomography scan of head and neck will show an elongated styloid process of at least 25 mm (Casale M Rinaldi V Quattrocchi C Bressi F Vincenzi B Santini D Tonini G Salvinelli FAtypical chronic head and neck pain dont forget Eagles syndrome Eur Rev Med Pharmacol Sci 2008 Mar-Apr12(2)131-3)
How do you treat Eagles syndromebull Medical treatment represents the first choice
bull Use non-steroidal anti- inflammatory drugs
bull If this is not effective then surgical resection of the styloid process may help (cutting off the tip)
bull In the case of persistence or ingravescence of the complaint surgery may be the only option
bull Symptoms may overlap with glossopharyngealneuritis (presented later)
bull (Casale M Rinaldi V Quattrocchi C Bressi F Vincenzi B Santini D Tonini G
Salvinelli F Atypical chronic head and neck pain dont forget Eagles syndrome EurRev Med Pharmacol Sci 2008 Mar-Apr12(2)131-3)
What is Sjogrenrsquos Syndrome bull Sjogren syndrome is an autoimmune disorder in
which the glands that produce tears and saliva are destroyed causing dry mouth and dry eyes
bull Symptoms may include itching eyes feeling that something is in the eye difficulty swallowing loss of sense of taste thick saliva mouth sores many cavities in the teeth and pain in the face usually in the trigeminal or facial nerve
bull (Kruszka P OBrian RJ Diagnosis and management of Sjogren syndrome Am Fam Physician 200979465-470)
What are symptoms of Sjogrenrsquos Syndromebull Can be mistaken for trigeminal neuralgia
bull When there is unexplained pain in the face take a careful history Ask about dry mouth itchy eyes lots of cavities trouble swallowing SICCA complex (1)
bull Trigeminal sensory neuropathy can occur with Sjogrenrsquos and may be characterized by progressive sensory complaints on the face They could start on one side and subsequently become bilateral (2)
bull (1) Wu AJ Optimizing dry mouth treatment for individuals with Sjoumlgrens syndrome Rheum Dis Clin North Am 2008 Nov34(4)1001-1010
bull (2) Steven Mandel MD Clinical Professor of Neurology Jefferson Medical College)
Laboratory Tests to Diagnose Sjogrenrsquos
bull SSA and SSB ANA sed rate rheumatoid factor (RF) Schirmer test corneal fluorescein staining conjunctival lissamine green staining tear-film breakup time and lip biopsy are useful methods of diagnosing Sjogrenrsquos but not always reliable
bull Yang Z Liang Y Zhong R Is identification of anti-SSA andor -SSB antibodies necessary in serum samples referred for antinuclear antibodies testing J Clin Lab Anal 2012 Nov26(6)447-51 Br J Ophthalmol 2012 Dec96(12)1498-503 doi with aqueous-deficient dry eye
bull Liew MS Zhang M Kim E Akpek EK Prevalence and predictors of Sjogrens syndrome in a prospective cohort of patients with aqueous-deficient dry eye Br J Ophthalmol 2012 Dec96(12)1498-503
Treatment of Sjogrenrsquosbull Conventional immunosuppressive therapies
such as corticosteroids have been used in some patients with variable benefit
bull Current therapy for dryness is principally symptomatic
bull The greatest experience in pSS is with rituximab an anti-CD20 (expressed on a subset of B-cells) monoclonal antibody
(Bowman S Barone F Biologic treatments in Sjoumlgrens syndrome Presse Med 2012 Sep41(9 Pt 2)e495-509)
Symptoms of Glossopharyngeal Neuritis
bull Glossopharyngeal neuralgia is classically described as ldquostabbingrdquo or ldquoelectrical shock-likerdquo in nature Episodes are typically brief lasting a couple of seconds up to a few minutes and can be quite debilitating They can occur in the tongue throat ear and tonsils on one side of the head but in rare cases may be bilateral Painful attacks may occur spontaneously but are more often associated with a specific stimulus such as chewing speaking coughing swallowing or laughing The attacks can occur frequently throughout the day or once every couple of weeks and they can also disturb sleep
bull httpwwwcolumbianeurosurgeryorgconditionsglossopharyngeal-neuralgia
Causes of Glossopharyngeal Neuralgiabull Neuralgia is typically caused by compression of the
glossopharyngeal nerve
bull Compression of the nerve by an adjacent artery andor vein can occur near the brainstem
bull Compression of the nerve more distally in the neck by an elongated styloid process (a bone in the neck) can cause this pain syndrome as well and this is called Eagle syndrome
bull Glossopharyngeal neuralgia can be caused by a tumor or infection in the region of ninth cranial nerve or by multiple sclerosis
bull httpwwwcolumbianeurosurgeryorgconditionsglossopharyngeal-neuralgia
Differential diagnosis of Glossopharyngeal Neuritis (IX)
bull The diagnosis is made on clinical grounds alone
bull no specific test can be done to prove the diagnosis in any given patient Episodic ear andor throat pain often triggered by touching the palate or tonsil is strongly suggestive of the disorder
bull High resolution MRI or CT imaging of the brainstem may reveal vascular compression tumors or demyelinating lesions involving the ninth cranial nerve
bull High resolution CT scanning of the neck can reveal an elongated styloid process suggestive of Eagle syndrome
bull Pain that distinctly involves the ear rear 13 of the tongue andor throat is the classic pain distribution seen in glossopharyngeal neuralgia (ninth cranial nerve or IX N)
httpwwwcolumbianeurosurgeryorgconditionsglossopharyngeal-neuralgia
Treatment of Glossopharyngeal Neuritisbull Most treatment for Glossopharyngeal Neuralgia is
pharmacological Anticonvulsant medications including oxcarbazepine carbamazepine gabapentin and pregabalin Some antidepressants can also be helpful either alone or in combination with the seizure medications If medications fail surgical intervention is indicated
bull The first-choice treatment microvascular decompression (MVD) as it has the highest initial and long-term success rates Patients unable to tolerate such an operation may consider one of the many less invasive options including Gamma Knife radiosurgery
bull Eagles syndrome TMJ and Trigeminal neuralgia can co-exist with glossopharyngeal neuritis and mask the diagnosis or be mistaken for it
httpwwwcolumbianeurosurgeryorgconditionsglossopharyngeal-neuralgia
What is trigeminal neuralgia
bull A second form of trigeminal neuralgia is a herpetic-like viral irritation of the trigeminal nerve (Empting L Hendler N Kolodny L Kraus H Tips on hard to manage pain syndromes Patient Care page 26-46 April 30 1990)
bull Interestingly the pain of trigeminal neuralgia from both sources is clinically the same
bull This is an example of ldquoconvergencerdquo which is a single clinical manifestation due to several etiologies
How do you diagnose trigeminal neuralgia
bull The pain of both types of trigeminal neuralgia can be constant or intermittent and follows the path of the sensory branches of the trigeminal nerve into the forehead along the cheek bone or in the jaw line or any combination of the three (Empting L Hendler N Kolodny L Kraus H Tips on hard to manage pain syndromes Patient Care page 26-46 April 30 1990)
bull The pain is sensitive to light touch and may occur in burst of pain rather than being constant This occurs in both types of trigeminal neuralgia
How do you diagnose trigeminal neuralgia
bull In order to differentiate between the two forms of trigeminal neuralgia an MRI of the brain with vascular enhancement is useful
bull If there is no vessel compression it is highly likely the cause of the pain is post-herpetic
bull In addition to an MRI with vascular enhancement an MRI of the brain with thin-sections (2 mm slices) through the trigeminal nerve and an MRA (Magnet resonance angiography) may be helpful in differentiating the post-viral etiology from the compression of the nerve by the blood vessel
What is the treatment of trigeminal neuralgia
bull Since there are two main etiologies to the pain of trigeminal neuralgia there are two types of treatment
bull If the etiology is compression of the trigeminal nerve by the middle cerebral artery in the skull then the treatment is intra-cranial decompression of the nerve by inserting a sponge between the artery and the nerve (Sekula RF Marchan EM Fletcher LH Casey KF Jannetta PJ Microvascular decompression for trigeminal neuralgia in elderly patients J NeuroNeurosurg2008 Apr108(4)689-91)
What is the treatment of trigeminal neuralgia
bull If the etiology is due to a herpetic-like viral irritation of the trigeminal nerve (Empting L Hendler N Kolodny L Kraus H Tips on hard to manage pain syndromes Patient Care page 26-46 April 30 1990) then the treatment is the use of medications
bull Some people respond to low dose anti-depressants but the most useful medication is an anti-convulsant such as gaba-pentin (Neurontin) or Tegretol
bull Anti-viral drug such as acyclovir will also help
bull Rarely radio-frequency lesions of the retro-gausserian ganglion can be employed if all else fails
What is Eagles syndrome
bull Eagles Syndrome is characterized by an a specific orofacial pain secondary to calcification of the stylohyoid ligament or elongated styoid process In about 4 of general population an elongated styloid process occurs while only about 4 of these patients are symptomatic The stylo-hyoid bone is considered abnormal if it is greater than 25 mm in length (Casale M Rinaldi V Quattrocchi C Bressi F Vincenzi B Santini D Tonini G Salvinelli F Atypical chronic head and neck pain dont forget Eagles syndrome Eur Rev Med Pharmacol Sci 2008 Mar-Apr12(2)131-3)
3D-CT of a very long
stylo-hyoid
bonehellipmuch longer
than 25 mm Follow
the numbers 123
and 4 on both sides of
the 3D-CT and the is
the stylo-hyoid bone
which is about 39 mm
in this 3D-CT The
bone marked H is the
hyoid bone In this
3D-CT the stylo-hyoid
bone is so long that is
spontaneously broke
between 2 and 3 but it
is still symptomatic
and causes pain with
swollowing Removal
of the bone from the
break to 4 on the 3D-
CT will relieve
symptoms
What are the symptoms of Eagles syndrome and how do you diagnosis itbull The patient has a sensation of a foreign body localized at
the tonsillar fossa associated with a dull intermittent painbull Pain is usually worse with swallowingbull A bony projection is usually palpable with bimanual trans-
oral explorationbull Basically you put your finger in the mouth of the patient
down their throat and push towards the outside where the stylohyoid bone is If this reproduces the pain this confirms the diagnosis
bull A lateral radiograph and a computed tomography scan of head and neck will show an elongated styloid process of at least 25 mm (Casale M Rinaldi V Quattrocchi C Bressi F Vincenzi B Santini D Tonini G Salvinelli FAtypical chronic head and neck pain dont forget Eagles syndrome Eur Rev Med Pharmacol Sci 2008 Mar-Apr12(2)131-3)
How do you treat Eagles syndromebull Medical treatment represents the first choice
bull Use non-steroidal anti- inflammatory drugs
bull If this is not effective then surgical resection of the styloid process may help (cutting off the tip)
bull In the case of persistence or ingravescence of the complaint surgery may be the only option
bull Symptoms may overlap with glossopharyngealneuritis (presented later)
bull (Casale M Rinaldi V Quattrocchi C Bressi F Vincenzi B Santini D Tonini G
Salvinelli F Atypical chronic head and neck pain dont forget Eagles syndrome EurRev Med Pharmacol Sci 2008 Mar-Apr12(2)131-3)
What is Sjogrenrsquos Syndrome bull Sjogren syndrome is an autoimmune disorder in
which the glands that produce tears and saliva are destroyed causing dry mouth and dry eyes
bull Symptoms may include itching eyes feeling that something is in the eye difficulty swallowing loss of sense of taste thick saliva mouth sores many cavities in the teeth and pain in the face usually in the trigeminal or facial nerve
bull (Kruszka P OBrian RJ Diagnosis and management of Sjogren syndrome Am Fam Physician 200979465-470)
What are symptoms of Sjogrenrsquos Syndromebull Can be mistaken for trigeminal neuralgia
bull When there is unexplained pain in the face take a careful history Ask about dry mouth itchy eyes lots of cavities trouble swallowing SICCA complex (1)
bull Trigeminal sensory neuropathy can occur with Sjogrenrsquos and may be characterized by progressive sensory complaints on the face They could start on one side and subsequently become bilateral (2)
bull (1) Wu AJ Optimizing dry mouth treatment for individuals with Sjoumlgrens syndrome Rheum Dis Clin North Am 2008 Nov34(4)1001-1010
bull (2) Steven Mandel MD Clinical Professor of Neurology Jefferson Medical College)
Laboratory Tests to Diagnose Sjogrenrsquos
bull SSA and SSB ANA sed rate rheumatoid factor (RF) Schirmer test corneal fluorescein staining conjunctival lissamine green staining tear-film breakup time and lip biopsy are useful methods of diagnosing Sjogrenrsquos but not always reliable
bull Yang Z Liang Y Zhong R Is identification of anti-SSA andor -SSB antibodies necessary in serum samples referred for antinuclear antibodies testing J Clin Lab Anal 2012 Nov26(6)447-51 Br J Ophthalmol 2012 Dec96(12)1498-503 doi with aqueous-deficient dry eye
bull Liew MS Zhang M Kim E Akpek EK Prevalence and predictors of Sjogrens syndrome in a prospective cohort of patients with aqueous-deficient dry eye Br J Ophthalmol 2012 Dec96(12)1498-503
