atlas of uncommon pain syndromes. expert consult - online and print

8/20/2019 Atlas of Uncommon Pain Syndromes. Expert Consult - Online and Print

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THE CLINICAL S YNDROME

Ice pick headache is a constellation of symptoms consisting ofparoxysms of stabbing jabs and jolts that occur primarily inthe first division of the trigeminal nerve. These paroxysms ofpain may occur as a single jab or a series of jabs that last for afraction of a second followed by relatively pain-free episodes.The pain of ice pick headache occurs in irregular intervals ofhours to days. Similar to cluster headache, ice pick headache isan episodic disorder that is characterized by “clusters” of pain-

ful attacks followed by pain-free periods. Episodes of ice pickheadache usually occur on the same side, but in rare patients,the pain may move to the same anatomical region on the con-tralateral side. Ice pick headache occurs more commonly in

women and is generally not seen before the fourth decade oflife, but rare reports of children suffering from ice pick head-ache sporadically appear in the literature. Synonyms for icepick headache include jabs and jolts headache and idiopathicstabbing headache.

SIGNS AND S YMPTOMS

A patient suffering from ice pick headache complains of jolts or jabs of pain in the orbit, temple, or parietal region (Figure 1-1).

Some patients describe the pain of ice pick headache as a suddensmack or slap on the side of the head. Similar to patients suffer-ing from trigeminal neuralgia, a patient suffering from ice pickheadache may exhibit involuntary muscle spasms of the affectedarea in response to the paroxysms of pain. In contrast to tri-geminal neuralgia, involving the first division of the trigeminalnerve, there are no trigger areas that induce the pain of ice pickheadache. The neurological examination of a patient sufferingfrom ice pick headache is normal. Some patients exhibit anxietyand depression because the intensity of pain associated with icepick headache leads many patients to believe they have a braintumor.

TESTING

Magnetic resonance imaging (MRI) of the brain provides the be

information regarding the cranial vault and its contents. MRIhighly accurate and helps identify abnormalities that may put tpatient at risk for neurological disasters secondary to intracranand brainstem pathological conditions, including tumors andemyelinating disease (Figure 1-2). Magnetic resonance angioraphy (MRA) also may be useful in helping identify aneurysm

which may be responsible for the patient’s neurological findingIn patients who cannot undergo MRI, such as a patient withpacemaker, computed tomography (CT) is a reasonable seconchoice. Radionuclide bone scanning and plain radiography aindicated if fracture or bony abnormality, such as metastatic dease, is considered in the differential diagnosis.

Chapter 1

ICE PICK HEADACHE

ICD-9 CODE 784.0

ICD-10 CODE R51

SECTION 1 • Headache and Facial Pain Syndromes

Figure 1-1 Ice pick headache is characterized by jabs or jolts in torbit, temple, or parietal region.

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2 SECTION 1 • Headache and Facial Pain Syndromes

Screening laboratory tests consisting of complete blood cellcount, erythrocyte sedimentation rate, and automated blood

chemistry should be performed if the diagnosis of ice pick head-ache is in question. Intraocular pressure should be measured ifglaucoma is suspected.

DIFFERENTIAL DIAGNOSIS

Ice pick headache is a clinical diagnosis supported by a combi-nation of clinical history, normal physical examination, radiogra-phy, and MRI. Pain syndromes that may mimic ice pick headacheinclude trigeminal neuralgia involving the first division of thetrigeminal nerve, demyelinating disease, and chronic paroxysmalhemicrania. Trigeminal neuralgia involving the first division ofthe trigeminal nerve is uncommon and is characterized by trig-ger areas and tic-like movements. Demyelinating disease is gener-

ally associated with other neurological findings, including opticneuritis and other motor and sensory abnormalities. The pain of

chronic paroxysmal hemicrania lasts much longer than the pain ofice pick headache and is associated with redness and watering ofthe ipsilateral eye.

TREATMENT

Ice pick headache uniformly responds to treatment with indo-methacin. Failure to respond to indomethacin puts the diagnosisof ice pick headache in question. A starting dosage of 25 mg dailyfor 2 days and titrating to 25 mg three times per day is a reason-able treatment approach. This dose may be carefully increasedto 150 mg per day. Indomethacin must be used carefully, if atall, in patients with peptic ulcer disease or impaired renal func-tion. Anecdotal reports of a positive response to cyclooxygenase-2(COX-2) inhibitors in the treatment of ice pick headache havebeen noted in the headache literature. Underlying sleep distur-bance and depression are best treated with a tricyclic antidepres-sant compound, such as nortriptyline, which can be started at asingle bedtime dose of 25 mg.

