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primary cough headache, primary exertional head-

ache, spontaneous intracranial hypotension, and

Alice in Wonderland syndrome. This review paper

will focus on the first four of these headache syn-

dromes, and include CC, which is also considered an

unusual headache. In fact, they all may not actually be

uncommon but rather under-recognized and/or

underreported in the literature.

METHODS

This review was initiated with a PubMed search

employing the terms unusual headache or unusual

headaches or uncommon headache or uncom-

mon headaches. After deciding which headache

syndromes upon which this review paper would con-

centrate, a second PubMed search was conducted for

each of the 5 disorders. For the term exploding headsyndrome, 21 records were returned, 23 records for

RES or erythromelalgia and ear, 25 records for

NTS, 47 records for numular headache or NH,

and 15 records for CC or cardiac cephalalgia. An

identical search was also performed, utilizing the

same terms, in the ISI Web of Knowledge, in an effort

to extend the comprehensiveness of the review. Some

additional papers were also found by reviewing the

references of published papers. All pertinent publica-

tions in scientific journals published in English-,

Spanish-, or the Portuguese-language, including origi-nal articles, reviews, meeting abstracts, and letters or

correspondences to the editors, with relevant infor-

mation on the subject matter were included. The

majority of evidence was in the form of case reports.

FINDINGS

Exploding Head Syndrome.Exploding head

syndrome (EHS) was first described by Armstrong-

Jones, in 1920, as a snapping of the brain.7 He

described a complaint by some patients of a sudden

crash or noise as if something had snapped or given

way on my brain. He associated this disorder with

the suffering of early melancholia, neurasthenia, and

psychastenia. Presently, some authors do not think

that this syndrome is related to neurosis,8 although

others state that it may be related to emotional tense-

ness, as many patients report a stressful life situation

in periods when attacks are intense and frequent.9

This syndrome is considered a benign sleepwake

transition disorder of unknown etiology. Attacks

occur predominantly in relaxed wakefulness or at the

transition from wakefulness to sleep;10 but less often

patients waken during the night with it and then fall

sleep.11 The attacks typically present with sudden loud

banging noises, like a bomb explosion or a gun

shot. There is no real headache, but patients are so

alarmed that at first they may inaccurately describe it

as a pain; upon closer questioning, there is no descrip-

tion of pain but rather a noise in the center or back of

the head.8 The attacks can be mild, but they are

usually so unpleasant and sometimes terrifying

to patients that they may raise concern in doctors

that an acute subarachnoid hemorrhage may have

occurred.10 Patients become concerned and may

develop tachycardia, sweating and have difficultybreathing.8,11 In 1020% of patients, attacks are

accompanied by a sensation of flashing lights.10 Some

cases of EHS are followed by a sleep paralysis.12,13

EHS may exacerbate migraine attacks, in patients

with chronic migraine.10

The pattern of episodes is variable. Some may

have 24 attacks followed by a prolonged, or total,

remission while others have frequent attacks, up to 7

in one night, for several nights each week.11 The

prevalence of EHS is unknown.

The differential diagnosis includes thunderclapheadaches, hypnic headache, migraine, cluster head-

ache, and paroxysmal hemicranias, as well as noctur-

nal nightmares and sleep myoclonus (sleep starts).13,14

Sacks and Svanborgn9 have recorded attacks

during polysomnography in 5 patients, all when they

were awake and relaxed. In 2 patients, they found an

alerting effect; the others had no electroencephalog-

raphy (EEG) changes. In 3 cases, the patients stated

afterwards that they had attacks when sleeping, but

there were no signs of sudden increasing of alertness

in EEG to support their reports. In another case,15 a

video-EEG showed that attacks correlated with the

transition from nonrapid eye movement (REM)

stage 1 sleep to wake. Some of the events were asso-

ciated with a hypnic jerk. A video- polysomnography

of 11 attacks showed the phenomenon occurring

during non-REM sleep stages 1 and 2, and that the

attacks were preceded by mild snoring.16

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The basis of this syndrome may be a delay in the

reduction of activity in selected areas of the brain-

stem reticular formation as the patient passes from

wakefulness to sleep.12 At the onset of sleep, the neu-

ronal activity in the brainstem reticular formation

subsides. This, in turn, switches off the motor, sensory,

visual, and auditory parts of the cerebral hemi-

spheres; if there is a delay in selected areas of the

reticular formation in switching off, then a paroxysm

of neuronal activity is manifested by the alarming

experiences, such as a loud noise, a flash of light, or a

myoclonus.17 Jacome18 has reported a case of a patient

with EHS and idiopathic stabbing headache relieved

by nifedipine, a calcium channel blocker, 90 mg a day,

speculating that EHS is perhaps a paroxysmal

symptom arising from transient calcium channel dys-

function, similar to the one observed in patients withepisodic ataxia and familial hemiplegic migraine that

results from a mutation in the CACNA1A gene.