Treatment of Sjogrenrsquosbull Conventional immunosuppressive therapies
such as corticosteroids have been used in some patients with variable benefit
bull Current therapy for dryness is principally symptomatic
bull The greatest experience in pSS is with rituximab an anti-CD20 (expressed on a subset of B-cells) monoclonal antibody
(Bowman S Barone F Biologic treatments in Sjoumlgrens syndrome Presse Med 2012 Sep41(9 Pt 2)e495-509)
Symptoms of Glossopharyngeal Neuritis
bull Glossopharyngeal neuralgia is classically described as ldquostabbingrdquo or ldquoelectrical shock-likerdquo in nature Episodes are typically brief lasting a couple of seconds up to a few minutes and can be quite debilitating They can occur in the tongue throat ear and tonsils on one side of the head but in rare cases may be bilateral Painful attacks may occur spontaneously but are more often associated with a specific stimulus such as chewing speaking coughing swallowing or laughing The attacks can occur frequently throughout the day or once every couple of weeks and they can also disturb sleep
bull httpwwwcolumbianeurosurgeryorgconditionsglossopharyngeal-neuralgia
Causes of Glossopharyngeal Neuralgiabull Neuralgia is typically caused by compression of the
glossopharyngeal nerve
bull Compression of the nerve by an adjacent artery andor vein can occur near the brainstem
bull Compression of the nerve more distally in the neck by an elongated styloid process (a bone in the neck) can cause this pain syndrome as well and this is called Eagle syndrome
bull Glossopharyngeal neuralgia can be caused by a tumor or infection in the region of ninth cranial nerve or by multiple sclerosis
bull httpwwwcolumbianeurosurgeryorgconditionsglossopharyngeal-neuralgia
Differential diagnosis of Glossopharyngeal Neuritis (IX)
bull The diagnosis is made on clinical grounds alone
bull no specific test can be done to prove the diagnosis in any given patient Episodic ear andor throat pain often triggered by touching the palate or tonsil is strongly suggestive of the disorder
bull High resolution MRI or CT imaging of the brainstem may reveal vascular compression tumors or demyelinating lesions involving the ninth cranial nerve
bull High resolution CT scanning of the neck can reveal an elongated styloid process suggestive of Eagle syndrome
bull Pain that distinctly involves the ear rear 13 of the tongue andor throat is the classic pain distribution seen in glossopharyngeal neuralgia (ninth cranial nerve or IX N)
httpwwwcolumbianeurosurgeryorgconditionsglossopharyngeal-neuralgia
Treatment of Glossopharyngeal Neuritisbull Most treatment for Glossopharyngeal Neuralgia is
pharmacological Anticonvulsant medications including oxcarbazepine carbamazepine gabapentin and pregabalin Some antidepressants can also be helpful either alone or in combination with the seizure medications If medications fail surgical intervention is indicated
bull The first-choice treatment microvascular decompression (MVD) as it has the highest initial and long-term success rates Patients unable to tolerate such an operation may consider one of the many less invasive options including Gamma Knife radiosurgery
bull Eagles syndrome TMJ and Trigeminal neuralgia can co-exist with glossopharyngeal neuritis and mask the diagnosis or be mistaken for it
httpwwwcolumbianeurosurgeryorgconditionsglossopharyngeal-neuralgia
How do you diagnose trigeminal neuralgia
bull The pain of both types of trigeminal neuralgia can be constant or intermittent and follows the path of the sensory branches of the trigeminal nerve into the forehead along the cheek bone or in the jaw line or any combination of the three (Empting L Hendler N Kolodny L Kraus H Tips on hard to manage pain syndromes Patient Care page 26-46 April 30 1990)
bull The pain is sensitive to light touch and may occur in burst of pain rather than being constant This occurs in both types of trigeminal neuralgia
How do you diagnose trigeminal neuralgia
bull In order to differentiate between the two forms of trigeminal neuralgia an MRI of the brain with vascular enhancement is useful
bull If there is no vessel compression it is highly likely the cause of the pain is post-herpetic
bull In addition to an MRI with vascular enhancement an MRI of the brain with thin-sections (2 mm slices) through the trigeminal nerve and an MRA (Magnet resonance angiography) may be helpful in differentiating the post-viral etiology from the compression of the nerve by the blood vessel
What is the treatment of trigeminal neuralgia
bull Since there are two main etiologies to the pain of trigeminal neuralgia there are two types of treatment
bull If the etiology is compression of the trigeminal nerve by the middle cerebral artery in the skull then the treatment is intra-cranial decompression of the nerve by inserting a sponge between the artery and the nerve (Sekula RF Marchan EM Fletcher LH Casey KF Jannetta PJ Microvascular decompression for trigeminal neuralgia in elderly patients J NeuroNeurosurg2008 Apr108(4)689-91)
What is the treatment of trigeminal neuralgia
bull If the etiology is due to a herpetic-like viral irritation of the trigeminal nerve (Empting L Hendler N Kolodny L Kraus H Tips on hard to manage pain syndromes Patient Care page 26-46 April 30 1990) then the treatment is the use of medications
bull Some people respond to low dose anti-depressants but the most useful medication is an anti-convulsant such as gaba-pentin (Neurontin) or Tegretol
bull Anti-viral drug such as acyclovir will also help
bull Rarely radio-frequency lesions of the retro-gausserian ganglion can be employed if all else fails
What is Eagles syndrome
bull Eagles Syndrome is characterized by an a specific orofacial pain secondary to calcification of the stylohyoid ligament or elongated styoid process In about 4 of general population an elongated styloid process occurs while only about 4 of these patients are symptomatic The stylo-hyoid bone is considered abnormal if it is greater than 25 mm in length (Casale M Rinaldi V Quattrocchi C Bressi F Vincenzi B Santini D Tonini G Salvinelli F Atypical chronic head and neck pain dont forget Eagles syndrome Eur Rev Med Pharmacol Sci 2008 Mar-Apr12(2)131-3)
3D-CT of a very long
stylo-hyoid
bonehellipmuch longer
than 25 mm Follow
the numbers 123
and 4 on both sides of
the 3D-CT and the is
the stylo-hyoid bone
which is about 39 mm
in this 3D-CT The
bone marked H is the
hyoid bone In this
3D-CT the stylo-hyoid
bone is so long that is
spontaneously broke
between 2 and 3 but it
is still symptomatic
and causes pain with
swollowing Removal
of the bone from the
break to 4 on the 3D-
CT will relieve
symptoms
What are the symptoms of Eagles syndrome and how do you diagnosis itbull The patient has a sensation of a foreign body localized at
the tonsillar fossa associated with a dull intermittent painbull Pain is usually worse with swallowingbull A bony projection is usually palpable with bimanual trans-
oral explorationbull Basically you put your finger in the mouth of the patient
down their throat and push towards the outside where the stylohyoid bone is If this reproduces the pain this confirms the diagnosis
bull A lateral radiograph and a computed tomography scan of head and neck will show an elongated styloid process of at least 25 mm (Casale M Rinaldi V Quattrocchi C Bressi F Vincenzi B Santini D Tonini G Salvinelli FAtypical chronic head and neck pain dont forget Eagles syndrome Eur Rev Med Pharmacol Sci 2008 Mar-Apr12(2)131-3)
How do you treat Eagles syndromebull Medical treatment represents the first choice
bull Use non-steroidal anti- inflammatory drugs
bull If this is not effective then surgical resection of the styloid process may help (cutting off the tip)
bull In the case of persistence or ingravescence of the complaint surgery may be the only option
bull Symptoms may overlap with glossopharyngealneuritis (presented later)
bull (Casale M Rinaldi V Quattrocchi C Bressi F Vincenzi B Santini D Tonini G
Salvinelli F Atypical chronic head and neck pain dont forget Eagles syndrome EurRev Med Pharmacol Sci 2008 Mar-Apr12(2)131-3)
What is Sjogrenrsquos Syndrome bull Sjogren syndrome is an autoimmune disorder in
which the glands that produce tears and saliva are destroyed causing dry mouth and dry eyes
bull Symptoms may include itching eyes feeling that something is in the eye difficulty swallowing loss of sense of taste thick saliva mouth sores many cavities in the teeth and pain in the face usually in the trigeminal or facial nerve
bull (Kruszka P OBrian RJ Diagnosis and management of Sjogren syndrome Am Fam Physician 200979465-470)
What are symptoms of Sjogrenrsquos Syndromebull Can be mistaken for trigeminal neuralgia
bull When there is unexplained pain in the face take a careful history Ask about dry mouth itchy eyes lots of cavities trouble swallowing SICCA complex (1)
bull Trigeminal sensory neuropathy can occur with Sjogrenrsquos and may be characterized by progressive sensory complaints on the face They could start on one side and subsequently become bilateral (2)
bull (1) Wu AJ Optimizing dry mouth treatment for individuals with Sjoumlgrens syndrome Rheum Dis Clin North Am 2008 Nov34(4)1001-1010
bull (2) Steven Mandel MD Clinical Professor of Neurology Jefferson Medical College)
Laboratory Tests to Diagnose Sjogrenrsquos
bull SSA and SSB ANA sed rate rheumatoid factor (RF) Schirmer test corneal fluorescein staining conjunctival lissamine green staining tear-film breakup time and lip biopsy are useful methods of diagnosing Sjogrenrsquos but not always reliable
bull Yang Z Liang Y Zhong R Is identification of anti-SSA andor -SSB antibodies necessary in serum samples referred for antinuclear antibodies testing J Clin Lab Anal 2012 Nov26(6)447-51 Br J Ophthalmol 2012 Dec96(12)1498-503 doi with aqueous-deficient dry eye
bull Liew MS Zhang M Kim E Akpek EK Prevalence and predictors of Sjogrens syndrome in a prospective cohort of patients with aqueous-deficient dry eye Br J Ophthalmol 2012 Dec96(12)1498-503
Treatment of Sjogrenrsquosbull Conventional immunosuppressive therapies
such as corticosteroids have been used in some patients with variable benefit
bull Current therapy for dryness is principally symptomatic
bull The greatest experience in pSS is with rituximab an anti-CD20 (expressed on a subset of B-cells) monoclonal antibody
(Bowman S Barone F Biologic treatments in Sjoumlgrens syndrome Presse Med 2012 Sep41(9 Pt 2)e495-509)
Symptoms of Glossopharyngeal Neuritis
bull Glossopharyngeal neuralgia is classically described as ldquostabbingrdquo or ldquoelectrical shock-likerdquo in nature Episodes are typically brief lasting a couple of seconds up to a few minutes and can be quite debilitating They can occur in the tongue throat ear and tonsils on one side of the head but in rare cases may be bilateral Painful attacks may occur spontaneously but are more often associated with a specific stimulus such as chewing speaking coughing swallowing or laughing The attacks can occur frequently throughout the day or once every couple of weeks and they can also disturb sleep
bull httpwwwcolumbianeurosurgeryorgconditionsglossopharyngeal-neuralgia
Causes of Glossopharyngeal Neuralgiabull Neuralgia is typically caused by compression of the
glossopharyngeal nerve
bull Compression of the nerve by an adjacent artery andor vein can occur near the brainstem
bull Compression of the nerve more distally in the neck by an elongated styloid process (a bone in the neck) can cause this pain syndrome as well and this is called Eagle syndrome
bull Glossopharyngeal neuralgia can be caused by a tumor or infection in the region of ninth cranial nerve or by multiple sclerosis
bull httpwwwcolumbianeurosurgeryorgconditionsglossopharyngeal-neuralgia
Differential diagnosis of Glossopharyngeal Neuritis (IX)
bull The diagnosis is made on clinical grounds alone
bull no specific test can be done to prove the diagnosis in any given patient Episodic ear andor throat pain often triggered by touching the palate or tonsil is strongly suggestive of the disorder
bull High resolution MRI or CT imaging of the brainstem may reveal vascular compression tumors or demyelinating lesions involving the ninth cranial nerve
bull High resolution CT scanning of the neck can reveal an elongated styloid process suggestive of Eagle syndrome
bull Pain that distinctly involves the ear rear 13 of the tongue andor throat is the classic pain distribution seen in glossopharyngeal neuralgia (ninth cranial nerve or IX N)
httpwwwcolumbianeurosurgeryorgconditionsglossopharyngeal-neuralgia
Treatment of Glossopharyngeal Neuritisbull Most treatment for Glossopharyngeal Neuralgia is
pharmacological Anticonvulsant medications including oxcarbazepine carbamazepine gabapentin and pregabalin Some antidepressants can also be helpful either alone or in combination with the seizure medications If medications fail surgical intervention is indicated
bull The first-choice treatment microvascular decompression (MVD) as it has the highest initial and long-term success rates Patients unable to tolerate such an operation may consider one of the many less invasive options including Gamma Knife radiosurgery
bull Eagles syndrome TMJ and Trigeminal neuralgia can co-exist with glossopharyngeal neuritis and mask the diagnosis or be mistaken for it