COMPLICATIONS AND PITFALLS

Failure to correctly diagnose ice pick headache may put the patientat risk if intracranial pathological conditions or demyelinating dis-ease, which may mimic the clinical presentation of chronic parox-ysmal hemicrania, is overlooked. MRI is indicated in all patientsthought to be suffering from ice pick headache. Failure to diag-nose glaucoma, which also may cause intermittent ocular pain,may result in permanent loss of sight.

SUGGESTED READINGS

Cutrer FM, Boes CJ: Cough, exertional, and sex headaches, Neurol Clin 22:133–149, 2004.

Dafer RM: Neurostimulation in headache disorders, Neurol Clin 28:835–841,2010.

Mathew NT: Indomethacin responsive headache syndromes: headache, J HeadFace Pain 21:147–150, 1981.

Pascual J: Other primary headaches, Neurol Clin 27:557–571, 2009.Tuğba T, Serap Ü, Esra O, et al: Features of stabbing, cough, exertional and sex-

ual headaches in a Turkish population of headache patients, J Clin Neurosci 15:774–777, 2008.

Figure 1-2 Diffuse pachymeningeal and calvarial metastasis from car-cinoma of the breast. Axial T1-weighted postgadolinium MRI showsdiffuse nodular and bandlike contrast-enhanced thickening of the duraover the high right frontoparietal convexity. (From Haaga JR, LanzieriCF, Gilkeson RC, editors: CT and MR imaging of the whole body, 4th ed,Philadelphia, 2003, Mosby, p 198.)

Clinical Pearls

The diagnosis of ice pick headache is made by obtaininga thorough, targeted headache history. Patients sufferingfrom ice pick headache should have a normal neurologicalexamination. If the results of the neurological examinationare abnormal, the diagnosis of ice pick headache should bediscarded and a careful search for the cause of the neurologi-cal findings should be undertaken.


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The pain of supraorbital neuralgia is characterized as persistentpain in the supraorbital region and forehead with occasional sud-den, shocklike paresthesias in the distribution of the supraorbitalnerves. Sinus headache involving the frontal sinuses, which ismuch more common than supraorbital neuralgia, can mimic thepain of supraorbital neuralgia. Supraorbital neuralgia is the resultof compression or trauma of the supraorbital nerves as the nervesexit the supraorbital foramen. Such trauma can be in the form ofblunt trauma directly to the nerve, such as when the forehead hitsthe steering wheel during a motor vehicle accident, or repetitivemicrotrauma resulting from wearing welding or swim goggles thatare too tight. This clinical syndrome also is known as swimmer’sheadache.

SIGNS AND S YMPTOMS

The supraorbital nerve arises from fibers of the frontal nerve, which is the largest branch of the ophthalmic nerve. The frontalnerve enters the orbit via the superior orbital fissure and passesanteriorly beneath the periosteum of the roof of the orbit. Thefrontal nerve gives off a larger lateral branch, the supraorbitalnerve, and a smaller medial branch, the supratrochlear nerve. Bothexit the orbit anteriorly. The supraorbital nerve sends fibers allthe way to the vertex of the scalp and provides sensory innerva-tion to the forehead, upper eyelid, and anterior scalp (Figure 2-1).The pain of supraorbital neuralgia is characterized as persistentpain in the supraorbital region and forehead with occasional sud-

den, shocklike paresthesias in the distribution of the supraorbitalnerves. Occasionally, a patient suffering from supraorbital neu-ralgia complains that the hair on the front of the head “hurts”(Figure 2-2). Supraorbital nerve block is useful in the diagnosisand treatment of supraorbital neuralgia.

TESTING

Magnetic resonance imaging (MRI) of the brain provides the bestinformation regarding the cranial vault and its contents. MRI ishighly accurate and helps identify abnormalities that may put thepatient at risk for neurological disasters secondary to intracranial

and brainstem pathological conditions, including tumors andemyelinating disease (Figure 2-3). Magnetic resonance angioraphy (MRA) also may be useful in helping identify aneurysm

which may be responsible for the patient’s neurological findingIn patients who cannot undergo MRI, such as a patient withpacemaker, computed tomography (CT) is a reasonable seconchoice. Radionuclide bone scan, CT, and plain radiography aindicated if sinus disease, fracture, or bony abnormality such

metastatic disease is considered in the differential diagnosis.Screening laboratory tests consisting of complete blood ccount, erythrocyte sedimentation rate, and automated bloochemistry testing should be performed if the diagnosis of supraobital neuralgia is in question. Intraocular pressure should be mesured if glaucoma is suspected (Figure 2-4).