To date, there have been no clinical trials of

therapies for EHS. In the majority of cases, firm reas-

surance that the condition is benign is appropriate;

patients are not treated with medication.8,14 In

patients who complain of sleeping difficulties due to

frequent attacks, clomipramine, 50 mg at night, has

been used with success.9 Topiramate, 200 mg per

day;15 nifedipine, 90 mg per day;18 flunarizine, 10 mg

per day;19 and imipramine 10 mg plus alprazolam0.25 mg, at night13 have been reported as effective.

Red Ear Syndrome.The RES was first described

by Lance, in 1994.20 This syndrome is commonly uni-

lateral and implies discomfort or burning pain as well

as change in color (erythema) that may involve the

adjacent skin of the cheek. The pain may radiate to

the cheek, forehead, a strip behind or below the man-

dible, the area behind the ear, occiput, and the upper

posterior aspect of the neck on the same side.21,22

Attacks may be either spontaneous or may be pre-

cipitated by exercise, stress, exposure to heat or cold,

touching the ear, drinking, coughing, chewing, sneez-

ing, or neck movement.23,24 The involvement, in fact,

may be unilateral or bilateral;23,25,26 symptoms are

often present beyond the ear, and color change is only

one of the many symptoms reported by patients.27 The

duration of the pain is variable, lasting from 10

minutes to many hours.22 The frequency is also vari-

able, from less than one per month to four per day.22

The cutaneous erythema should be observable to the

patient and the examiner, during the attack.28 Various

case reports have shown that the disorder need not

necessarily result in significant ear pain but rather

only redness of the ear.29 More than 80 cases have

been reported, so far. About 60% of the cases are in

women and 40% are in men.30

No association with primary headache disorders,

including migraine, hemicrania continua, chronic par-

oxysmal hemicrania, and SUNCT has been reported

in adults. Raieli et al23,31,32 have shown that this syn-

drome is very frequent (24%) in children and adoles-

cents with migraine with and without aura. In this

population, RES was significantly more prevalent in

males, with throbbing pain and vomiting.31 If patients

also experience migraine, RES may be in associationwith migraine attacks or not.22

Because of varied etiology, this entity may have

primary and secondary forms. RES may exist as an

idiopathic, primary trigeminal or cervical autonomic

cephalgia, it may coexist with migraine and other

trigeminal autonomic cephalgias,33 and there may be

secondary forms, in association upper cervical disor-

ders with irritation of the third cervical root, temporo-

mandibular joint dysfunction, glossopharyngeal, and

trigeminal neuralgia, or thalamic syndrome.21,29,30

Probably, the idiopathic forms are more frequent inchildren while the secondary ones are more common

in the elderly.24,34 In a child with systemic lupus

erythematosus, a thalamic lesion, caused by a vascu-

litis of the middle cerebral artery, was the most

probable cause of RES.35 Activation of the trigemi-

novascular system may represent a common patho-

physiologic mechanism of all etiologies,23 with an

antidromic discharge of impulses along the third cer-

vical root, and release of vasodilator peptides in the

ear, such as substance P, calcitonin gene-related

peptide, and nitric oxide, which are responsible for

pain and vasodilatation.21,27,30,36

Differential diagnoses of RES includes erysi-

pelas,herpes zoster, diffuse otitis externa (Pseudomo-

nas aeroginosa), streptococcal infections, borrelial

lymphocytoma, Hansens disease, erythromelalgia,

relapsing polychondritis, chondrodermatitis, nodu-

laris helices, cartilaginous pseudocyst, eczematous

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in the literature.In a hospital-based tertiary center,the

estimated incidence of NH was 6.4/100,000/year.59

The pain affects exclusively a circumscribed

cranial area,round (70%) or elliptical (30%), typically

with 16 cm of diameter, most at the parietal region,

and with no changes in size or shape with time. Pain is

usually mild to moderate (but maybe severe),continu-

ous, persisting for days to years, with lancinating exac-

erbations lasting for several seconds or gradually

increasing for 10 minutes to 2 hours.57 Exacerbations

commonly have a throbbing, electric, or stabbing

quality. They may be precipitated by touching the

symptomatic area,by combing the hair,by head move-

ments, or Valsalva maneuvers.59,60 Periods of remis-

sion, for weeks or months, are frequently reported.