httpwwwcolumbianeurosurgeryorgconditionsglossopharyngeal-neuralgia
How do you diagnose trigeminal neuralgia
bull In order to differentiate between the two forms of trigeminal neuralgia an MRI of the brain with vascular enhancement is useful
bull If there is no vessel compression it is highly likely the cause of the pain is post-herpetic
bull In addition to an MRI with vascular enhancement an MRI of the brain with thin-sections (2 mm slices) through the trigeminal nerve and an MRA (Magnet resonance angiography) may be helpful in differentiating the post-viral etiology from the compression of the nerve by the blood vessel
What is the treatment of trigeminal neuralgia
bull Since there are two main etiologies to the pain of trigeminal neuralgia there are two types of treatment
bull If the etiology is compression of the trigeminal nerve by the middle cerebral artery in the skull then the treatment is intra-cranial decompression of the nerve by inserting a sponge between the artery and the nerve (Sekula RF Marchan EM Fletcher LH Casey KF Jannetta PJ Microvascular decompression for trigeminal neuralgia in elderly patients J NeuroNeurosurg2008 Apr108(4)689-91)
What is the treatment of trigeminal neuralgia
bull If the etiology is due to a herpetic-like viral irritation of the trigeminal nerve (Empting L Hendler N Kolodny L Kraus H Tips on hard to manage pain syndromes Patient Care page 26-46 April 30 1990) then the treatment is the use of medications
bull Some people respond to low dose anti-depressants but the most useful medication is an anti-convulsant such as gaba-pentin (Neurontin) or Tegretol
bull Anti-viral drug such as acyclovir will also help
bull Rarely radio-frequency lesions of the retro-gausserian ganglion can be employed if all else fails
What is Eagles syndrome
bull Eagles Syndrome is characterized by an a specific orofacial pain secondary to calcification of the stylohyoid ligament or elongated styoid process In about 4 of general population an elongated styloid process occurs while only about 4 of these patients are symptomatic The stylo-hyoid bone is considered abnormal if it is greater than 25 mm in length (Casale M Rinaldi V Quattrocchi C Bressi F Vincenzi B Santini D Tonini G Salvinelli F Atypical chronic head and neck pain dont forget Eagles syndrome Eur Rev Med Pharmacol Sci 2008 Mar-Apr12(2)131-3)
3D-CT of a very long
stylo-hyoid
bonehellipmuch longer
than 25 mm Follow
the numbers 123
and 4 on both sides of
the 3D-CT and the is
the stylo-hyoid bone
which is about 39 mm
in this 3D-CT The
bone marked H is the
hyoid bone In this
3D-CT the stylo-hyoid
bone is so long that is
spontaneously broke
between 2 and 3 but it
is still symptomatic
and causes pain with
swollowing Removal
of the bone from the
break to 4 on the 3D-
CT will relieve
symptoms
What are the symptoms of Eagles syndrome and how do you diagnosis itbull The patient has a sensation of a foreign body localized at
the tonsillar fossa associated with a dull intermittent painbull Pain is usually worse with swallowingbull A bony projection is usually palpable with bimanual trans-
oral explorationbull Basically you put your finger in the mouth of the patient
down their throat and push towards the outside where the stylohyoid bone is If this reproduces the pain this confirms the diagnosis
bull A lateral radiograph and a computed tomography scan of head and neck will show an elongated styloid process of at least 25 mm (Casale M Rinaldi V Quattrocchi C Bressi F Vincenzi B Santini D Tonini G Salvinelli FAtypical chronic head and neck pain dont forget Eagles syndrome Eur Rev Med Pharmacol Sci 2008 Mar-Apr12(2)131-3)
How do you treat Eagles syndromebull Medical treatment represents the first choice
bull Use non-steroidal anti- inflammatory drugs
bull If this is not effective then surgical resection of the styloid process may help (cutting off the tip)
bull In the case of persistence or ingravescence of the complaint surgery may be the only option
bull Symptoms may overlap with glossopharyngealneuritis (presented later)
bull (Casale M Rinaldi V Quattrocchi C Bressi F Vincenzi B Santini D Tonini G
Salvinelli F Atypical chronic head and neck pain dont forget Eagles syndrome EurRev Med Pharmacol Sci 2008 Mar-Apr12(2)131-3)
What is Sjogrenrsquos Syndrome bull Sjogren syndrome is an autoimmune disorder in
which the glands that produce tears and saliva are destroyed causing dry mouth and dry eyes
bull Symptoms may include itching eyes feeling that something is in the eye difficulty swallowing loss of sense of taste thick saliva mouth sores many cavities in the teeth and pain in the face usually in the trigeminal or facial nerve
bull (Kruszka P OBrian RJ Diagnosis and management of Sjogren syndrome Am Fam Physician 200979465-470)
What are symptoms of Sjogrenrsquos Syndromebull Can be mistaken for trigeminal neuralgia
bull When there is unexplained pain in the face take a careful history Ask about dry mouth itchy eyes lots of cavities trouble swallowing SICCA complex (1)
bull Trigeminal sensory neuropathy can occur with Sjogrenrsquos and may be characterized by progressive sensory complaints on the face They could start on one side and subsequently become bilateral (2)
bull (1) Wu AJ Optimizing dry mouth treatment for individuals with Sjoumlgrens syndrome Rheum Dis Clin North Am 2008 Nov34(4)1001-1010
bull (2) Steven Mandel MD Clinical Professor of Neurology Jefferson Medical College)
Laboratory Tests to Diagnose Sjogrenrsquos
bull SSA and SSB ANA sed rate rheumatoid factor (RF) Schirmer test corneal fluorescein staining conjunctival lissamine green staining tear-film breakup time and lip biopsy are useful methods of diagnosing Sjogrenrsquos but not always reliable
bull Yang Z Liang Y Zhong R Is identification of anti-SSA andor -SSB antibodies necessary in serum samples referred for antinuclear antibodies testing J Clin Lab Anal 2012 Nov26(6)447-51 Br J Ophthalmol 2012 Dec96(12)1498-503 doi with aqueous-deficient dry eye
bull Liew MS Zhang M Kim E Akpek EK Prevalence and predictors of Sjogrens syndrome in a prospective cohort of patients with aqueous-deficient dry eye Br J Ophthalmol 2012 Dec96(12)1498-503
Treatment of Sjogrenrsquosbull Conventional immunosuppressive therapies
such as corticosteroids have been used in some patients with variable benefit
bull Current therapy for dryness is principally symptomatic
bull The greatest experience in pSS is with rituximab an anti-CD20 (expressed on a subset of B-cells) monoclonal antibody
(Bowman S Barone F Biologic treatments in Sjoumlgrens syndrome Presse Med 2012 Sep41(9 Pt 2)e495-509)
Symptoms of Glossopharyngeal Neuritis
bull Glossopharyngeal neuralgia is classically described as ldquostabbingrdquo or ldquoelectrical shock-likerdquo in nature Episodes are typically brief lasting a couple of seconds up to a few minutes and can be quite debilitating They can occur in the tongue throat ear and tonsils on one side of the head but in rare cases may be bilateral Painful attacks may occur spontaneously but are more often associated with a specific stimulus such as chewing speaking coughing swallowing or laughing The attacks can occur frequently throughout the day or once every couple of weeks and they can also disturb sleep
bull httpwwwcolumbianeurosurgeryorgconditionsglossopharyngeal-neuralgia
Causes of Glossopharyngeal Neuralgiabull Neuralgia is typically caused by compression of the
glossopharyngeal nerve
bull Compression of the nerve by an adjacent artery andor vein can occur near the brainstem
bull Compression of the nerve more distally in the neck by an elongated styloid process (a bone in the neck) can cause this pain syndrome as well and this is called Eagle syndrome
bull Glossopharyngeal neuralgia can be caused by a tumor or infection in the region of ninth cranial nerve or by multiple sclerosis
bull httpwwwcolumbianeurosurgeryorgconditionsglossopharyngeal-neuralgia
Differential diagnosis of Glossopharyngeal Neuritis (IX)
bull The diagnosis is made on clinical grounds alone
bull no specific test can be done to prove the diagnosis in any given patient Episodic ear andor throat pain often triggered by touching the palate or tonsil is strongly suggestive of the disorder
bull High resolution MRI or CT imaging of the brainstem may reveal vascular compression tumors or demyelinating lesions involving the ninth cranial nerve
bull High resolution CT scanning of the neck can reveal an elongated styloid process suggestive of Eagle syndrome
bull Pain that distinctly involves the ear rear 13 of the tongue andor throat is the classic pain distribution seen in glossopharyngeal neuralgia (ninth cranial nerve or IX N)
httpwwwcolumbianeurosurgeryorgconditionsglossopharyngeal-neuralgia
Treatment of Glossopharyngeal Neuritisbull Most treatment for Glossopharyngeal Neuralgia is
pharmacological Anticonvulsant medications including oxcarbazepine carbamazepine gabapentin and pregabalin Some antidepressants can also be helpful either alone or in combination with the seizure medications If medications fail surgical intervention is indicated
bull The first-choice treatment microvascular decompression (MVD) as it has the highest initial and long-term success rates Patients unable to tolerate such an operation may consider one of the many less invasive options including Gamma Knife radiosurgery
bull Eagles syndrome TMJ and Trigeminal neuralgia can co-exist with glossopharyngeal neuritis and mask the diagnosis or be mistaken for it
httpwwwcolumbianeurosurgeryorgconditionsglossopharyngeal-neuralgia
What is the treatment of trigeminal neuralgia
bull Since there are two main etiologies to the pain of trigeminal neuralgia there are two types of treatment
bull If the etiology is compression of the trigeminal nerve by the middle cerebral artery in the skull then the treatment is intra-cranial decompression of the nerve by inserting a sponge between the artery and the nerve (Sekula RF Marchan EM Fletcher LH Casey KF Jannetta PJ Microvascular decompression for trigeminal neuralgia in elderly patients J NeuroNeurosurg2008 Apr108(4)689-91)
What is the treatment of trigeminal neuralgia
bull If the etiology is due to a herpetic-like viral irritation of the trigeminal nerve (Empting L Hendler N Kolodny L Kraus H Tips on hard to manage pain syndromes Patient Care page 26-46 April 30 1990) then the treatment is the use of medications
bull Some people respond to low dose anti-depressants but the most useful medication is an anti-convulsant such as gaba-pentin (Neurontin) or Tegretol
bull Anti-viral drug such as acyclovir will also help
bull Rarely radio-frequency lesions of the retro-gausserian ganglion can be employed if all else fails
What is Eagles syndrome
bull Eagles Syndrome is characterized by an a specific orofacial pain secondary to calcification of the stylohyoid ligament or elongated styoid process In about 4 of general population an elongated styloid process occurs while only about 4 of these patients are symptomatic The stylo-hyoid bone is considered abnormal if it is greater than 25 mm in length (Casale M Rinaldi V Quattrocchi C Bressi F Vincenzi B Santini D Tonini G Salvinelli F Atypical chronic head and neck pain dont forget Eagles syndrome Eur Rev Med Pharmacol Sci 2008 Mar-Apr12(2)131-3)
3D-CT of a very long
stylo-hyoid
bonehellipmuch longer
than 25 mm Follow
the numbers 123
and 4 on both sides of
the 3D-CT and the is
the stylo-hyoid bone
which is about 39 mm
in this 3D-CT The
bone marked H is the
hyoid bone In this
3D-CT the stylo-hyoid
bone is so long that is
spontaneously broke
between 2 and 3 but it
is still symptomatic
and causes pain with
swollowing Removal
of the bone from the
break to 4 on the 3D-
CT will relieve
symptoms
What are the symptoms of Eagles syndrome and how do you diagnosis itbull The patient has a sensation of a foreign body localized at
the tonsillar fossa associated with a dull intermittent painbull Pain is usually worse with swallowingbull A bony projection is usually palpable with bimanual trans-
oral explorationbull Basically you put your finger in the mouth of the patient
down their throat and push towards the outside where the stylohyoid bone is If this reproduces the pain this confirms the diagnosis
bull A lateral radiograph and a computed tomography scan of head and neck will show an elongated styloid process of at least 25 mm (Casale M Rinaldi V Quattrocchi C Bressi F Vincenzi B Santini D Tonini G Salvinelli FAtypical chronic head and neck pain dont forget Eagles syndrome Eur Rev Med Pharmacol Sci 2008 Mar-Apr12(2)131-3)
How do you treat Eagles syndromebull Medical treatment represents the first choice
bull Use non-steroidal anti- inflammatory drugs
bull If this is not effective then surgical resection of the styloid process may help (cutting off the tip)
bull In the case of persistence or ingravescence of the complaint surgery may be the only option
bull Symptoms may overlap with glossopharyngealneuritis (presented later)
bull (Casale M Rinaldi V Quattrocchi C Bressi F Vincenzi B Santini D Tonini G
Salvinelli F Atypical chronic head and neck pain dont forget Eagles syndrome EurRev Med Pharmacol Sci 2008 Mar-Apr12(2)131-3)
What is Sjogrenrsquos Syndrome bull Sjogren syndrome is an autoimmune disorder in