Supraorbital neuralgia is a clinical diagnosis supported by a combnation of clinical history, normal physical examination, radiogrphy, CT, and MRI. Pain syndromes that may mimic supraorbi

Chapter 2

SUPRAORBITAL NEURALGIA

ICD-9 CODE 350.1

ICD-10 CODE G50.0

Inflamedsupraorbital n.

Figure 2-1 The supraorbital nerve sends fibers all the way to the vertof the scalp and provides sensory innervation to the forehead, uppeyelid, and anterior scalp. n, Nerve.

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neuralgia include ice pick headache, trigeminal neuralgia involv-ing the first division of the trigeminal nerve, demyelinating dis-ease, and chronic paroxysmal hemicrania. Trigeminal neuralgiainvolving the first division of the trigeminal nerve is uncommonand is characterized by trigger areas and tic-like movements.Demyelinating disease is generally associated with other neurolog-ical findings, including optic neuritis and other motor and sensory

abnormalities. The pain of chronic paroxysmal hemicrania lastsmuch longer than the paroxysmal pain of supraorbital neuralgiaand is associated with redness and watering of the ipsilateral eye.

TREATMENT

The primary treatment intervention for supraorbital neuralgia isthe identification and removal of anything causing compressionof the supraorbital nerves (e.g., tight welding or swim goggles).

A brief trial of simple analgesics alone or in combination withgabapentin also should be considered. For patients who do notrespond to these treatments, supraorbital nerve block with localanesthetic and a steroid is a reasonable next step.

To perform supraorbital nerve block, the patient is placed in

the supine position. Using a 10-mL sterile syringe, 3 mL of localanesthetic is drawn up. When treating supraorbital neuralgia with

supraorbital nerve block, 80 mg of depot steroid is added to thelocal anesthetic with the first block, and 40 mg of depot steroid isadded with subsequent blocks.

The supraorbital notch on the affected side is identified bypalpation. The skin overlying the notch is prepared with anti-septic solution, with care taken to avoid spillage into the eye. A25-gauge, 1½-inch needle is inserted at the level of the supraor-bital notch and is advanced medially approximately 15 degreesoff the perpendicular to avoid entering the foramen. The needleis advanced until it approaches the periosteum of the underlyingbone (Figure 2-5). A paresthesia may be elicited, and the patientshould be warned of such. The needle should not enter the supra-orbital foramen; if this occurs, the needle should be withdrawnand redirected slightly more medially.

Because of the loose alveolar tissue of the eyelid, a gauze spongeshould be used to apply gentle pressure on the upper eyelid andsupraorbital tissues before injection of solution to prevent theinjectate from dissecting inferiorly into these tissues. This pres-sure should be maintained after the procedure to avoid periorbitalhematoma and ecchymosis.

After gentle aspiration, 3 mL of solution is injected in a fanlikedistribution. If blockade of the supratrochlear nerve also is desired,

the needle is redirected medially and, after careful aspiration, anadditional 3 mL of solution is injected in a fanlike manner.Underlying sleep disturbance and depression associated with

the pain of supraorbital neuralgia are best treated with a tricy-clic antidepressant compound, such as nortriptyline. The tricyclicantidepressant can be started at a single bedtime dose of 25 mg.


Failure to diagnose supraorbital neuralgia correctly may put thepatient at risk if an intracranial pathological condition or demy-elinating disease, which may mimic the clinical presentation ofsupraorbital neuralgia, is overlooked. MRI is indicated in allpatients thought to have supraorbital neuralgia. Failure to diag-

nose glaucoma, which also may cause intermittent ocular pain,may result in permanent loss of sight.The forehead and scalp are highly vascular, and when per-

forming supraorbital nerve block the clinician should carefullycalculate the total milligram dosage of local anesthetic that maybe given safely, especially if bilateral nerve blocks are being per-formed. This vascularity gives rise to an increased incidence ofpostblock ecchymosis and hematoma formation. Despite the

vascularity of this anatomical region, this technique can be per-formed safely in the presence of anticoagulation by using a 25-or 27-gauge needle, albeit at increased risk for hematoma, if theclinical situation dictates a favorable risk-to-benefit ratio. Thesecomplications can be decreased if manual pressure is applied tothe area of the block immediately after injection. Application of

cold packs for 20-minute periods after the block also decreases theamount of postprocedure pain and bleeding.