Most patients also describe a combination of hypoes-

thesia, hyperesthesia, dysesthesia, tenderness, and/ortouch-evoked paresthesias.57,59,61 The pain is usually

unifocal, but bifocal and even multifocal cases have

been reported.60,62-66 Patients, when explaining their

symptoms, usually finger point to a specific area of the

head. Some patients have shown trophic changes

withinthe painful area,including thinnessof the skin,67

and one patient had hair heterochromia at the pain

site.68 There are a few cases reported with the painful

area larger than 6 cm of diameter up to 10 cm. 69,70

Some studies have shown evidence of increased sensi-

tivity a lower pain threshold with pressure algometry restricted to the symptomatic area, with no pericra-

nial tenderness in the remainder of the scalp, and with

no signs of a generalized pain state.71-73

The characteristics of clinical observations

suggest a non-psychogenic origin. Fernndez-de-las-

Peas and colleagues74 have shown that NH is not

associated with either depression or anxiety. A few

patients have reported mild head trauma previous to

the onset of NH.57,66,70 Many patients have also a past

or present diagnosis of migraine, but the pain from a

migraine headache can be easily distinguished from

the NH pain.57,70,75-78 Similar to menstrually related

migraine, there is a case report of menstrually related

NH.75

Focal headaches may result from a variety of

underlying causes, including metastatic disease or

myeloma of the skull, focal intracranial tumors

involving the dura mater, Pagets disease, and local

infection or inflammatory diseases.79 A diagnostic

evaluation should include a complete clinical and

neurological examination, laboratory tests, and neu-

roimaging studies.61 NH seems to be mainly a primary

disorder, but secondary forms have been described: a

fusiform extracranial aneurysm of the scalp,80 a

cranial wall lesion,61 post-transsphenoidal surgery for

pituitary adenoma,61 meningioma in the left posterior

fossa,81 arachnoid cysts,82 and Varicella-zoster virus

infection.83 Chen et al84 have noticed an extraordinar-

ily high (65%) association of NH with autoimmune

disorders, including Sjogren/sicca syndrome,

rheumatoid arthritis, and antiphospholipid antibody

syndrome.

The pathogenesis of NH is still not completely

known. The superficial location and consistent char-

acteristics of the pain, local sensory symptoms, thefocal increased sensitivity with algometry, and the

trophic changes confined to the affected area, provide

evidence for an epicranial source of the pain, stem-

ming from the bone, scalp, or pericranial nerves.59,85

Some authors, however, suggest that there may be

also NH of central origin because of some migrainous

features in some cases and the absence of pain relief

with local nerve blocks.78,86

Patients are usually more concerned with the

possibility of having a serious disease than with the

pain or with the sensory disturbances.57 To date, nospecific therapy was identified to be effective for most

patients. As there are a rather high percentage of

patients with spontaneous remission of the pain, we

should be careful when analyzing isolated good

results of any treatment.66 Local subcutaneous anes-

thetic injections are considered as not effective, but

occasionally it may work.65 NH may respond to

onabolulinumtoxinA, but the response is not univer-

sal.77,87,88 Gabapentin seems to be the most used pre-

ventive treatment for NH, although the results are

inconsistent.60,63,66,89-91 Other treatments potentially

helpful are naproxen,76 indomethacin,86 cyclobenza-

prine,75 nortriptyline,79 carbamazepine,69 lamot-

rigine,60 neurotropin,92 and transcutaneous

electrical nerve stimulation.93

Cardiac Cephalgia.The term CC was coined by

Lipton et al in 1997,94 as a form of exertional head-

ache. In the ICHD-II, it was included in group 10,

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headaches attributed to disorders of homeostasis.58

The association of headache and ischemic heart

disease, however, has been described at least since

1978.95 Bini et al in 2009,96 reviewing the literature,

compiled 30 cases of CC.After that, 6 cases have been

reported.97-101 Of the 36 cases, 21 (58.3%) were men

and 15 (41.7%) women.

CC usually occurs after 50 years of age, in subjects

at risk for cardiovascular diseases. They may have or

not a previous history of headache. The clinical char-

acteristic of the pain is not homogeneous. It may

involve any part of the head; it may be unilateral or

bilateral;it is generallysevere or excruciating,constric-

tive,and oppressive;but it may be similar to a migraine

headache, with accompanying symptoms such as pho-

tophobia, phonophobia, osmophobia, and nausea.96

Nausea is probably not prevalent enough (23%) to beconsidered an important accompanying symptom.102

Some cases have presented as a thunderclap head-

ache.97,100,103,104 Although most patients with CC were

older than 50 years, 22% of them (8/36) were younger

than that the youngest being 35 years old.95,98-100,104-107

CC is frequently related to exertion, even when mild,

such as walking. It is not obligatorily precipitated by

exercise. Cardiac symptoms, such as chest, mandible,

left arm, or epigastric pain are present in one half of

the patients.96 The frequency of headache is variable,

from bouts of a few weeks to several years.There are 4 theories as to the pathogenesis of

CC:96,97,105

1. Anginal pain is mediated by sympathetic fibers in

5060% of cases, by vagal fibers in 1020%, and by

both in 3040%. The convergence of autonomic

sensory fibers and trigeminal somatic fibers into

the descending trigeminal nucleus is responsible

for referred pain into the lower dental arch and in

the head; the convergence of autonomic and

somatic fibers in the upper cervical spinal cord

could cause referred pain in the occipital region.