which the glands that produce tears and saliva are destroyed causing dry mouth and dry eyes
bull Symptoms may include itching eyes feeling that something is in the eye difficulty swallowing loss of sense of taste thick saliva mouth sores many cavities in the teeth and pain in the face usually in the trigeminal or facial nerve
bull (Kruszka P OBrian RJ Diagnosis and management of Sjogren syndrome Am Fam Physician 200979465-470)
What are symptoms of Sjogrenrsquos Syndromebull Can be mistaken for trigeminal neuralgia
bull When there is unexplained pain in the face take a careful history Ask about dry mouth itchy eyes lots of cavities trouble swallowing SICCA complex (1)
bull Trigeminal sensory neuropathy can occur with Sjogrenrsquos and may be characterized by progressive sensory complaints on the face They could start on one side and subsequently become bilateral (2)
bull (1) Wu AJ Optimizing dry mouth treatment for individuals with Sjoumlgrens syndrome Rheum Dis Clin North Am 2008 Nov34(4)1001-1010
bull (2) Steven Mandel MD Clinical Professor of Neurology Jefferson Medical College)
Laboratory Tests to Diagnose Sjogrenrsquos
bull SSA and SSB ANA sed rate rheumatoid factor (RF) Schirmer test corneal fluorescein staining conjunctival lissamine green staining tear-film breakup time and lip biopsy are useful methods of diagnosing Sjogrenrsquos but not always reliable
bull Yang Z Liang Y Zhong R Is identification of anti-SSA andor -SSB antibodies necessary in serum samples referred for antinuclear antibodies testing J Clin Lab Anal 2012 Nov26(6)447-51 Br J Ophthalmol 2012 Dec96(12)1498-503 doi with aqueous-deficient dry eye
bull Liew MS Zhang M Kim E Akpek EK Prevalence and predictors of Sjogrens syndrome in a prospective cohort of patients with aqueous-deficient dry eye Br J Ophthalmol 2012 Dec96(12)1498-503
Treatment of Sjogrenrsquosbull Conventional immunosuppressive therapies
such as corticosteroids have been used in some patients with variable benefit
bull Current therapy for dryness is principally symptomatic
bull The greatest experience in pSS is with rituximab an anti-CD20 (expressed on a subset of B-cells) monoclonal antibody
(Bowman S Barone F Biologic treatments in Sjoumlgrens syndrome Presse Med 2012 Sep41(9 Pt 2)e495-509)
Symptoms of Glossopharyngeal Neuritis
bull Glossopharyngeal neuralgia is classically described as ldquostabbingrdquo or ldquoelectrical shock-likerdquo in nature Episodes are typically brief lasting a couple of seconds up to a few minutes and can be quite debilitating They can occur in the tongue throat ear and tonsils on one side of the head but in rare cases may be bilateral Painful attacks may occur spontaneously but are more often associated with a specific stimulus such as chewing speaking coughing swallowing or laughing The attacks can occur frequently throughout the day or once every couple of weeks and they can also disturb sleep
bull httpwwwcolumbianeurosurgeryorgconditionsglossopharyngeal-neuralgia
Causes of Glossopharyngeal Neuralgiabull Neuralgia is typically caused by compression of the
glossopharyngeal nerve
bull Compression of the nerve by an adjacent artery andor vein can occur near the brainstem
bull Compression of the nerve more distally in the neck by an elongated styloid process (a bone in the neck) can cause this pain syndrome as well and this is called Eagle syndrome
bull Glossopharyngeal neuralgia can be caused by a tumor or infection in the region of ninth cranial nerve or by multiple sclerosis
bull httpwwwcolumbianeurosurgeryorgconditionsglossopharyngeal-neuralgia
Differential diagnosis of Glossopharyngeal Neuritis (IX)
bull The diagnosis is made on clinical grounds alone
bull no specific test can be done to prove the diagnosis in any given patient Episodic ear andor throat pain often triggered by touching the palate or tonsil is strongly suggestive of the disorder
bull High resolution MRI or CT imaging of the brainstem may reveal vascular compression tumors or demyelinating lesions involving the ninth cranial nerve
bull High resolution CT scanning of the neck can reveal an elongated styloid process suggestive of Eagle syndrome
bull Pain that distinctly involves the ear rear 13 of the tongue andor throat is the classic pain distribution seen in glossopharyngeal neuralgia (ninth cranial nerve or IX N)
httpwwwcolumbianeurosurgeryorgconditionsglossopharyngeal-neuralgia
Treatment of Glossopharyngeal Neuritisbull Most treatment for Glossopharyngeal Neuralgia is
pharmacological Anticonvulsant medications including oxcarbazepine carbamazepine gabapentin and pregabalin Some antidepressants can also be helpful either alone or in combination with the seizure medications If medications fail surgical intervention is indicated
bull The first-choice treatment microvascular decompression (MVD) as it has the highest initial and long-term success rates Patients unable to tolerate such an operation may consider one of the many less invasive options including Gamma Knife radiosurgery
bull Eagles syndrome TMJ and Trigeminal neuralgia can co-exist with glossopharyngeal neuritis and mask the diagnosis or be mistaken for it
httpwwwcolumbianeurosurgeryorgconditionsglossopharyngeal-neuralgia
What is the treatment of trigeminal neuralgia
bull If the etiology is due to a herpetic-like viral irritation of the trigeminal nerve (Empting L Hendler N Kolodny L Kraus H Tips on hard to manage pain syndromes Patient Care page 26-46 April 30 1990) then the treatment is the use of medications
bull Some people respond to low dose anti-depressants but the most useful medication is an anti-convulsant such as gaba-pentin (Neurontin) or Tegretol
bull Anti-viral drug such as acyclovir will also help
bull Rarely radio-frequency lesions of the retro-gausserian ganglion can be employed if all else fails
What is Eagles syndrome
bull Eagles Syndrome is characterized by an a specific orofacial pain secondary to calcification of the stylohyoid ligament or elongated styoid process In about 4 of general population an elongated styloid process occurs while only about 4 of these patients are symptomatic The stylo-hyoid bone is considered abnormal if it is greater than 25 mm in length (Casale M Rinaldi V Quattrocchi C Bressi F Vincenzi B Santini D Tonini G Salvinelli F Atypical chronic head and neck pain dont forget Eagles syndrome Eur Rev Med Pharmacol Sci 2008 Mar-Apr12(2)131-3)
3D-CT of a very long
stylo-hyoid
bonehellipmuch longer
than 25 mm Follow
the numbers 123
and 4 on both sides of
the 3D-CT and the is
the stylo-hyoid bone
which is about 39 mm
in this 3D-CT The
bone marked H is the
hyoid bone In this
3D-CT the stylo-hyoid
bone is so long that is
spontaneously broke
between 2 and 3 but it
is still symptomatic
and causes pain with
swollowing Removal
of the bone from the
break to 4 on the 3D-
CT will relieve
symptoms
What are the symptoms of Eagles syndrome and how do you diagnosis itbull The patient has a sensation of a foreign body localized at
the tonsillar fossa associated with a dull intermittent painbull Pain is usually worse with swallowingbull A bony projection is usually palpable with bimanual trans-
oral explorationbull Basically you put your finger in the mouth of the patient
down their throat and push towards the outside where the stylohyoid bone is If this reproduces the pain this confirms the diagnosis
bull A lateral radiograph and a computed tomography scan of head and neck will show an elongated styloid process of at least 25 mm (Casale M Rinaldi V Quattrocchi C Bressi F Vincenzi B Santini D Tonini G Salvinelli FAtypical chronic head and neck pain dont forget Eagles syndrome Eur Rev Med Pharmacol Sci 2008 Mar-Apr12(2)131-3)
How do you treat Eagles syndromebull Medical treatment represents the first choice
bull Use non-steroidal anti- inflammatory drugs
bull If this is not effective then surgical resection of the styloid process may help (cutting off the tip)
bull In the case of persistence or ingravescence of the complaint surgery may be the only option
bull Symptoms may overlap with glossopharyngealneuritis (presented later)
bull (Casale M Rinaldi V Quattrocchi C Bressi F Vincenzi B Santini D Tonini G
Salvinelli F Atypical chronic head and neck pain dont forget Eagles syndrome EurRev Med Pharmacol Sci 2008 Mar-Apr12(2)131-3)
What is Sjogrenrsquos Syndrome bull Sjogren syndrome is an autoimmune disorder in
which the glands that produce tears and saliva are destroyed causing dry mouth and dry eyes
bull Symptoms may include itching eyes feeling that something is in the eye difficulty swallowing loss of sense of taste thick saliva mouth sores many cavities in the teeth and pain in the face usually in the trigeminal or facial nerve
bull (Kruszka P OBrian RJ Diagnosis and management of Sjogren syndrome Am Fam Physician 200979465-470)
What are symptoms of Sjogrenrsquos Syndromebull Can be mistaken for trigeminal neuralgia
bull When there is unexplained pain in the face take a careful history Ask about dry mouth itchy eyes lots of cavities trouble swallowing SICCA complex (1)
bull Trigeminal sensory neuropathy can occur with Sjogrenrsquos and may be characterized by progressive sensory complaints on the face They could start on one side and subsequently become bilateral (2)
bull (1) Wu AJ Optimizing dry mouth treatment for individuals with Sjoumlgrens syndrome Rheum Dis Clin North Am 2008 Nov34(4)1001-1010
bull (2) Steven Mandel MD Clinical Professor of Neurology Jefferson Medical College)
Laboratory Tests to Diagnose Sjogrenrsquos
bull SSA and SSB ANA sed rate rheumatoid factor (RF) Schirmer test corneal fluorescein staining conjunctival lissamine green staining tear-film breakup time and lip biopsy are useful methods of diagnosing Sjogrenrsquos but not always reliable
bull Yang Z Liang Y Zhong R Is identification of anti-SSA andor -SSB antibodies necessary in serum samples referred for antinuclear antibodies testing J Clin Lab Anal 2012 Nov26(6)447-51 Br J Ophthalmol 2012 Dec96(12)1498-503 doi with aqueous-deficient dry eye
bull Liew MS Zhang M Kim E Akpek EK Prevalence and predictors of Sjogrens syndrome in a prospective cohort of patients with aqueous-deficient dry eye Br J Ophthalmol 2012 Dec96(12)1498-503
Treatment of Sjogrenrsquosbull Conventional immunosuppressive therapies
such as corticosteroids have been used in some patients with variable benefit
bull Current therapy for dryness is principally symptomatic
bull The greatest experience in pSS is with rituximab an anti-CD20 (expressed on a subset of B-cells) monoclonal antibody
(Bowman S Barone F Biologic treatments in Sjoumlgrens syndrome Presse Med 2012 Sep41(9 Pt 2)e495-509)
Symptoms of Glossopharyngeal Neuritis
bull Glossopharyngeal neuralgia is classically described as ldquostabbingrdquo or ldquoelectrical shock-likerdquo in nature Episodes are typically brief lasting a couple of seconds up to a few minutes and can be quite debilitating They can occur in the tongue throat ear and tonsils on one side of the head but in rare cases may be bilateral Painful attacks may occur spontaneously but are more often associated with a specific stimulus such as chewing speaking coughing swallowing or laughing The attacks can occur frequently throughout the day or once every couple of weeks and they can also disturb sleep
bull httpwwwcolumbianeurosurgeryorgconditionsglossopharyngeal-neuralgia
Causes of Glossopharyngeal Neuralgiabull Neuralgia is typically caused by compression of the
glossopharyngeal nerve
bull Compression of the nerve by an adjacent artery andor vein can occur near the brainstem
bull Compression of the nerve more distally in the neck by an elongated styloid process (a bone in the neck) can cause this pain syndrome as well and this is called Eagle syndrome
bull Glossopharyngeal neuralgia can be caused by a tumor or infection in the region of ninth cranial nerve or by multiple sclerosis
bull httpwwwcolumbianeurosurgeryorgconditionsglossopharyngeal-neuralgia
Differential diagnosis of Glossopharyngeal Neuritis (IX)
bull The diagnosis is made on clinical grounds alone
bull no specific test can be done to prove the diagnosis in any given patient Episodic ear andor throat pain often triggered by touching the palate or tonsil is strongly suggestive of the disorder
bull High resolution MRI or CT imaging of the brainstem may reveal vascular compression tumors or demyelinating lesions involving the ninth cranial nerve
bull High resolution CT scanning of the neck can reveal an elongated styloid process suggestive of Eagle syndrome
bull Pain that distinctly involves the ear rear 13 of the tongue andor throat is the classic pain distribution seen in glossopharyngeal neuralgia (ninth cranial nerve or IX N)
httpwwwcolumbianeurosurgeryorgconditionsglossopharyngeal-neuralgia
Treatment of Glossopharyngeal Neuritisbull Most treatment for Glossopharyngeal Neuralgia is
pharmacological Anticonvulsant medications including oxcarbazepine carbamazepine gabapentin and pregabalin Some antidepressants can also be helpful either alone or in combination with the seizure medications If medications fail surgical intervention is indicated
bull The first-choice treatment microvascular decompression (MVD) as it has the highest initial and long-term success rates Patients unable to tolerate such an operation may consider one of the many less invasive options including Gamma Knife radiosurgery