Figure 2-2 Occasionally, a patient with supraorbital neuralgia com-plains that the hair on the front of the head “hurts.” The supraorbitalnerve sends fibers all the way to the vertex of the scalp and providessensory innervation to the forehead, upper eyelid, and anterior scalp.

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SUGGESTED READINGS

Levin M: Nerve blocks and nerve stimulation in headache disorders, Tech Reg

Anesth Pain Manage 13:42–49, 2009.Levin M: Nerve blocks in the treatment of headache, Neurotherapeutics 7:197–

203, 2010. Waldman SD: The trigeminal nerve. In Waldman SD, editor: Pain review , Phila-

delphia, 2009, Saunders, pp 15–17. Waldman SD: Swimmer’s headache. In Waldman SD, editor: Atlas of pain man-

agement injection techniques , Philadelphia, 2007, Saunders, pp 7–10.

Hazy corneal reflexsignifying edema

Fixedsemidilatedpupil

Cataractous

lens

Opaque thickenededematous cornea

Shallow anteriorchamber

Cataractous lens

Figure 2-4 Acute angle closure resulting from an intumescent cataractous lens. The eye is red with a hazy view of the anterior segment from cornealedema, with a fixed, irregular, semidilated pupil from iris infarction. The slit image shows the corneal edema and a very shallow anterior chamber.Some uveitis may be present because of ischemia, and this must be differentiated from the larger accumulations of lens material and macrophagesseen with phacolytic glaucoma. (From Spalton DJ, Hitchings RA, Hunter P: Atlas of clinical ophthalmology, 3rd ed, London, 2005, Mosby, p 225.)

Supraorbital n.

Supraorbital notch

Figure 2-5 Injection technique for relieving the pain of supraorbitalneuralgia. (From Waldman SD: Atlas of pain management injection tech-niques, 2nd ed, Philadelphia, 2007, Saunders.)

Clinical Pearls

Supraorbital nerve block is especially useful in the diagnosis

and palliation of pain secondary to supraorbital neuralgia.The first step in the management of this unusual cause ofheadache is the correct fitting of swimming goggles thatdo not compress the supraorbital nerves. Coexistent fron-tal sinusitis should be ruled out in patients who do notrespond rapidly to a change in swim goggles and a series ofthe previously mentioned nerve blocks. Any patient withheadaches severe enough to require neural blockade as partof the treatment plan should undergo MRI of the head torule out unsuspected intracranial pathological conditions.


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Chronic paroxysmal hemicrania shares many characteristics of its

more common analogue, cluster headache, but has several impor-tant differences (Table 3-1). Similar to cluster headache, chronicparoxysmal hemicrania is a severe, episodic, unilateral headachethat affects the periorbital and retroorbital regions. In contrastto cluster headache, which occurs 10 times more commonly inmen, chronic paroxysmal hemicrania occurs primarily in women(Figure 3-1). The duration of pain associated with chronic parox-ysmal hemicrania is shorter than that of cluster headache, lasting 5to 45 minutes. This pain does not follow the chronobiological pat-tern seen in patients with cluster headache. Patients with chronicparoxysmal hemicrania usually experience more than five attacksper day. Chronic paroxysmal hemicrania uniformly responds toindomethacin, whereas cluster headache does not.

SIGNS AND S YMPTOMS

During attacks of chronic paroxysmal hemicrania, patients exhibitthe following physical findings suggestive of Horner’s syndromeon the ipsilateral side of the pain: • Conjunctival and scleral injection • Lacrimation • Nasal congestion • Rhinorrhea • Ptosis of the eyelid

As in cluster headache, the patient may become agitated duriattacks, rather than seeking dark and quiet as does the patient wimigraine. In contrast to cluster headache, alcohol consumptiodoes not seem to trigger attacks of chronic paroxysmal hemicrnia. Between attacks, the neurological examination of a patie

with chronic paroxysmal hemicrania should be normal.

TESTING

Magnetic resonance imaging (MRI) of the brain provides the beinformation regarding the cranial vault and its contents. MRIhighly accurate and helps identify abnormalities that may put tpatient at risk for neurological disasters secondary to intracranand brainstem pathological conditions, including tumors andemyelinating disease (Figure 3-2). Magnetic resonance angioraphy (MRA) also may be useful in identifying aneurysms, whimay be responsible for the patient’s neurological findings. patients who cannot undergo MRI, such as a patient with a pacmaker, computed tomography (CT) is a reasonable second choicRadionuclide bone scanning and plain radiography are indicatif fracture or bony abnormality such as metastatic disease is cosidered in the differential diagnosis.