2. The sudden reduction of cardiac output, caused

by cardiac ischemia, increases pressure in the left

ventricle and in the right atrium. Consequently,

there is a reduction of venous blood flow from

the brain, an elevation of intracranial pressure,

and distention of intracranial structures, causing

pain.

3. As a result of myocardial ischemia, there is a

release of neurochemical mediators (serotonin,

bradykinin,histamine, substance P, and atrial natri-

uretic factor), with potent vasodilating action in

the brain, causing headache.

4. CC could be secondary to a concomitant occur-

rence of vasospasm in both coronary and cerebral

vessels.

To make the diagnosis of CC, it is necessary to

have a medical history demonstrating the onset of

the headache in close temporal relationship with

acute myocardial ischemia. An electrocardiogram

(ECG) must be performed at rest and under stress.

The baseline ECG may be normal or show patho-

logical alterations (in 57%); these alterations may

only appear under stress ECG. Elevated cardiacmarkers (CPK-MB, myoglobin, and troponin) may

occur. In doubtful cases, coronary angiography can

confirm the diagnosis, by detecting coronary artery

stenosis.96 It is uncommon for myocardial ischemia

to produce headache as its only presenting symp-

tom.108 If CC manifests without cardiac symptoms,

physicians should think about this diagnosis when

patients are over 50 years of age, have significant

risk factors for cardiovascular diseases (male sex,

smoking, diabetes, hypertension, hypercholester-

olemia, and obesity), and the onset of headache isduring/after exertion.96,102,109

Distinguishing CC from migraine is of great

importance. Migraine-like characteristics of CC can

lead to use of vasoconstrictor agents (triptans, ergota-

mine, and dihydroergotamine), which are contraindi-

cated for these patients because of their ability to

constrict coronary arteries. CC usually responds to

therapy with nitrates, which are used to treat cardio-

vascular diseases.94,96,99,109-111 Nitrates, by vasodilating

cerebral arteries, may precipitate a headache

in normal subjects and a migraine attack in

migraineurs.112 The response to nitrates by abating

the headache corroborates the diagnosis of CC.110

Patients submitted to angioplasty or bypass surgery

find that both their cardiac condition and also the

headache resolve. CC may occur again, if there is

restenosis of the coronary artery.96,98,113 Potential risk

of mortality exists.106,110

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CONCLUSIONS

The ICHD-II comprises almost 200 headache

types and subtypes.58 Diagnosing and treating the

most common headache disorders are not so difficult.

Recognizing some unusual and uncommon head-

aches, either primary or secondary, may be a chal-

lenge for many non-headache specialist physicians.

Some of these syndromes are scarcely reported in the

literature. However, it is important to learn about

them because correct diagnosis may result in specific

treatments that improve the quality of life of these

patients and can even be life saving, such as in the

case of CC.

This paper reviewed the clinical characteristics,

pathogenesis, diagnosis, and the treatment of five

unusual, uncommon, and interesting headache syn-

dromes: EHS, RES, NTS, NH, and CC.Acknowledgment: The author wishes to thank Dr.

Alan M. Rapoport (University of California Los Angeles,

USA) for reviewing the manuscript.

STATEMENT OF AUTHORSHIP

Category 1

(a) Conception and Design

Luiz P. Queiroz

(b) Acquisition of Data

Luiz P. Queiroz(c) Analysis and Interpretation of Data

Luiz P. Queiroz

Category 2

(a) Drafting the Manuscript

Luiz P. Queiroz

(b) Revising It for Intellectual Content

Luiz P. Queiroz

Category 3(a) Final Approval of the Completed Manuscript

Luiz P. Queiroz

REFERENCES

1. Casucci G, dOnofrio F, Torelli P. Rare primary

headaches: Clinical insights. Neurol Sci. 2004;25

(Suppl. 3):S77-S83.

2. Bigal ME. Expert commentary-unusual headache

syndromes.Curr Pain Headache Rep. 2012;16:287-

288.

3. Sandrini G, Tassorelli C, Ghiotto N, Nappi G.

Uncommon primary headaches. Curr Opin Neurol.

2006;19:299-304.

4. Bamford CC, Mays M, Tepper SJ. Unusual head-aches in the elderly. Curr Pain Headache Rep.

2011;15:295-301.