bull Eagles syndrome TMJ and Trigeminal neuralgia can co-exist with glossopharyngeal neuritis and mask the diagnosis or be mistaken for it
httpwwwcolumbianeurosurgeryorgconditionsglossopharyngeal-neuralgia
What is Eagles syndrome
bull Eagles Syndrome is characterized by an a specific orofacial pain secondary to calcification of the stylohyoid ligament or elongated styoid process In about 4 of general population an elongated styloid process occurs while only about 4 of these patients are symptomatic The stylo-hyoid bone is considered abnormal if it is greater than 25 mm in length (Casale M Rinaldi V Quattrocchi C Bressi F Vincenzi B Santini D Tonini G Salvinelli F Atypical chronic head and neck pain dont forget Eagles syndrome Eur Rev Med Pharmacol Sci 2008 Mar-Apr12(2)131-3)
3D-CT of a very long
stylo-hyoid
bonehellipmuch longer
than 25 mm Follow
the numbers 123
and 4 on both sides of
the 3D-CT and the is
the stylo-hyoid bone
which is about 39 mm
in this 3D-CT The
bone marked H is the
hyoid bone In this
3D-CT the stylo-hyoid
bone is so long that is
spontaneously broke
between 2 and 3 but it
is still symptomatic
and causes pain with
swollowing Removal
of the bone from the
break to 4 on the 3D-
CT will relieve
symptoms
What are the symptoms of Eagles syndrome and how do you diagnosis itbull The patient has a sensation of a foreign body localized at
the tonsillar fossa associated with a dull intermittent painbull Pain is usually worse with swallowingbull A bony projection is usually palpable with bimanual trans-
oral explorationbull Basically you put your finger in the mouth of the patient
down their throat and push towards the outside where the stylohyoid bone is If this reproduces the pain this confirms the diagnosis
bull A lateral radiograph and a computed tomography scan of head and neck will show an elongated styloid process of at least 25 mm (Casale M Rinaldi V Quattrocchi C Bressi F Vincenzi B Santini D Tonini G Salvinelli FAtypical chronic head and neck pain dont forget Eagles syndrome Eur Rev Med Pharmacol Sci 2008 Mar-Apr12(2)131-3)
How do you treat Eagles syndromebull Medical treatment represents the first choice
bull Use non-steroidal anti- inflammatory drugs
bull If this is not effective then surgical resection of the styloid process may help (cutting off the tip)
bull In the case of persistence or ingravescence of the complaint surgery may be the only option
bull Symptoms may overlap with glossopharyngealneuritis (presented later)
bull (Casale M Rinaldi V Quattrocchi C Bressi F Vincenzi B Santini D Tonini G
Salvinelli F Atypical chronic head and neck pain dont forget Eagles syndrome EurRev Med Pharmacol Sci 2008 Mar-Apr12(2)131-3)
What is Sjogrenrsquos Syndrome bull Sjogren syndrome is an autoimmune disorder in
which the glands that produce tears and saliva are destroyed causing dry mouth and dry eyes
bull Symptoms may include itching eyes feeling that something is in the eye difficulty swallowing loss of sense of taste thick saliva mouth sores many cavities in the teeth and pain in the face usually in the trigeminal or facial nerve
bull (Kruszka P OBrian RJ Diagnosis and management of Sjogren syndrome Am Fam Physician 200979465-470)
What are symptoms of Sjogrenrsquos Syndromebull Can be mistaken for trigeminal neuralgia
bull When there is unexplained pain in the face take a careful history Ask about dry mouth itchy eyes lots of cavities trouble swallowing SICCA complex (1)
bull Trigeminal sensory neuropathy can occur with Sjogrenrsquos and may be characterized by progressive sensory complaints on the face They could start on one side and subsequently become bilateral (2)
bull (1) Wu AJ Optimizing dry mouth treatment for individuals with Sjoumlgrens syndrome Rheum Dis Clin North Am 2008 Nov34(4)1001-1010
bull (2) Steven Mandel MD Clinical Professor of Neurology Jefferson Medical College)
Laboratory Tests to Diagnose Sjogrenrsquos
bull SSA and SSB ANA sed rate rheumatoid factor (RF) Schirmer test corneal fluorescein staining conjunctival lissamine green staining tear-film breakup time and lip biopsy are useful methods of diagnosing Sjogrenrsquos but not always reliable
bull Yang Z Liang Y Zhong R Is identification of anti-SSA andor -SSB antibodies necessary in serum samples referred for antinuclear antibodies testing J Clin Lab Anal 2012 Nov26(6)447-51 Br J Ophthalmol 2012 Dec96(12)1498-503 doi with aqueous-deficient dry eye
bull Liew MS Zhang M Kim E Akpek EK Prevalence and predictors of Sjogrens syndrome in a prospective cohort of patients with aqueous-deficient dry eye Br J Ophthalmol 2012 Dec96(12)1498-503
Treatment of Sjogrenrsquosbull Conventional immunosuppressive therapies
such as corticosteroids have been used in some patients with variable benefit
bull Current therapy for dryness is principally symptomatic
bull The greatest experience in pSS is with rituximab an anti-CD20 (expressed on a subset of B-cells) monoclonal antibody
(Bowman S Barone F Biologic treatments in Sjoumlgrens syndrome Presse Med 2012 Sep41(9 Pt 2)e495-509)
Symptoms of Glossopharyngeal Neuritis
bull Glossopharyngeal neuralgia is classically described as ldquostabbingrdquo or ldquoelectrical shock-likerdquo in nature Episodes are typically brief lasting a couple of seconds up to a few minutes and can be quite debilitating They can occur in the tongue throat ear and tonsils on one side of the head but in rare cases may be bilateral Painful attacks may occur spontaneously but are more often associated with a specific stimulus such as chewing speaking coughing swallowing or laughing The attacks can occur frequently throughout the day or once every couple of weeks and they can also disturb sleep
bull httpwwwcolumbianeurosurgeryorgconditionsglossopharyngeal-neuralgia
Causes of Glossopharyngeal Neuralgiabull Neuralgia is typically caused by compression of the
glossopharyngeal nerve
bull Compression of the nerve by an adjacent artery andor vein can occur near the brainstem
bull Compression of the nerve more distally in the neck by an elongated styloid process (a bone in the neck) can cause this pain syndrome as well and this is called Eagle syndrome
bull Glossopharyngeal neuralgia can be caused by a tumor or infection in the region of ninth cranial nerve or by multiple sclerosis
bull httpwwwcolumbianeurosurgeryorgconditionsglossopharyngeal-neuralgia
Differential diagnosis of Glossopharyngeal Neuritis (IX)
bull The diagnosis is made on clinical grounds alone
bull no specific test can be done to prove the diagnosis in any given patient Episodic ear andor throat pain often triggered by touching the palate or tonsil is strongly suggestive of the disorder
bull High resolution MRI or CT imaging of the brainstem may reveal vascular compression tumors or demyelinating lesions involving the ninth cranial nerve
bull High resolution CT scanning of the neck can reveal an elongated styloid process suggestive of Eagle syndrome
bull Pain that distinctly involves the ear rear 13 of the tongue andor throat is the classic pain distribution seen in glossopharyngeal neuralgia (ninth cranial nerve or IX N)
httpwwwcolumbianeurosurgeryorgconditionsglossopharyngeal-neuralgia
Treatment of Glossopharyngeal Neuritisbull Most treatment for Glossopharyngeal Neuralgia is
pharmacological Anticonvulsant medications including oxcarbazepine carbamazepine gabapentin and pregabalin Some antidepressants can also be helpful either alone or in combination with the seizure medications If medications fail surgical intervention is indicated
bull The first-choice treatment microvascular decompression (MVD) as it has the highest initial and long-term success rates Patients unable to tolerate such an operation may consider one of the many less invasive options including Gamma Knife radiosurgery
bull Eagles syndrome TMJ and Trigeminal neuralgia can co-exist with glossopharyngeal neuritis and mask the diagnosis or be mistaken for it
httpwwwcolumbianeurosurgeryorgconditionsglossopharyngeal-neuralgia
3D-CT of a very long
stylo-hyoid
bonehellipmuch longer
than 25 mm Follow
the numbers 123
and 4 on both sides of
the 3D-CT and the is
the stylo-hyoid bone
which is about 39 mm
in this 3D-CT The
bone marked H is the
hyoid bone In this
3D-CT the stylo-hyoid
bone is so long that is
spontaneously broke
between 2 and 3 but it
is still symptomatic
and causes pain with
swollowing Removal
of the bone from the
break to 4 on the 3D-
CT will relieve
symptoms
What are the symptoms of Eagles syndrome and how do you diagnosis itbull The patient has a sensation of a foreign body localized at
the tonsillar fossa associated with a dull intermittent painbull Pain is usually worse with swallowingbull A bony projection is usually palpable with bimanual trans-
oral explorationbull Basically you put your finger in the mouth of the patient
down their throat and push towards the outside where the stylohyoid bone is If this reproduces the pain this confirms the diagnosis
bull A lateral radiograph and a computed tomography scan of head and neck will show an elongated styloid process of at least 25 mm (Casale M Rinaldi V Quattrocchi C Bressi F Vincenzi B Santini D Tonini G Salvinelli FAtypical chronic head and neck pain dont forget Eagles syndrome Eur Rev Med Pharmacol Sci 2008 Mar-Apr12(2)131-3)
How do you treat Eagles syndromebull Medical treatment represents the first choice
bull Use non-steroidal anti- inflammatory drugs
bull If this is not effective then surgical resection of the styloid process may help (cutting off the tip)
bull In the case of persistence or ingravescence of the complaint surgery may be the only option
bull Symptoms may overlap with glossopharyngealneuritis (presented later)
bull (Casale M Rinaldi V Quattrocchi C Bressi F Vincenzi B Santini D Tonini G
Salvinelli F Atypical chronic head and neck pain dont forget Eagles syndrome EurRev Med Pharmacol Sci 2008 Mar-Apr12(2)131-3)
What is Sjogrenrsquos Syndrome bull Sjogren syndrome is an autoimmune disorder in
which the glands that produce tears and saliva are destroyed causing dry mouth and dry eyes
bull Symptoms may include itching eyes feeling that something is in the eye difficulty swallowing loss of sense of taste thick saliva mouth sores many cavities in the teeth and pain in the face usually in the trigeminal or facial nerve
bull (Kruszka P OBrian RJ Diagnosis and management of Sjogren syndrome Am Fam Physician 200979465-470)
What are symptoms of Sjogrenrsquos Syndromebull Can be mistaken for trigeminal neuralgia
bull When there is unexplained pain in the face take a careful history Ask about dry mouth itchy eyes lots of cavities trouble swallowing SICCA complex (1)
bull Trigeminal sensory neuropathy can occur with Sjogrenrsquos and may be characterized by progressive sensory complaints on the face They could start on one side and subsequently become bilateral (2)
bull (1) Wu AJ Optimizing dry mouth treatment for individuals with Sjoumlgrens syndrome Rheum Dis Clin North Am 2008 Nov34(4)1001-1010
bull (2) Steven Mandel MD Clinical Professor of Neurology Jefferson Medical College)
Laboratory Tests to Diagnose Sjogrenrsquos
bull SSA and SSB ANA sed rate rheumatoid factor (RF) Schirmer test corneal fluorescein staining conjunctival lissamine green staining tear-film breakup time and lip biopsy are useful methods of diagnosing Sjogrenrsquos but not always reliable
bull Yang Z Liang Y Zhong R Is identification of anti-SSA andor -SSB antibodies necessary in serum samples referred for antinuclear antibodies testing J Clin Lab Anal 2012 Nov26(6)447-51 Br J Ophthalmol 2012 Dec96(12)1498-503 doi with aqueous-deficient dry eye
bull Liew MS Zhang M Kim E Akpek EK Prevalence and predictors of Sjogrens syndrome in a prospective cohort of patients with aqueous-deficient dry eye Br J Ophthalmol 2012 Dec96(12)1498-503
Treatment of Sjogrenrsquosbull Conventional immunosuppressive therapies
such as corticosteroids have been used in some patients with variable benefit
bull Current therapy for dryness is principally symptomatic
bull The greatest experience in pSS is with rituximab an anti-CD20 (expressed on a subset of B-cells) monoclonal antibody
(Bowman S Barone F Biologic treatments in Sjoumlgrens syndrome Presse Med 2012 Sep41(9 Pt 2)e495-509)
Symptoms of Glossopharyngeal Neuritis
bull Glossopharyngeal neuralgia is classically described as ldquostabbingrdquo or ldquoelectrical shock-likerdquo in nature Episodes are typically brief lasting a couple of seconds up to a few minutes and can be quite debilitating They can occur in the tongue throat ear and tonsils on one side of the head but in rare cases may be bilateral Painful attacks may occur spontaneously but are more often associated with a specific stimulus such as chewing speaking coughing swallowing or laughing The attacks can occur frequently throughout the day or once every couple of weeks and they can also disturb sleep
bull httpwwwcolumbianeurosurgeryorgconditionsglossopharyngeal-neuralgia
Causes of Glossopharyngeal Neuralgiabull Neuralgia is typically caused by compression of the
glossopharyngeal nerve
bull Compression of the nerve by an adjacent artery andor vein can occur near the brainstem