Screening laboratory tests consisting of complete blood c

count, erythrocyte sedimentation rate, and automated bloochemistry testing should be performed if the diagnosis of chron

Chapter 3

CHRONIC PAROXYSMAL HEMICRANIA

TABLE 3-1

Comparison of Cluster Headache and Chronic

Paroxysmal Hemicrania

Comparison FactorsClusterHeadache

ChronicParoxysmalHemicrania

Gender predominance Male Female

Response to indomethacin Negative Positive

Chronobiological pattern Positive Negative

Alcohol trigger Positive Negative

Length of attacks Longer Shorter

Horner’s syndrome Present Present

ICD-9 CODE 784.0

ICD-10 CODE R51

Figure 3-1 In contrast to cluster headache, which occurs primarily men, chronic paroxysmal hemicrania occurs primarily in women.

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paroxysmal hemicrania is in question. Intraocular pressure shouldbe measured if glaucoma is suspected.


Chronic paroxysmal hemicrania is a clinical diagnosis supported bya combination of clinical history, abnormal physical examinationduring attacks, radiography, and MRI. Pain syndromes that maymimic chronic paroxysmal hemicrania include cluster headache,trigeminal neuralgia involving the first division of the trigeminalnerve, demyelinating disease, and ice pick headache. Trigeminalneuralgia involving the first division of the trigeminal nerve is

uncommon and is characterized by trigger areas and tic-like move-ments. Demyelinating disease is generally associated with otherneurological findings, including optic neuritis and other motorand sensory abnormalities. The pain of cluster headache lasts muchlonger than the pain of chronic paroxysmal hemicrania, and clusterheadache has a male predominance, a chronobiological pattern ofattacks, and a lack of response to treatment with indomethacin.

TREATMENTChronic paroxysmal hemicrania uniformly responds to treatment

with indomethacin. Failure to respond to indomethacin puts thediagnosis of chronic paroxysmal hemicrania in question. A start-ing dose of 25 mg daily for 2 days and titrating to 25 mg threetimes per day is a reasonable treatment approach. This dose maybe carefully increased up to 150 mg per day. Indomethacin mustbe used carefully, if at all, in patients with peptic ulcer disease orimpaired renal function. Anecdotal reports of a positive responseto cyclooxygenase-2 (COX-2) inhibitors in the treatment ofchronic paroxysmal hemicrania have been noted in the headacheliterature. Underlying sleep disturbance and depression are besttreated with a tricyclic antidepressant compound, such as nortrip-

tyline, which can be started at a single bedtime dose of 25 mg.


Failure to diagnose chronic paroxysmal hemicrania correctly mayput the patient at risk if intracranial pathological conditions ordemyelinating disease that may mimic the clinical presentation ofchronic paroxysmal hemicrania is overlooked. MRI is indicated inall patients thought to have chronic paroxysmal hemicrania. Fail-ure to diagnose glaucoma, which may cause intermittent ocularpain, may result in permanent loss of sight.

SUGGESTED READINGS

Benitez-Rosario MA, McDarby G, Doyle R, Fabby C: Chronic cluster-like head-ache secondary to prolactinoma: uncommon cephalalgia in association withbrain tumors, J Pain Symptom Manage 37:271–276, 2009.

Benoliel R, Sharav Y: Paroxysmal hemicrania: case studies and review of the lit-erature, Oral Surg Oral Med Oral Pathol Oral Radiol Endodontol 85:285–292,1998.

Camarda C, Camarda R, Monastero R: Chronic paroxysmal hemicrania andhemicrania continua responding to topiramate: two case reports, Clin NeurolNeurosurg 110:88–91, 2008.

Klasser GD, Balasubramaniam R: Trigeminal autonomic cephalalgias. II. Par-oxysmal hemicrania, Oral Surg Oral Med Oral Pathol Oral Radiol Endodontol 104:640–646, 2007.

Sjaastad O: Chronic paroxysmal hemicrania: clinical aspects and controversies. InBlau JN, editor: Migraine: clinical, therapeutic, conceptual and research aspects ,London, 1987, Chapman & Hall, pp 135–152.

Talvik I, Koch K, Kolk A, Talvik T: Chronic paroxysmal hemicrania in a 3-year,10-month-old female, Pediatr Neurol 34:225–227, 2006.

A

B

Figure 3-2 Sagittal (A) and semiaxial (B) T2-weighted images of a mas-sive prolactinoma in a 41-year-old man with chronic daily headache.(From Benitez-Rosario MA, McDarby G, Doyle R, Fabby G. Chronic cluster-like headache secondary to prolactinoma: uncommon cephalalgia in asso-ciation with brain tumors, J Pain Symptom Manage 37:271–276, 2009.