5. Arruda MA, Albuquerque RCAP, Bigal ME.

Uncommon headache syndromes in the pediatric

population.Curr Pain Headache Rep. 2011;15:280-

288.

6. Evans RW. Case studies of uncommon headaches.

Neurol Clin. 2006;24:347-362.

7. Armstrong-Jones R. Snapping of the brain. Lancet.

1920;ii:720.

8. Pearce JM. Exploding head syndrome. Lancet.

1988;2:270-271.

9. Sachs C, Svanborg E. The exploding head syn-

drome: Polysomnographic recordings and thera-

peutic suggestions.Sleep. 1991;14:263-266.

10. Kallweit U, Khatami R, Bassetti CL. Exploding

head syndrome more than snapping of the

brain?Sleep Med. 2008;9:589.

11. Pearce JM. Clinical features of the exploding head

syndrome.J Neurol Neurosurg Psychiatry. 1989;52:

907-910.

12. Evans RW. Exploding head syndrome followed by

sleep paralysis: A rare migraine aura. Headache.2006;46:682-683.

13. Prez-Prez H,Prez-Lorensu PJ, Juan-Hernndez

P. Parlisis del sueo hipnopmpica con alucina-

ciones y sndrome de estallido ceflico: Una

asociacin infrecuente. Rev Neurol. 2010;51:255-

256.

14. Green MW. The exploding head syndrome. Curr

Pain Headache Rep. 2001;5:279-280.

15. Palikh GM, Vaughn BV. Topiramate responsive

exploding head syndrome.J Clin Sleep Med. 2010;

6:382-383.

16. Okura M, Taniguchi M, Muraki H, Sugita H, Ohi

M. Case of exploding head syndrome. Brain Nerve.

2010;62:85-88.

17. Evans RW, Pearce JM. Exploding head syndrome.

Headache. 2001;41:602-603.

18. Jacome DE. Exploding head syndrome and idio-

pathic stabbing headache relieved by nifedipine.

Cephalalgia. 2001;21:617-618.

18 January 2013

8/12/2019 Head 12002

8/11

19. Chakravarty A. Exploding head syndrome: Report

of two new cases.Cephalalgia. 2008;28:399-400.

20. Lance JW. The mystery of one red ear.Clin Exp

Neurol. 1994;31:13-18.

21. Lance JW. The red ear syndrome. Neurology.

1996;47:617-620.

22. Al-Din AS, Mir R, Davey R, Lily O, Ghaus N.Trigeminal cephalgias and facial pain syndromes

associated with autonomic dysfunction. Cephalal-

gia. 2005;25:605-611.

23. Raieli V, Monastero R, Santangelo G, Eliseo GL,

Eliseo M, Camarda R. Red ear syndrome and

migraine: Report of eight cases. Headache. 2002;

42:147-151.

24. Selekler M, Kutlu A, Uar S, Alma A. Immediate

response to greater auricular nerve blockade in red

ear syndrome.Cephalalgia. 2009;29:478-479.

25. Maranho-Filho PA, Vincent MB. The mystery of

two red ears: A bilateral simultaneous case.Ceph-

alalgia. 2011;31:1587.

26. Hirsch AR. Red ear syndrome.Neurology. 1997;

49:1190.

27. Kumar N, Swanson JW. The red ear syndrome

revisited: Two cases and a review of literature.

Cephalalgia. 2004;24:305-308.

28. Sklebar D, Sklebar I, Vrabec-Matkovic D, Cengic

L. Red ear syndrome chronic pain syndrome of

low prevalence.Acta Clin Croat. 2010;49:327-329.

29. Boulton P, Purdy RA, Bosch EP, Dodick DW.

Primary and secondary red ear syndrome: Implica-tions for treatment.Cephalalgia. 2007;27:107-110.

30. Eismann R, Gaul C, Wohlrab J, Marsch WC,

Fiedler E. Red ear syndrome: Case report and

review of the literature. Dermatology. 2011;223:

196-199.

31. Raieli V, Compagno A, Brighina F, et al. Preva-

lence of red ear syndrome in juvenile primary

headaches.Cephalalgia. 2011;31:597-602.

32. Raieli V, Pandolfi E, La Vecchia M, et al. The

prevalence of allodynia, osmophobia and red ear

syndrome in the juvenile headache: Preliminary

data.J Headache Pain. 2005;6:271-273.

33. Crach C, Duthel R, Barral F, et al. Positional

cluster-like headache. A case report of a neurovas-

cular compression between the third cervical root

and the vertebral artery. Cephalalgia. 2010;30:1509-

1513.

34. Donnet A, Valade D. The red ear syndrome. J

Neurol Neurosurg Psychiatry. 2004;75:1077.