bull Compression of the nerve more distally in the neck by an elongated styloid process (a bone in the neck) can cause this pain syndrome as well and this is called Eagle syndrome
bull Glossopharyngeal neuralgia can be caused by a tumor or infection in the region of ninth cranial nerve or by multiple sclerosis
bull httpwwwcolumbianeurosurgeryorgconditionsglossopharyngeal-neuralgia
Differential diagnosis of Glossopharyngeal Neuritis (IX)
bull The diagnosis is made on clinical grounds alone
bull no specific test can be done to prove the diagnosis in any given patient Episodic ear andor throat pain often triggered by touching the palate or tonsil is strongly suggestive of the disorder
bull High resolution MRI or CT imaging of the brainstem may reveal vascular compression tumors or demyelinating lesions involving the ninth cranial nerve
bull High resolution CT scanning of the neck can reveal an elongated styloid process suggestive of Eagle syndrome
bull Pain that distinctly involves the ear rear 13 of the tongue andor throat is the classic pain distribution seen in glossopharyngeal neuralgia (ninth cranial nerve or IX N)
httpwwwcolumbianeurosurgeryorgconditionsglossopharyngeal-neuralgia
Treatment of Glossopharyngeal Neuritisbull Most treatment for Glossopharyngeal Neuralgia is
pharmacological Anticonvulsant medications including oxcarbazepine carbamazepine gabapentin and pregabalin Some antidepressants can also be helpful either alone or in combination with the seizure medications If medications fail surgical intervention is indicated
bull The first-choice treatment microvascular decompression (MVD) as it has the highest initial and long-term success rates Patients unable to tolerate such an operation may consider one of the many less invasive options including Gamma Knife radiosurgery
bull Eagles syndrome TMJ and Trigeminal neuralgia can co-exist with glossopharyngeal neuritis and mask the diagnosis or be mistaken for it
httpwwwcolumbianeurosurgeryorgconditionsglossopharyngeal-neuralgia
What are the symptoms of Eagles syndrome and how do you diagnosis itbull The patient has a sensation of a foreign body localized at
the tonsillar fossa associated with a dull intermittent painbull Pain is usually worse with swallowingbull A bony projection is usually palpable with bimanual trans-
oral explorationbull Basically you put your finger in the mouth of the patient
down their throat and push towards the outside where the stylohyoid bone is If this reproduces the pain this confirms the diagnosis
bull A lateral radiograph and a computed tomography scan of head and neck will show an elongated styloid process of at least 25 mm (Casale M Rinaldi V Quattrocchi C Bressi F Vincenzi B Santini D Tonini G Salvinelli FAtypical chronic head and neck pain dont forget Eagles syndrome Eur Rev Med Pharmacol Sci 2008 Mar-Apr12(2)131-3)
How do you treat Eagles syndromebull Medical treatment represents the first choice
bull Use non-steroidal anti- inflammatory drugs
bull If this is not effective then surgical resection of the styloid process may help (cutting off the tip)
bull In the case of persistence or ingravescence of the complaint surgery may be the only option
bull Symptoms may overlap with glossopharyngealneuritis (presented later)
bull (Casale M Rinaldi V Quattrocchi C Bressi F Vincenzi B Santini D Tonini G
Salvinelli F Atypical chronic head and neck pain dont forget Eagles syndrome EurRev Med Pharmacol Sci 2008 Mar-Apr12(2)131-3)
What is Sjogrenrsquos Syndrome bull Sjogren syndrome is an autoimmune disorder in
which the glands that produce tears and saliva are destroyed causing dry mouth and dry eyes
bull Symptoms may include itching eyes feeling that something is in the eye difficulty swallowing loss of sense of taste thick saliva mouth sores many cavities in the teeth and pain in the face usually in the trigeminal or facial nerve
bull (Kruszka P OBrian RJ Diagnosis and management of Sjogren syndrome Am Fam Physician 200979465-470)
What are symptoms of Sjogrenrsquos Syndromebull Can be mistaken for trigeminal neuralgia
bull When there is unexplained pain in the face take a careful history Ask about dry mouth itchy eyes lots of cavities trouble swallowing SICCA complex (1)
bull Trigeminal sensory neuropathy can occur with Sjogrenrsquos and may be characterized by progressive sensory complaints on the face They could start on one side and subsequently become bilateral (2)
bull (1) Wu AJ Optimizing dry mouth treatment for individuals with Sjoumlgrens syndrome Rheum Dis Clin North Am 2008 Nov34(4)1001-1010
bull (2) Steven Mandel MD Clinical Professor of Neurology Jefferson Medical College)
Laboratory Tests to Diagnose Sjogrenrsquos
bull SSA and SSB ANA sed rate rheumatoid factor (RF) Schirmer test corneal fluorescein staining conjunctival lissamine green staining tear-film breakup time and lip biopsy are useful methods of diagnosing Sjogrenrsquos but not always reliable
bull Yang Z Liang Y Zhong R Is identification of anti-SSA andor -SSB antibodies necessary in serum samples referred for antinuclear antibodies testing J Clin Lab Anal 2012 Nov26(6)447-51 Br J Ophthalmol 2012 Dec96(12)1498-503 doi with aqueous-deficient dry eye
bull Liew MS Zhang M Kim E Akpek EK Prevalence and predictors of Sjogrens syndrome in a prospective cohort of patients with aqueous-deficient dry eye Br J Ophthalmol 2012 Dec96(12)1498-503
Treatment of Sjogrenrsquosbull Conventional immunosuppressive therapies
such as corticosteroids have been used in some patients with variable benefit
bull Current therapy for dryness is principally symptomatic
bull The greatest experience in pSS is with rituximab an anti-CD20 (expressed on a subset of B-cells) monoclonal antibody
(Bowman S Barone F Biologic treatments in Sjoumlgrens syndrome Presse Med 2012 Sep41(9 Pt 2)e495-509)
Symptoms of Glossopharyngeal Neuritis
bull Glossopharyngeal neuralgia is classically described as ldquostabbingrdquo or ldquoelectrical shock-likerdquo in nature Episodes are typically brief lasting a couple of seconds up to a few minutes and can be quite debilitating They can occur in the tongue throat ear and tonsils on one side of the head but in rare cases may be bilateral Painful attacks may occur spontaneously but are more often associated with a specific stimulus such as chewing speaking coughing swallowing or laughing The attacks can occur frequently throughout the day or once every couple of weeks and they can also disturb sleep
bull httpwwwcolumbianeurosurgeryorgconditionsglossopharyngeal-neuralgia
Causes of Glossopharyngeal Neuralgiabull Neuralgia is typically caused by compression of the
glossopharyngeal nerve
bull Compression of the nerve by an adjacent artery andor vein can occur near the brainstem
bull Compression of the nerve more distally in the neck by an elongated styloid process (a bone in the neck) can cause this pain syndrome as well and this is called Eagle syndrome
bull Glossopharyngeal neuralgia can be caused by a tumor or infection in the region of ninth cranial nerve or by multiple sclerosis
bull httpwwwcolumbianeurosurgeryorgconditionsglossopharyngeal-neuralgia
Differential diagnosis of Glossopharyngeal Neuritis (IX)
bull The diagnosis is made on clinical grounds alone
bull no specific test can be done to prove the diagnosis in any given patient Episodic ear andor throat pain often triggered by touching the palate or tonsil is strongly suggestive of the disorder
bull High resolution MRI or CT imaging of the brainstem may reveal vascular compression tumors or demyelinating lesions involving the ninth cranial nerve
bull High resolution CT scanning of the neck can reveal an elongated styloid process suggestive of Eagle syndrome
bull Pain that distinctly involves the ear rear 13 of the tongue andor throat is the classic pain distribution seen in glossopharyngeal neuralgia (ninth cranial nerve or IX N)
httpwwwcolumbianeurosurgeryorgconditionsglossopharyngeal-neuralgia
Treatment of Glossopharyngeal Neuritisbull Most treatment for Glossopharyngeal Neuralgia is
pharmacological Anticonvulsant medications including oxcarbazepine carbamazepine gabapentin and pregabalin Some antidepressants can also be helpful either alone or in combination with the seizure medications If medications fail surgical intervention is indicated
bull The first-choice treatment microvascular decompression (MVD) as it has the highest initial and long-term success rates Patients unable to tolerate such an operation may consider one of the many less invasive options including Gamma Knife radiosurgery
bull Eagles syndrome TMJ and Trigeminal neuralgia can co-exist with glossopharyngeal neuritis and mask the diagnosis or be mistaken for it
httpwwwcolumbianeurosurgeryorgconditionsglossopharyngeal-neuralgia
How do you treat Eagles syndromebull Medical treatment represents the first choice
bull Use non-steroidal anti- inflammatory drugs
bull If this is not effective then surgical resection of the styloid process may help (cutting off the tip)
bull In the case of persistence or ingravescence of the complaint surgery may be the only option
bull Symptoms may overlap with glossopharyngealneuritis (presented later)
bull (Casale M Rinaldi V Quattrocchi C Bressi F Vincenzi B Santini D Tonini G
Salvinelli F Atypical chronic head and neck pain dont forget Eagles syndrome EurRev Med Pharmacol Sci 2008 Mar-Apr12(2)131-3)
What is Sjogrenrsquos Syndrome bull Sjogren syndrome is an autoimmune disorder in
which the glands that produce tears and saliva are destroyed causing dry mouth and dry eyes
bull Symptoms may include itching eyes feeling that something is in the eye difficulty swallowing loss of sense of taste thick saliva mouth sores many cavities in the teeth and pain in the face usually in the trigeminal or facial nerve
bull (Kruszka P OBrian RJ Diagnosis and management of Sjogren syndrome Am Fam Physician 200979465-470)
What are symptoms of Sjogrenrsquos Syndromebull Can be mistaken for trigeminal neuralgia
bull When there is unexplained pain in the face take a careful history Ask about dry mouth itchy eyes lots of cavities trouble swallowing SICCA complex (1)
bull Trigeminal sensory neuropathy can occur with Sjogrenrsquos and may be characterized by progressive sensory complaints on the face They could start on one side and subsequently become bilateral (2)
bull (1) Wu AJ Optimizing dry mouth treatment for individuals with Sjoumlgrens syndrome Rheum Dis Clin North Am 2008 Nov34(4)1001-1010
bull (2) Steven Mandel MD Clinical Professor of Neurology Jefferson Medical College)
Laboratory Tests to Diagnose Sjogrenrsquos
bull SSA and SSB ANA sed rate rheumatoid factor (RF) Schirmer test corneal fluorescein staining conjunctival lissamine green staining tear-film breakup time and lip biopsy are useful methods of diagnosing Sjogrenrsquos but not always reliable
bull Yang Z Liang Y Zhong R Is identification of anti-SSA andor -SSB antibodies necessary in serum samples referred for antinuclear antibodies testing J Clin Lab Anal 2012 Nov26(6)447-51 Br J Ophthalmol 2012 Dec96(12)1498-503 doi with aqueous-deficient dry eye
bull Liew MS Zhang M Kim E Akpek EK Prevalence and predictors of Sjogrens syndrome in a prospective cohort of patients with aqueous-deficient dry eye Br J Ophthalmol 2012 Dec96(12)1498-503
Treatment of Sjogrenrsquosbull Conventional immunosuppressive therapies
such as corticosteroids have been used in some patients with variable benefit
bull Current therapy for dryness is principally symptomatic
bull The greatest experience in pSS is with rituximab an anti-CD20 (expressed on a subset of B-cells) monoclonal antibody
(Bowman S Barone F Biologic treatments in Sjoumlgrens syndrome Presse Med 2012 Sep41(9 Pt 2)e495-509)
Symptoms of Glossopharyngeal Neuritis
bull Glossopharyngeal neuralgia is classically described as ldquostabbingrdquo or ldquoelectrical shock-likerdquo in nature Episodes are typically brief lasting a couple of seconds up to a few minutes and can be quite debilitating They can occur in the tongue throat ear and tonsils on one side of the head but in rare cases may be bilateral Painful attacks may occur spontaneously but are more often associated with a specific stimulus such as chewing speaking coughing swallowing or laughing The attacks can occur frequently throughout the day or once every couple of weeks and they can also disturb sleep
bull httpwwwcolumbianeurosurgeryorgconditionsglossopharyngeal-neuralgia
Causes of Glossopharyngeal Neuralgiabull Neuralgia is typically caused by compression of the
glossopharyngeal nerve
bull Compression of the nerve by an adjacent artery andor vein can occur near the brainstem
bull Compression of the nerve more distally in the neck by an elongated styloid process (a bone in the neck) can cause this pain syndrome as well and this is called Eagle syndrome
bull Glossopharyngeal neuralgia can be caused by a tumor or infection in the region of ninth cranial nerve or by multiple sclerosis
bull httpwwwcolumbianeurosurgeryorgconditionsglossopharyngeal-neuralgia
Differential diagnosis of Glossopharyngeal Neuritis (IX)
bull The diagnosis is made on clinical grounds alone
bull no specific test can be done to prove the diagnosis in any given patient Episodic ear andor throat pain often triggered by touching the palate or tonsil is strongly suggestive of the disorder
