Neruro-ophthalmic Causes of Headache

Raed Behbehani , MD FRCSC

Pain•Periocular pain due to diseases of the face, orbit, sinuses , and intracranial cavity.•Trigeminal innervation (V1-V3).

Approach• Any headache can cause eye pain (and vice versa).

• Take good history ( loss of vision, diplopia, transient visual obscurrations, redness, photophobia, jaw claudication, systemic symptoms).

• Examination : check vision at least grossly, look for redness, ptosis, corneal edema, check pupil reactions, palpate the eyes and orbits, check sensation v1-v3 and other cranial nerves.•FUNDOSCOPY !

Primary Headache Syndrome• Migraine (with / without aura)•Cluster Headache .•Tension Headache.•Chronic Daily Headache.•Medication overuse.

Secondary Headache Syndrome• Ocular disease ( dry eye, uveitis, acute glaucoma).• Orbital disease (Thyroid eye disease, idiopathic orbital inflammatory disease).• Vasculitis ( Giant cell arteritis)• High intracranial pressure (Pseuotumor cerebri , cerebral venous sinus thrombosis)

Ocular Disease

Dry eye syndrome• Inadequate tear production.• Primary / Secondary to rheumatological conditions.• Slit lamp examination : Flourescin stain/ Rose bengal• Artificial tears/ punctal occlusion is the treatment.

Uveitis• Anterior/Posterior Uveitis.• Pain and Photophobia.• Cells in the anterior chamber/ Ciliary injection/ Posterior synechiae.• Idiopathic/ associated with rheumatologic conditions/ infectious (post-operative).

Acute Angle-Closure Glaucoma• Severe periocular pain +- headache.• Blurred vision , nausea , and vomiting.• Cilliary injection/ corneal edema/ fixed mid-dilated pupil.• Previous history of transient visual disturbances .

70 year old patient with acute loss of vision , temporal headache, jaw claudication , fever and weight loss.

Giant-Cell Arteritis• New onset of headache (temporal) , acute or transient loss of vision, jaw claudication, weight loss, fever, and myalgias.• Age usually over 60.• Occult GCA ( No systemic symptoms).

GCAArteritic ischmeic opic neuropathy.

GCACentral retinal artery occlusion/ Branch retinal artery occlusion

GCAOphthalmoplegia

Giant Cell Arteritis (GCA)

Stat ESR , CRP and CBC (platelets).ESR can be normal in 15-20% of cases.CRP is more sensitive and specific.CRP and CBC have 97% sensitivity and specifity.Start high dose systemic steroids (IV or Oral ) immediately upon suspicioun ! Arrange for temporal artery biopsy within 2 weeks , while patient is on steroids.

Temporal Artery Biopsy

Temporal Artery Biopsy (TAB)

Temporal Arteritis• Treatment is long term high dose systemic steroids (1-2 years)

• Rheumatological consultation to rule out systemic involvement ( aortic aneurysm or dissection)

• Follow up clinically and with CRP and ESR to titrate steroid dose

• Protention against steroid complications ( diabetes, osteoporosis)

Orbital Disease• Optic neuritis.• Orbital inflammtory disease.

Optic Neuritis• Dull , aching pain worse with eye movements.• Loss of vision.• Pupil testing : relative afferent pupillary defect (RAPD).• Loss of color vision (Dyschromatopsia).• Fundus : optic disc normal in 70% (retrobulbar optic neuritis).

Orbital inflammation• Sudden onset. • Pain, proptosis, limited eye movement, chemosis.• Idiopathic or due to Wegener’s granulmatosis, Grave s’ disease, sarcoidosis)

25 year old lady with new onset of headache, diplopia, and transient visual obscurration. She is overwight and has gained about 10 Kg over the last 6 months.

Idiopathic Intracranial Hypertension (pseudotumor cerebri)

• Headache, pain in the neck and shoulders and upper back.• Worse with coughing/straining.• Pulsatile tinnitis.•Transient visual obscurations.• Diplopia ( Abducens nerve palsy )

Modified Dandy Criteria

1) Symptoms of raised intracranial pressure (headache, nausea, vomiting, transient visual obscurations, or papilledema)

2) No localizing signs with the exception of abducens (sixth) nerve palsy

3) The patient is awake and alert 4) Normal CT/MRI findings without evidence of

thrombosis 5) LP opening pressure of >25 cmH2O and normal

biochemical and cytological composition of CSF 6 No other explanation for the raised intracranial pressure

IIH Treatment• Medical ( Diuretics): Acetazolmide , Freusomide • Surgical :

visual loss Optic nerve sheath fenstration.

headache and vision loss ventriculoperitoneal or lumboperitoneal shunt.• Cerebral venous sinus thrombosis : Anti-coauglants (warfarin) , ? venous stenting.

Optic Nerve Sheath Fenestration (ONSF)

Summary• Take good history ( try to distinguish primary from secondary headache syndrome).• Look for abnormal neuro-ophthalmic signs ( Ptosis, ophthalmoplegia, abnormal facial sensation, check visual acuity, and pupils, and look for papilledema).• Giant cell arteritis is vision-threatening.• Papilledema ican be life threatening.