35. Lee HS, Bae SC, Uhm WS, Jun JB, Lee IH, Kim

SY. Red ear(s) syndrome associated with child

neuropsychiatric systemic lupus erythematosus.

Lupus. 2000;9:301-303.

36. Evans RW, Lance JW. The red ear syndrome: An

auriculo-autonomic cephalgia. Headache. 2004;44:

835-836.37. Brill TJ, Funk B, Thai D, Kaufmann R. Red ear

syndrome and auricular erythromelalgia:The same

condition?Clin Exp Dermatol. 2009;34:e626-e628.

38. Ramirez CC, Kirsner RS. A refractory case of

erythromelalgia involving the ears.Am J Otolaryn-

gol. 2004;25:251-254.

39. Berk DR, Eisen AZ. Erythromelalgia of the ears:

An unusual variant and response to therapy. J

Drugs Dermatol. 2008;7:285-287.

40. Arjona A, Serrano-Castro PJ, Fernndez-Romero

E, Rub J. The red ear syndrome: Five new cases.

Cephalalgia. 2005;25:479-480.

41. Purdy RA, Dodick DW. Red ear syndrome.Curr

Pain Headache Rep. 2007;11:313-316.

42. Lance JW, Anthony M. Neck-tongue syndrome on

sudden turning of the head. J Neurol Neurosurg

Psychiatry. 1980;43:97-101.

43. Queiroz LP, Cavallazzi LO. Neck-tongue syn-

drome with twisting of the tongue: Report of two

cases.Cephalalgia. 1999;19:443-444.

44. Fortin CJ, Biller J. Neck tongue syndrome.Head-

ache. 1985;25:255-258.

45. Orrell RW, Marsden CD. The neck-tongue syn-drome. J Neurol Neurosurg Psychiatry. 1994;57:

348-352.

46. Elisevich K, Stratford J, Bray G, Finlayson M. Neck

tongue syndrome: Operative management. J

Neurol Neurosurg Psychiatry. 1984;47:407-409.

47. Wig S, Romanowski C, Akil M. An unusual cause

of the neck-tongue syndrome. J Rheumatol.

2009;36:857-858.

48. Sjaastad O, Bakketeig LS. Neck-tongue syndrome

and related (?) conditions. Cephalalgia. 2006;26:

233-240.

49. Belfort ES, Westerberg CE. Further observations

on the neck-tongue syndrome. Cephalalgia. 1985;

5(Suppl. 3):312-313.

50. Lewis DW, Frank LM, Toor S. Familial neck-

tongue syndrome.Headache. 2003;43:132-134.

51. Bogduk N. An anatomical basis for the neck-

tongue syndrome.J Neurol Neurosurg Psychiatry.

1981;44:202-208.

Headache 19

8/12/2019 Head 12002

9/11

52. Tsakotos GA, Anagnostopoulou SI, Evangelopou-

los DS, Vasilopoulou M, Kontovazenitis PI, Korres

SD. Arnolds ligament and its contribution to the

neck-tongue syndrome. Eur J Orthop Surg Trau-

matol. 2007;17:527-531.

53. Noda S,Umezaki H.Spinal neck-tongue syndrome.

J Neurol Neurosurg Psychiatry. 1984;47:751.54. Wong S, Paviour DC, Clifford-Jones RE. Chiari-1

malformation and the neck-tongue syndrome:

Cause or coincidence? Cephalalgia. 2008;28:994-

995.

55. Webb J, March L, Tyndall A. The neck-tongue syn-

drome: Occurrence with cervical arthritis as well as

normals.J Rheumatol. 1984;11:530-533.

56. Chedrawi AK, Fishman MA, Miller G. Neck-

tongue syndrome. Pediatr Neurol. 2000;22:397-

399.

57. Pareja JA, Caminero AB, Serra J, et al. Nummular

headache: A coin-shaped cephalgia. Neurology.

2002;58:1678-1679.

58. Headache Classification Subcommittee of the

International Headache Society. The international

classification of headache disorders: 2nd edition.

Cephalalgia. 2004;24(Suppl. 1):9-160.

59. Pareja JA, Pareja J, Barriga FJ, et al. Nummular

headache: A prospective series of 14 new cases.

Headache. 2004;44:611-614.

60. Guerrero AL, Cortijo E, Herrero-Velzquez S,

et al. Nummular headache with and without exac-

erbations: Comparative characteristics in a series of72 patients.Cephalalgia. 2012;32:649-653.

61. Alvaro LC, Garca JM,Areitio E. Nummular head-

ache:A series with symptomatic and primary cases.

Cephalalgia. 2009;29:379-383.

62. Cuadrado ML, Valle B, Fernndez-de-las-Peas C,

Barriga FJ, Pareja JA. Bifocal nummular headache:

The first three cases. Cephalalgia. 2009;29:583-

586.