bull High resolution MRI or CT imaging of the brainstem may reveal vascular compression tumors or demyelinating lesions involving the ninth cranial nerve
bull High resolution CT scanning of the neck can reveal an elongated styloid process suggestive of Eagle syndrome
bull Pain that distinctly involves the ear rear 13 of the tongue andor throat is the classic pain distribution seen in glossopharyngeal neuralgia (ninth cranial nerve or IX N)
httpwwwcolumbianeurosurgeryorgconditionsglossopharyngeal-neuralgia
Treatment of Glossopharyngeal Neuritisbull Most treatment for Glossopharyngeal Neuralgia is
pharmacological Anticonvulsant medications including oxcarbazepine carbamazepine gabapentin and pregabalin Some antidepressants can also be helpful either alone or in combination with the seizure medications If medications fail surgical intervention is indicated
bull The first-choice treatment microvascular decompression (MVD) as it has the highest initial and long-term success rates Patients unable to tolerate such an operation may consider one of the many less invasive options including Gamma Knife radiosurgery
bull Eagles syndrome TMJ and Trigeminal neuralgia can co-exist with glossopharyngeal neuritis and mask the diagnosis or be mistaken for it
httpwwwcolumbianeurosurgeryorgconditionsglossopharyngeal-neuralgia
What is Sjogrenrsquos Syndrome bull Sjogren syndrome is an autoimmune disorder in
which the glands that produce tears and saliva are destroyed causing dry mouth and dry eyes
bull Symptoms may include itching eyes feeling that something is in the eye difficulty swallowing loss of sense of taste thick saliva mouth sores many cavities in the teeth and pain in the face usually in the trigeminal or facial nerve
bull (Kruszka P OBrian RJ Diagnosis and management of Sjogren syndrome Am Fam Physician 200979465-470)
What are symptoms of Sjogrenrsquos Syndromebull Can be mistaken for trigeminal neuralgia
bull When there is unexplained pain in the face take a careful history Ask about dry mouth itchy eyes lots of cavities trouble swallowing SICCA complex (1)
bull Trigeminal sensory neuropathy can occur with Sjogrenrsquos and may be characterized by progressive sensory complaints on the face They could start on one side and subsequently become bilateral (2)
bull (1) Wu AJ Optimizing dry mouth treatment for individuals with Sjoumlgrens syndrome Rheum Dis Clin North Am 2008 Nov34(4)1001-1010
bull (2) Steven Mandel MD Clinical Professor of Neurology Jefferson Medical College)
Laboratory Tests to Diagnose Sjogrenrsquos
bull SSA and SSB ANA sed rate rheumatoid factor (RF) Schirmer test corneal fluorescein staining conjunctival lissamine green staining tear-film breakup time and lip biopsy are useful methods of diagnosing Sjogrenrsquos but not always reliable
bull Yang Z Liang Y Zhong R Is identification of anti-SSA andor -SSB antibodies necessary in serum samples referred for antinuclear antibodies testing J Clin Lab Anal 2012 Nov26(6)447-51 Br J Ophthalmol 2012 Dec96(12)1498-503 doi with aqueous-deficient dry eye
bull Liew MS Zhang M Kim E Akpek EK Prevalence and predictors of Sjogrens syndrome in a prospective cohort of patients with aqueous-deficient dry eye Br J Ophthalmol 2012 Dec96(12)1498-503
Treatment of Sjogrenrsquosbull Conventional immunosuppressive therapies
such as corticosteroids have been used in some patients with variable benefit
bull Current therapy for dryness is principally symptomatic
bull The greatest experience in pSS is with rituximab an anti-CD20 (expressed on a subset of B-cells) monoclonal antibody
(Bowman S Barone F Biologic treatments in Sjoumlgrens syndrome Presse Med 2012 Sep41(9 Pt 2)e495-509)
Symptoms of Glossopharyngeal Neuritis
bull Glossopharyngeal neuralgia is classically described as ldquostabbingrdquo or ldquoelectrical shock-likerdquo in nature Episodes are typically brief lasting a couple of seconds up to a few minutes and can be quite debilitating They can occur in the tongue throat ear and tonsils on one side of the head but in rare cases may be bilateral Painful attacks may occur spontaneously but are more often associated with a specific stimulus such as chewing speaking coughing swallowing or laughing The attacks can occur frequently throughout the day or once every couple of weeks and they can also disturb sleep
bull httpwwwcolumbianeurosurgeryorgconditionsglossopharyngeal-neuralgia
Causes of Glossopharyngeal Neuralgiabull Neuralgia is typically caused by compression of the
glossopharyngeal nerve
bull Compression of the nerve by an adjacent artery andor vein can occur near the brainstem
bull Compression of the nerve more distally in the neck by an elongated styloid process (a bone in the neck) can cause this pain syndrome as well and this is called Eagle syndrome
bull Glossopharyngeal neuralgia can be caused by a tumor or infection in the region of ninth cranial nerve or by multiple sclerosis
bull httpwwwcolumbianeurosurgeryorgconditionsglossopharyngeal-neuralgia
Differential diagnosis of Glossopharyngeal Neuritis (IX)
bull The diagnosis is made on clinical grounds alone
bull no specific test can be done to prove the diagnosis in any given patient Episodic ear andor throat pain often triggered by touching the palate or tonsil is strongly suggestive of the disorder
bull High resolution MRI or CT imaging of the brainstem may reveal vascular compression tumors or demyelinating lesions involving the ninth cranial nerve
bull High resolution CT scanning of the neck can reveal an elongated styloid process suggestive of Eagle syndrome
bull Pain that distinctly involves the ear rear 13 of the tongue andor throat is the classic pain distribution seen in glossopharyngeal neuralgia (ninth cranial nerve or IX N)
httpwwwcolumbianeurosurgeryorgconditionsglossopharyngeal-neuralgia
Treatment of Glossopharyngeal Neuritisbull Most treatment for Glossopharyngeal Neuralgia is
pharmacological Anticonvulsant medications including oxcarbazepine carbamazepine gabapentin and pregabalin Some antidepressants can also be helpful either alone or in combination with the seizure medications If medications fail surgical intervention is indicated
bull The first-choice treatment microvascular decompression (MVD) as it has the highest initial and long-term success rates Patients unable to tolerate such an operation may consider one of the many less invasive options including Gamma Knife radiosurgery
bull Eagles syndrome TMJ and Trigeminal neuralgia can co-exist with glossopharyngeal neuritis and mask the diagnosis or be mistaken for it
httpwwwcolumbianeurosurgeryorgconditionsglossopharyngeal-neuralgia
What are symptoms of Sjogrenrsquos Syndromebull Can be mistaken for trigeminal neuralgia
bull When there is unexplained pain in the face take a careful history Ask about dry mouth itchy eyes lots of cavities trouble swallowing SICCA complex (1)
bull Trigeminal sensory neuropathy can occur with Sjogrenrsquos and may be characterized by progressive sensory complaints on the face They could start on one side and subsequently become bilateral (2)
bull (1) Wu AJ Optimizing dry mouth treatment for individuals with Sjoumlgrens syndrome Rheum Dis Clin North Am 2008 Nov34(4)1001-1010
bull (2) Steven Mandel MD Clinical Professor of Neurology Jefferson Medical College)
Laboratory Tests to Diagnose Sjogrenrsquos
bull SSA and SSB ANA sed rate rheumatoid factor (RF) Schirmer test corneal fluorescein staining conjunctival lissamine green staining tear-film breakup time and lip biopsy are useful methods of diagnosing Sjogrenrsquos but not always reliable
bull Yang Z Liang Y Zhong R Is identification of anti-SSA andor -SSB antibodies necessary in serum samples referred for antinuclear antibodies testing J Clin Lab Anal 2012 Nov26(6)447-51 Br J Ophthalmol 2012 Dec96(12)1498-503 doi with aqueous-deficient dry eye
bull Liew MS Zhang M Kim E Akpek EK Prevalence and predictors of Sjogrens syndrome in a prospective cohort of patients with aqueous-deficient dry eye Br J Ophthalmol 2012 Dec96(12)1498-503
Treatment of Sjogrenrsquosbull Conventional immunosuppressive therapies
such as corticosteroids have been used in some patients with variable benefit
bull Current therapy for dryness is principally symptomatic
bull The greatest experience in pSS is with rituximab an anti-CD20 (expressed on a subset of B-cells) monoclonal antibody
(Bowman S Barone F Biologic treatments in Sjoumlgrens syndrome Presse Med 2012 Sep41(9 Pt 2)e495-509)
Symptoms of Glossopharyngeal Neuritis
bull Glossopharyngeal neuralgia is classically described as ldquostabbingrdquo or ldquoelectrical shock-likerdquo in nature Episodes are typically brief lasting a couple of seconds up to a few minutes and can be quite debilitating They can occur in the tongue throat ear and tonsils on one side of the head but in rare cases may be bilateral Painful attacks may occur spontaneously but are more often associated with a specific stimulus such as chewing speaking coughing swallowing or laughing The attacks can occur frequently throughout the day or once every couple of weeks and they can also disturb sleep
bull httpwwwcolumbianeurosurgeryorgconditionsglossopharyngeal-neuralgia
Causes of Glossopharyngeal Neuralgiabull Neuralgia is typically caused by compression of the
glossopharyngeal nerve
bull Compression of the nerve by an adjacent artery andor vein can occur near the brainstem
bull Compression of the nerve more distally in the neck by an elongated styloid process (a bone in the neck) can cause this pain syndrome as well and this is called Eagle syndrome
bull Glossopharyngeal neuralgia can be caused by a tumor or infection in the region of ninth cranial nerve or by multiple sclerosis
bull httpwwwcolumbianeurosurgeryorgconditionsglossopharyngeal-neuralgia
Differential diagnosis of Glossopharyngeal Neuritis (IX)
bull The diagnosis is made on clinical grounds alone
bull no specific test can be done to prove the diagnosis in any given patient Episodic ear andor throat pain often triggered by touching the palate or tonsil is strongly suggestive of the disorder
bull High resolution MRI or CT imaging of the brainstem may reveal vascular compression tumors or demyelinating lesions involving the ninth cranial nerve
bull High resolution CT scanning of the neck can reveal an elongated styloid process suggestive of Eagle syndrome
bull Pain that distinctly involves the ear rear 13 of the tongue andor throat is the classic pain distribution seen in glossopharyngeal neuralgia (ninth cranial nerve or IX N)
httpwwwcolumbianeurosurgeryorgconditionsglossopharyngeal-neuralgia
Treatment of Glossopharyngeal Neuritisbull Most treatment for Glossopharyngeal Neuralgia is
pharmacological Anticonvulsant medications including oxcarbazepine carbamazepine gabapentin and pregabalin Some antidepressants can also be helpful either alone or in combination with the seizure medications If medications fail surgical intervention is indicated
bull The first-choice treatment microvascular decompression (MVD) as it has the highest initial and long-term success rates Patients unable to tolerate such an operation may consider one of the many less invasive options including Gamma Knife radiosurgery
bull Eagles syndrome TMJ and Trigeminal neuralgia can co-exist with glossopharyngeal neuritis and mask the diagnosis or be mistaken for it
httpwwwcolumbianeurosurgeryorgconditionsglossopharyngeal-neuralgia
Laboratory Tests to Diagnose Sjogrenrsquos
bull SSA and SSB ANA sed rate rheumatoid factor (RF) Schirmer test corneal fluorescein staining conjunctival lissamine green staining tear-film breakup time and lip biopsy are useful methods of diagnosing Sjogrenrsquos but not always reliable
bull Yang Z Liang Y Zhong R Is identification of anti-SSA andor -SSB antibodies necessary in serum samples referred for antinuclear antibodies testing J Clin Lab Anal 2012 Nov26(6)447-51 Br J Ophthalmol 2012 Dec96(12)1498-503 doi with aqueous-deficient dry eye
bull Liew MS Zhang M Kim E Akpek EK Prevalence and predictors of Sjogrens syndrome in a prospective cohort of patients with aqueous-deficient dry eye Br J Ophthalmol 2012 Dec96(12)1498-503
Treatment of Sjogrenrsquosbull Conventional immunosuppressive therapies
such as corticosteroids have been used in some patients with variable benefit
bull Current therapy for dryness is principally symptomatic
bull The greatest experience in pSS is with rituximab an anti-CD20 (expressed on a subset of B-cells) monoclonal antibody
(Bowman S Barone F Biologic treatments in Sjoumlgrens syndrome Presse Med 2012 Sep41(9 Pt 2)e495-509)
Symptoms of Glossopharyngeal Neuritis
bull Glossopharyngeal neuralgia is classically described as ldquostabbingrdquo or ldquoelectrical shock-likerdquo in nature Episodes are typically brief lasting a couple of seconds up to a few minutes and can be quite debilitating They can occur in the tongue throat ear and tonsils on one side of the head but in rare cases may be bilateral Painful attacks may occur spontaneously but are more often associated with a specific stimulus such as chewing speaking coughing swallowing or laughing The attacks can occur frequently throughout the day or once every couple of weeks and they can also disturb sleep
bull httpwwwcolumbianeurosurgeryorgconditionsglossopharyngeal-neuralgia