Clinical Pearls

The diagnosis of chronic paroxysmal hemicrania is madeby obtaining a thorough, targeted headache history.Between attacks, patients with chronic paroxysmal hemi-crania should have a normal neurological examination. Ifthe neurological examination is abnormal between attacks,the diagnosis of chronic paroxysmal hemicrania should bediscarded and a careful search for the cause of the patient’sneurological findings should be undertaken.


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Charlin’s syndrome, also known as nasociliary neuralgia, is anuncommon cause of head and face pain. As with most headachesyndromes, the exact cause of the pain of Charlin’s syndrome isunknown. However, the pathogenesis of this uncommon causeof head and face pain is thought to be dysfunction of the naso-ciliary ganglion in a manner analogous to the dysfunction of thesphenopalatine ganglion thought to be the source of cluster head-ache. The pain of Charlin’s syndrome has a rapid onset to peak, with attacks lasting 45 to 60 minutes. In some patients, theseattacks can be triggered by sensory stimulation of the affectedareas. Although in many ways similar to cluster headache (e.g.,retroorbital location of pain, profuse unilateral rhinorrhea, rapidonset to peak, and short duration of attacks), many dissimilarities

also exist. In contrast to cluster headache, alcohol consumptiondoes not appear to trigger attacks of Charlin’s syndrome and theseasonal and chronobiological patterns so characteristic of clusterheadache do not seem to be a factor (Table 4-1). Blockade of thesphenopalatine ganglion, which is so effective in the treatment ofcluster headache, is of little value in the treatment of Charlin’ssyndrome. Patients suffering from Charlin’s syndrome uniformlyrespond to daily nasociliary nerve blocks with local anesthetic, asdescribed subsequently.

SIGNS AND S YMPTOMS

Patients suffering from Charlin’s syndrome present with the com-plaint of severe paroxysms of ocular or retroorbital pain that radi-

ates into the ipsilateral forehead, nose, and maxillary region. Thispain is associated with voluminous ipsilateral rhinorrhea and con-gestion of the nasal mucosa and significant inflammation of theaffected eye (Figure 4-1).

TESTING

Magnetic resonance imaging (MRI) of the brain provides the bestinformation regarding the cranial vault and its contents. MRI ishighly accurate and helps identify abnormalities that may put thepatient at risk for neurological disasters secondary to intracranialand brainstem pathological conditions, including tumors and

demyelinating disease (Figure 4-2). Magnetic resonance angioraphy (MRA) also may be useful in helping identify aneurysm which may be responsible for the patient’s neurological findingIn patients who cannot undergo MRI, such as a patient withpacemaker, computed tomography (CT) is a reasonable seconchoice. Radionuclide bone scanning and plain radiography aindicated if fracture or bony abnormality such as metastatic dease is considered in the differential diagnosis.

Screening laboratory tests consisting of complete blood ccount, erythrocyte sedimentation rate, and automated bloochemistry testing should be performed if the diagnosis of Chalin’s syndrome is in question. Intraocular pressure should be mesured if glaucoma is suspected.


Charlin’s syndrome is a clinical diagnosis supported by a combination of clinical history, normal physical examination, radography, and MRI. Pain syndromes that may mimic Charlinsyndrome include cluster headache, temporal arteritis, trigemnal neuralgia involving the first division of the trigeminal nervdemyelinating disease, and chronic paroxysmal hemicrania (s

Table 4-1). Trigeminal neuralgia involving the first division the trigeminal nerve is uncommon and is characterized by triger areas and tic-like movements. Demyelinating disease is geneally associated with other neurological findings, including optneuritis and other motor and sensory abnormalities. The pain chronic paroxysmal hemicrania lasts much longer than the pain Charlin’s syndrome.

TREATMENT

The treatment of Charlin’s syndrome is analogous to the treament of trigeminal neuralgia. The use of anticonvulsants such carbamazepine and gabapentin represents a reasonable startipoint. High-dose steroids tapered over 10 days also have be

anecdotally reported to provide relief. For patients who do nrespond to the previously mentioned treatments, daily nasociliaganglion block with local anesthetic and steroid is a reasonabnext step. Underlying sleep disturbance and depression associat with the pain of supraorbital neuralgia are best treated with a tcyclic antidepressant compound, such as nortriptyline, which cbe started at a single bedtime dose of 25 mg.