63. Guerrero AL, Cuadrado ML, Garca-Garca ME,

et al. Bifocal nummular headache: A series of 6

new cases.Headache. 2011;51:1161-1166.

64. Porta-Etessam J, Lapea T, Cuadrado M,Guerrero

A, Parejo B. Multifocal nummular headache with

trophic changes.Headache. 2010;50:1612-1613.

65. Rocha-Filho PAS. Nummular headache: Two

simultaneous areas of pain in the same patient.

Cephalalgia. 2011;31:874.

66. Cortijo E, Guerrero-Peral AL, Herrero-Velazquez

S, et al. Nummular headache: Clinical features and

therapeutic experience in a series of 30 new cases.

Rev Neurol. 2011;52:72-80.

67. Pareja JA, Cuadrado ML, Fernndez-de-las Peas

C, Nieto C, Sols M, Pinedo F. Nummular headache

with trophic changes inside the painful area. Ceph-

alalgia. 2008;28:186-190.

68. Dabscheck G, Andrews PI. Nummular headacheassociated with focal hair heterochromia in a child.

Cephalalgia. 2010;30:1403-1405.

69. Man Y, Yu T, Li L, Yao G, Mao X, Wu J. A new

variant nummular headache: Large diameter

accompanied with bitrigeminal hyperalgesia and

successful treatment with carbamazepine. Turk

Neurosurg. 2012;22:506-509.

70. Moon J, Ahmed K, Garza I. Case series of sixteen

patients with nummular headache. Cephalalgia.

2010;30:1527-1530.

71. Cuadrado ML, Valle B, Fernndez-de-las-Peas C,

et al. Pressure pain sensitivity of the scalp in

patients with nummular headache: A cartographic

study.Cephalalgia. 2010;30:200-206.

72. Fernndez-de-las-Peas C, Cuadrado ML, Barriga

FJ, Pareja JA. Pericranial tenderness is not related

to nummular headache. Cephalalgia. 2007;27:182-

186.

73. Fernndez-de-Las-Peas C,Cuadrado ML, Barriga

FJ, Pareja JA. Local decrease of pressure pain

threshold in nummular headache.Headache. 2006;

46:1195-1198.

74. Fernndez-de-Las-Peas C, Peacoba-Puente C,Lpez-Lpez A, et al. Depression and anxiety are

not related to nummular headache. J Headache

Pain. 2009;10:441-445.

75. Robbins MS, Grosberg BM. Menstrual-related

nummular headache. Cephalalgia. 2010;30:507-

508.

76. Monzillo PH, Lima Neto MM, Sanvito WL, Costa

ARD, Saab VM. Nummular headache: Case

report.Arq Neuropsiquiatr. 2004;62:903-905.

77. Ruscheweyh R, Buchheister A, Gregor N, Jung A,

Evers S. Nummular headache: Six new cases and

lancinating pain attacks as possible manifestation.

Cephalalgia. 2010;30:249-253.

78. Dach F, Speciali J, Eckeli A, Rodrigues GG,

Bordini CA. Nummular headache: Three new

cases.Cephalalgia. 2006;26:1234-1237.

79. Grosberg BM, Solomon S, Lipton RB. Nummular

headache. Curr Pain Headache Rep. 2007;11:310-

312.

20 January 2013

8/12/2019 Head 12002

10/11

80. Garca-Pastor A, Guillem-Mesado A, Salinero-

Paniagua J, Gimnez-Roldn S. Fusiform aneu-

rysm of the scalp: An unusual cause of focal

headache in Marfan syndrome. Headache. 2002;42:

908-910.

81. Guillem A, Barriga FJ, Gimnez-Roldn S. Num-

mular headache secondary to an intracranial masslesion.Cephalalgia. 2007;27:943-944.

82. Guillem A, Barriga FJ, Gimnez-Roldn S. Num-

mular headache associated to arachnoid cysts. J

Headache Pain. 2009;10:215-217.

83. Chen W, Li T, Lee L, Huang C. Varicella-zoster

virus infection and nummular headache:A possible

association with epicranial neuralgia. Intern Med.

2012;51:2439-2441.

84. Chen W, Chen Y, Lin C, Li T, Lee L, Chen C. A

high prevalence of autoimmune indices and disor-

ders in primary nummular headache.J Neurol Sci.

2012;320:127-130.

85. Pareja JA, Pareja J. Nummular headache: Diagno-

sis and treatment. Expert Rev Neurother. 2003;3:

289-292.

86. Baldacci F, Nuti A, Lucetti C, Borelli P, Bonuccelli

U. Nummular headache dramatically responsive to

indomethacin.Cephalalgia. 2010;30:1151-1152.