Causes of Glossopharyngeal Neuralgiabull Neuralgia is typically caused by compression of the
glossopharyngeal nerve
bull Compression of the nerve by an adjacent artery andor vein can occur near the brainstem
bull Compression of the nerve more distally in the neck by an elongated styloid process (a bone in the neck) can cause this pain syndrome as well and this is called Eagle syndrome
bull Glossopharyngeal neuralgia can be caused by a tumor or infection in the region of ninth cranial nerve or by multiple sclerosis
bull httpwwwcolumbianeurosurgeryorgconditionsglossopharyngeal-neuralgia
Differential diagnosis of Glossopharyngeal Neuritis (IX)
bull The diagnosis is made on clinical grounds alone
bull no specific test can be done to prove the diagnosis in any given patient Episodic ear andor throat pain often triggered by touching the palate or tonsil is strongly suggestive of the disorder
bull High resolution MRI or CT imaging of the brainstem may reveal vascular compression tumors or demyelinating lesions involving the ninth cranial nerve
bull High resolution CT scanning of the neck can reveal an elongated styloid process suggestive of Eagle syndrome
bull Pain that distinctly involves the ear rear 13 of the tongue andor throat is the classic pain distribution seen in glossopharyngeal neuralgia (ninth cranial nerve or IX N)
httpwwwcolumbianeurosurgeryorgconditionsglossopharyngeal-neuralgia
Treatment of Glossopharyngeal Neuritisbull Most treatment for Glossopharyngeal Neuralgia is
pharmacological Anticonvulsant medications including oxcarbazepine carbamazepine gabapentin and pregabalin Some antidepressants can also be helpful either alone or in combination with the seizure medications If medications fail surgical intervention is indicated
bull The first-choice treatment microvascular decompression (MVD) as it has the highest initial and long-term success rates Patients unable to tolerate such an operation may consider one of the many less invasive options including Gamma Knife radiosurgery
bull Eagles syndrome TMJ and Trigeminal neuralgia can co-exist with glossopharyngeal neuritis and mask the diagnosis or be mistaken for it
httpwwwcolumbianeurosurgeryorgconditionsglossopharyngeal-neuralgia
Treatment of Sjogrenrsquosbull Conventional immunosuppressive therapies
such as corticosteroids have been used in some patients with variable benefit
bull Current therapy for dryness is principally symptomatic
bull The greatest experience in pSS is with rituximab an anti-CD20 (expressed on a subset of B-cells) monoclonal antibody
(Bowman S Barone F Biologic treatments in Sjoumlgrens syndrome Presse Med 2012 Sep41(9 Pt 2)e495-509)
Symptoms of Glossopharyngeal Neuritis
bull Glossopharyngeal neuralgia is classically described as ldquostabbingrdquo or ldquoelectrical shock-likerdquo in nature Episodes are typically brief lasting a couple of seconds up to a few minutes and can be quite debilitating They can occur in the tongue throat ear and tonsils on one side of the head but in rare cases may be bilateral Painful attacks may occur spontaneously but are more often associated with a specific stimulus such as chewing speaking coughing swallowing or laughing The attacks can occur frequently throughout the day or once every couple of weeks and they can also disturb sleep
bull httpwwwcolumbianeurosurgeryorgconditionsglossopharyngeal-neuralgia
Causes of Glossopharyngeal Neuralgiabull Neuralgia is typically caused by compression of the
glossopharyngeal nerve
bull Compression of the nerve by an adjacent artery andor vein can occur near the brainstem
bull Compression of the nerve more distally in the neck by an elongated styloid process (a bone in the neck) can cause this pain syndrome as well and this is called Eagle syndrome
bull Glossopharyngeal neuralgia can be caused by a tumor or infection in the region of ninth cranial nerve or by multiple sclerosis
bull httpwwwcolumbianeurosurgeryorgconditionsglossopharyngeal-neuralgia
Differential diagnosis of Glossopharyngeal Neuritis (IX)
bull The diagnosis is made on clinical grounds alone
bull no specific test can be done to prove the diagnosis in any given patient Episodic ear andor throat pain often triggered by touching the palate or tonsil is strongly suggestive of the disorder
bull High resolution MRI or CT imaging of the brainstem may reveal vascular compression tumors or demyelinating lesions involving the ninth cranial nerve
bull High resolution CT scanning of the neck can reveal an elongated styloid process suggestive of Eagle syndrome
bull Pain that distinctly involves the ear rear 13 of the tongue andor throat is the classic pain distribution seen in glossopharyngeal neuralgia (ninth cranial nerve or IX N)
httpwwwcolumbianeurosurgeryorgconditionsglossopharyngeal-neuralgia
Treatment of Glossopharyngeal Neuritisbull Most treatment for Glossopharyngeal Neuralgia is
pharmacological Anticonvulsant medications including oxcarbazepine carbamazepine gabapentin and pregabalin Some antidepressants can also be helpful either alone or in combination with the seizure medications If medications fail surgical intervention is indicated
bull The first-choice treatment microvascular decompression (MVD) as it has the highest initial and long-term success rates Patients unable to tolerate such an operation may consider one of the many less invasive options including Gamma Knife radiosurgery
bull Eagles syndrome TMJ and Trigeminal neuralgia can co-exist with glossopharyngeal neuritis and mask the diagnosis or be mistaken for it
httpwwwcolumbianeurosurgeryorgconditionsglossopharyngeal-neuralgia
Symptoms of Glossopharyngeal Neuritis
bull Glossopharyngeal neuralgia is classically described as ldquostabbingrdquo or ldquoelectrical shock-likerdquo in nature Episodes are typically brief lasting a couple of seconds up to a few minutes and can be quite debilitating They can occur in the tongue throat ear and tonsils on one side of the head but in rare cases may be bilateral Painful attacks may occur spontaneously but are more often associated with a specific stimulus such as chewing speaking coughing swallowing or laughing The attacks can occur frequently throughout the day or once every couple of weeks and they can also disturb sleep
bull httpwwwcolumbianeurosurgeryorgconditionsglossopharyngeal-neuralgia
Causes of Glossopharyngeal Neuralgiabull Neuralgia is typically caused by compression of the
glossopharyngeal nerve
bull Compression of the nerve by an adjacent artery andor vein can occur near the brainstem
bull Compression of the nerve more distally in the neck by an elongated styloid process (a bone in the neck) can cause this pain syndrome as well and this is called Eagle syndrome
bull Glossopharyngeal neuralgia can be caused by a tumor or infection in the region of ninth cranial nerve or by multiple sclerosis
bull httpwwwcolumbianeurosurgeryorgconditionsglossopharyngeal-neuralgia
Differential diagnosis of Glossopharyngeal Neuritis (IX)
bull The diagnosis is made on clinical grounds alone
bull no specific test can be done to prove the diagnosis in any given patient Episodic ear andor throat pain often triggered by touching the palate or tonsil is strongly suggestive of the disorder
bull High resolution MRI or CT imaging of the brainstem may reveal vascular compression tumors or demyelinating lesions involving the ninth cranial nerve
bull High resolution CT scanning of the neck can reveal an elongated styloid process suggestive of Eagle syndrome
bull Pain that distinctly involves the ear rear 13 of the tongue andor throat is the classic pain distribution seen in glossopharyngeal neuralgia (ninth cranial nerve or IX N)
httpwwwcolumbianeurosurgeryorgconditionsglossopharyngeal-neuralgia
Treatment of Glossopharyngeal Neuritisbull Most treatment for Glossopharyngeal Neuralgia is
pharmacological Anticonvulsant medications including oxcarbazepine carbamazepine gabapentin and pregabalin Some antidepressants can also be helpful either alone or in combination with the seizure medications If medications fail surgical intervention is indicated
bull The first-choice treatment microvascular decompression (MVD) as it has the highest initial and long-term success rates Patients unable to tolerate such an operation may consider one of the many less invasive options including Gamma Knife radiosurgery
bull Eagles syndrome TMJ and Trigeminal neuralgia can co-exist with glossopharyngeal neuritis and mask the diagnosis or be mistaken for it
httpwwwcolumbianeurosurgeryorgconditionsglossopharyngeal-neuralgia
Causes of Glossopharyngeal Neuralgiabull Neuralgia is typically caused by compression of the
glossopharyngeal nerve
bull Compression of the nerve by an adjacent artery andor vein can occur near the brainstem
bull Compression of the nerve more distally in the neck by an elongated styloid process (a bone in the neck) can cause this pain syndrome as well and this is called Eagle syndrome
bull Glossopharyngeal neuralgia can be caused by a tumor or infection in the region of ninth cranial nerve or by multiple sclerosis
bull httpwwwcolumbianeurosurgeryorgconditionsglossopharyngeal-neuralgia
Differential diagnosis of Glossopharyngeal Neuritis (IX)
bull The diagnosis is made on clinical grounds alone
bull no specific test can be done to prove the diagnosis in any given patient Episodic ear andor throat pain often triggered by touching the palate or tonsil is strongly suggestive of the disorder
bull High resolution MRI or CT imaging of the brainstem may reveal vascular compression tumors or demyelinating lesions involving the ninth cranial nerve
bull High resolution CT scanning of the neck can reveal an elongated styloid process suggestive of Eagle syndrome
bull Pain that distinctly involves the ear rear 13 of the tongue andor throat is the classic pain distribution seen in glossopharyngeal neuralgia (ninth cranial nerve or IX N)
httpwwwcolumbianeurosurgeryorgconditionsglossopharyngeal-neuralgia
Treatment of Glossopharyngeal Neuritisbull Most treatment for Glossopharyngeal Neuralgia is
pharmacological Anticonvulsant medications including oxcarbazepine carbamazepine gabapentin and pregabalin Some antidepressants can also be helpful either alone or in combination with the seizure medications If medications fail surgical intervention is indicated
bull The first-choice treatment microvascular decompression (MVD) as it has the highest initial and long-term success rates Patients unable to tolerate such an operation may consider one of the many less invasive options including Gamma Knife radiosurgery
bull Eagles syndrome TMJ and Trigeminal neuralgia can co-exist with glossopharyngeal neuritis and mask the diagnosis or be mistaken for it
httpwwwcolumbianeurosurgeryorgconditionsglossopharyngeal-neuralgia
Differential diagnosis of Glossopharyngeal Neuritis (IX)
bull The diagnosis is made on clinical grounds alone
bull no specific test can be done to prove the diagnosis in any given patient Episodic ear andor throat pain often triggered by touching the palate or tonsil is strongly suggestive of the disorder
bull High resolution MRI or CT imaging of the brainstem may reveal vascular compression tumors or demyelinating lesions involving the ninth cranial nerve
bull High resolution CT scanning of the neck can reveal an elongated styloid process suggestive of Eagle syndrome
bull Pain that distinctly involves the ear rear 13 of the tongue andor throat is the classic pain distribution seen in glossopharyngeal neuralgia (ninth cranial nerve or IX N)
httpwwwcolumbianeurosurgeryorgconditionsglossopharyngeal-neuralgia
Treatment of Glossopharyngeal Neuritisbull Most treatment for Glossopharyngeal Neuralgia is
pharmacological Anticonvulsant medications including oxcarbazepine carbamazepine gabapentin and pregabalin Some antidepressants can also be helpful either alone or in combination with the seizure medications If medications fail surgical intervention is indicated
bull The first-choice treatment microvascular decompression (MVD) as it has the highest initial and long-term success rates Patients unable to tolerate such an operation may consider one of the many less invasive options including Gamma Knife radiosurgery
bull Eagles syndrome TMJ and Trigeminal neuralgia can co-exist with glossopharyngeal neuritis and mask the diagnosis or be mistaken for it
httpwwwcolumbianeurosurgeryorgconditionsglossopharyngeal-neuralgia
Treatment of Glossopharyngeal Neuritisbull Most treatment for Glossopharyngeal Neuralgia is
pharmacological Anticonvulsant medications including oxcarbazepine carbamazepine gabapentin and pregabalin Some antidepressants can also be helpful either alone or in combination with the seizure medications If medications fail surgical intervention is indicated
bull The first-choice treatment microvascular decompression (MVD) as it has the highest initial and long-term success rates Patients unable to tolerate such an operation may consider one of the many less invasive options including Gamma Knife radiosurgery
bull Eagles syndrome TMJ and Trigeminal neuralgia can co-exist with glossopharyngeal neuritis and mask the diagnosis or be mistaken for it
httpwwwcolumbianeurosurgeryorgconditionsglossopharyngeal-neuralgia
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