Failure to diagnose Charlin’s syndrome correctly may pthe patient at risk if an intracranial pathological condition

Chapter 4

CHARLIN’S SYNDROME

ICD-9 CODE 350.1

ICD-10 CODE G50.0

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demyelinating disease, which may mimic the clinical presenta-tion of Charlin’s syndrome, is overlooked. MRI is indicated in allpatients thought to have Charlin’s syndrome. Failure to diagnoseglaucoma or temporal arteritis, which also may cause intermittentocular pain, may result in permanent loss of sight.

SUGGESTED READINGSBecker M, Kohler R, Vargas MI, Viallon M, Delavelle J: Pathology of the trigeminal

nerve, Neuroimaging Clin N Am 18:283–307, 2008.Craven J: Anatomy of the cranial nerves, Anaesth Intensive Care Med 11:529–534,

2010.Lewis DW, Gozzo YF, Avner MT: The “other” primary headaches in children and

adolescents [review], Pediatr Neurol 33:303–313, 2005. Waldman SD: The trigeminal nerve. In Waldman SD, editor: Pain review , Phila-

delphia, 2009, Saunders, pp 15–17. Waldman SD: Charlin’s syndrome. In Waldman SD, editor: Atlas of pain manage-

ment injection techniques , Philadelphia, 2007, Saunders, pp 20–24.Figure 4-1 Patients suffering from Charlin’s syndrome present with thecomplaint of severe paroxysms of ocular or retroorbital pain that radi-ates into the ipsilateral forehead, nose, and maxillary region. The pain isassociated with voluminous ipsilateral rhinorrhea and congestion of thenasal mucosa and significant inflammation of the affected eye.

Figure 4-2 Multiple sclerosis. Fluid-attenuated inversion recovery (FLAIR)parasagittal MR image depicts the extensive demyelinated plaques ofprogressive multiple sclerosis. (From Haaga JR, Lanzieri CF, Gilkeson RC,editors: CT and MR imaging of the whole body, 4th ed, Philadelphia,2003, Mosby, p 466.)

TABLE 4-1

Comparison of Cluster Headacheand Charlin’s Syndrome

Comparison FactorsCluster

HeadacheCharlin’s

Syndrome

Ocular and retroorbital location Yes Yes

Unilateral Yes Yes

Rapid onset to peak Yes YesSevere intensity Yes Yes

Attacks occur in paroxysms Yes Yes

Duration of attacks short Yes Yes

Pain free between attacks Yes Yes

Significant rhinorrhea during attacks Yes Yes

Alcohol triggers attacks Yes No

Tactile trigger areas No Yes

Seasonal pattern of attacks Yes No

Chronobiological pattern of attacks Yes No

Significant eye inflammation No Yes

Responds to sphenopalatine

ganglion block

Yes No

Responds to nasociliary block No Yes

Clinical Pearls

Nasociliary nerve block via the medial orbital approachis especially useful in the diagnosis and palliation of pain

secondary to Charlin’s syndrome. Given the uncommonnature of this headache syndrome and its overlap with thesymptoms of cluster headache and other neurological prob-lems, including cavernous sinus thrombosis and intracranialand retroorbital tumors, Charlin’s syndrome must remain adiagnosis of exclusion. All patients suspected to have Char-lin’s syndrome require MRI of the brain with and withoutgadolinium contrast material and thorough ophthalmologi-cal and neurological evaluation. Nasociliary nerve block viathe medial orbital approach should be performed only byclinicians familiar with the regional anatomy.


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TESTING

Magnetic resonance imaging (MRI) of the brain provides the bestinformation regarding the cranial vault and its contents. MRI ishighly accurate and helps identify abnormalities that may put thepatient at risk for neurological disasters secondary to intracranialand brainstem pathological conditions, including tumors, demy-elinating disease, and hemorrhage. More important, MRI helpsidentify bleeding associated with leaking intracranial aneurysms.Magnetic resonance angiography (MRA) may be useful in help-ing identify aneurysms responsible for the patient’s neurologicalsymptoms. In patients who cannot undergo MRI, such as patients

with pacemakers, computed tomography (CT) is a reasonablesecond choice. Even if blood is not present on MRI or CT, ifintracranial hemorrhage is suspected, lumbar puncture should beperformed.

Screening laboratory tests consisting of complete blood cellcount, erythrocyte sedimentation rate, and automated bloodchemistry testing should be performed if the diagnosis of sexualheadache is in question. Intraocular pressure should be measuredif glaucoma is suspected.