87. Mathew NT, Kailasam J, Meadors L. Botulinum

toxin type A for the treatment of nummular head-

ache: Four case studies. Headache. 2008;48:442-

447.

88. Dusitanond P, Young W. Botulinum toxin type Asefficacy in nummular headache. Headache. 2008;48:

1379.

89. Trucco M. Nummular headache: Another case

treated with gabapentin.J Headache Pain. 2007;8:

137-138.

90. Trucco M, Mainardi F, Perego G, Zanchin G. Num-

mular headache: First Italian case and therapeutic

proposal.Cephalalgia. 2006;26:354-356.

91. Evans RW, Pareja JA. Nummular headache. Head-

ache. 2005;45:164-165.

92. Yamazaki Y, Kobatake K. Successful treatment of

nummular headache with Neurotropin.J Head-

ache Pain. 2011;12:661-662.

93. Tayeb Z, Hafeez F, Shafiq Q. Successful treatment

of nummular headache with TENS. Cephalalgia.

2008;28:897-898.

94. Lipton RB, Lowenkopf T, Bajwa ZH, et al. Cardiac

cephalgia:A treatable form of exertional headache.

Neurology. 1997;49:813-816.

95. Caskey WH, Spierings EL. Headache and heart-

ache.Headache. 1978;18:240-243.

96. Bini A, Evangelista A, Castellini P, et al. Cardiac

cephalgia.J Headache Pain. 2009;10:3-9.

97. Cheng P, Sy H, Chen W, Chen Y. Cardiac cephalal-

gia presented with a thunderclap headache and an

isolated exertional headache: Report of 2 cases.Acta Neurol Taiwan. 2010;19:57-61.

98. Saikia PK, Chakrabarti D. Headache as the sole

presenting symptom of acute myocardial infarc-

tion.J Assoc Physicians India. 2009;57:83-84.

99. Yang Y, Jeong D, Jin DG, et al. A case of cardiac

cephalalgia showing reversible coronary vaso-

spasm on coronary angiogram.J Clin Neurol. 2010;

6:99-101.

100. Dalzell JR, Jackson CE, Robertson KE, McEnte-

gart MB, Hogg KJ. A case of the heart ruling the

head: Acute myocardial infarction presenting with

thunderclap headache. Resuscitation. 2009;80:608-

609.

101. Costopoulos C. Acute coronary syndromes can be

a headache.Emerg Med J. 2011;28:71-73.

102. Chen S, Fuh J, Yu W, Wang S. Cardiac cephalalgia.

Case report and review of the literature with new

ICHD-II criteria revisited. Eur Neurol. 2004;51:

221-226.

103. Broner S, Lay C, Newman L, Swerdlow M. Thun-

derclap headache as the presenting symptom of

myocardial infarction.Headache. 2007;47:724-725.

104. Seow V, Chong C, Wang T, Ong J. Severe explosiveheadache: A sole presentation of acute myocardial

infarction in a young man. Am J Emerg Med.

2007;25:250-251.

105. Wei J, Wang H. Cardiac cephalalgia: Case reports

and review.Cephalalgia. 2008;28:892-896.

106. Auer J, Berent R, Lassnig E, Eber B. Headache as

a manifestation of fatal myocardial infarction.

Neurol Sci. 2001;22:395-397.

107. Blacky RA, Rittelmeyer JT, Wallace MR. Head-

ache angina.Am J Cardiol. 1987;60:730.

108. Sathirapanya P. Anginal cephalgia: A serious form

of exertional headache. Cephalalgia. 2004;24:231-

234.

109. Gutirrez-Morlote J, Pascual J. Cardiac cephalgia

is not necessarily an exertional headache: Case

report.Cephalalgia. 2002;22:765-766.

110. Gutirrez Morlote J, Fernndez Garca JM,

Timiraos Fernndez JJ, Llano Cardenal M,

Rodrguez Rodrguez E, Pascual Gmez J. Cardiac

Headache 21

8/12/2019 Head 12002

11/11

cephalgia:An underdiagnosed condition? . Rev Esp

Cardiol. 2005;58:1476-1478.

111. Martnez HR, Rangel-Guerra RA, Cant-Martnez

L, Garza-Gmez J, Gonzlez HC. Cardiac head-

ache: Hemicranial cephalalgia as the sole manifes-

tation of coronary ischemia. Headache. 2002;42:

1029-1032.

112. Famularo G, Polchi S, Tarroni P. Headache as a

presenting symptom of acute myocardial infarc-

tion.Headache. 2002;42:1025-1028.

113. Grace A, Horgan J, Breathnach K, Staunton H.

Anginal headache and its basis. Cephalalgia. 1997;

17:195-196.

22 January 2013