DIFFERENTIAL DIAGNOSISSexual headache is a clinical diagnosis supported by a combina-tion of clinical history, normal physical examination, radiogra-phy, MRI, and MRA. Pain syndromes that may mimic sexualheadache include trigeminal neuralgia involving the first divi-sion of the trigeminal nerve, demyelinating disease, cluster head-ache, migraine, and chronic paroxysmal hemicrania. Trigeminalneuralgia involving the first division of the trigeminal nerve isuncommon and is characterized by trigger areas and tic-likemovements. Demyelinating disease is generally associated withother neurological findings, including optic neuritis and othermotor and sensory abnormalities. The pain of chronic paroxys-mal hemicrania and cluster headache is associated with redness

and watering of the ipsilateral eye, nasal congestion, and rhinor-rhea during the headache. These findings are absent in all typesof sexual headache. Migraine headache may or may not be associ-ated with nonpainful neurological findings known as aura, butthe patient almost always reports some systemic symptoms, suchas nausea or photophobia, not typically associated with sexualheadache.

TREATMENT

It is generally thought that avoiding the inciting activity for a few weeks decreases the propensity to trigger sexual headaches. If thisavoidance technique fails or is impractical because of patient pref-erence, a trial of propranolol is a reasonable next step. A low dose

of 20 to 40 mg as a daily dose and titrating in 20-mg incrementsto 200 mg as a divided daily dose until prophylaxis occurs treats

most patients suffering from sexual headache. Propranolol shouldbe used with caution in patients with asthma or cardiac failure andpatients with brittle diabetes.

If propranolol is ineffective, indomethacin may be tried. Astarting dose of 25 mg daily for 2 days and titrating to 25 mgthree times per day is a reasonable treatment approach. This dosemay be carefully increased to 150 mg per day. Indomethacin mustbe used carefully, if at all, in patients with peptic ulcer disease orimpaired renal function. Anecdotal reports of a positive responseto cyclooxygenase-2 (COX-2) inhibitors in the treatment of sexualheadache have been noted in the headache literature. Underlyingsleep disturbance and depression are best treated with a tricyclicantidepressant compound, such as nortriptyline, which can bestarted at a single bedtime dose of 25 mg.


Failure to diagnose sexual headache correctly may put the patientat risk if intracranial pathology or demyelinating disease (whichmay mimic the clinical presentation of sexual headache) is over-looked. MRI and MRA are indicated in all patients thought tohave sexual headache. Failure to diagnose glaucoma, which also

may cause intermittent ocular pain, may result in permanent lossof sight.

SUGGESTED READINGS

Evans RW: Diagnostic testing for migraine and other primary headaches, NeurolClin 27:393–415, 2009.

Hu CM, Lin YJ, Fan YK, et al: Isolated thunderclap headache during sex: orgas-mic headache or reversible cerebral vasoconstriction syndrome? J Clin Neurosci 17:1349–1351, 2010.

Jolobe OMP: The differential diagnosis includes reversible cerebral vasoconstrictorsyndrome, Am J Emerg Med 28:637, 2010.

Kim HJ, Seo SY: Recurrent emotion-triggered headache following primary head-ache associated with sexual activity, J Neurol Sci 273:142–143, 2008.

Tuğba T, Serap Ü, Esra O, et al: Features of stabbing, cough, exertional and sex-ual headaches in a Turkish population of headache patients, J Clin Neurosci 15:774–777, 2008.

Clinical Pearls

The diagnosis of sexual headache is made by obtaining athorough, targeted headache history. As mentioned earlier,patients may not be forthcoming about the events surround-ing the onset of their headache, and the clinician should besensitive to this fact. Patients suffering from sexual head-ache should have a normal neurological examination. Ifthe neurological examination is abnormal, the diagnosis ofsexual headache should be discarded and a careful search

for the cause of the patient’s neurological findings shouldbe undertaken.


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Cough headache is a term used to describe headaches triggered bycoughing and other activities associated with a Valsalva maneuver,such as laughing, straining at stool, lifting, and bending the headtoward the ground (Figure 6-1). Clinicians have identified the fol-lowing two types of cough headache: • Benign • Symptomatic

Initially, both types of cough headache were thought to berelated to sexual and exertional headaches, but they are now con-sidered distinct clinical entities. A strong male predilection is seenfor benign cough headache and no gender predilection for symp-tomatic cough headache.

SIGNS AND S YMPTOMS

Patients suffering from cough headache present differentlydepending on the type of cough headache experienced. Each clini-cal presentation is discussed.

Benign Cough Headache

Benign cough headache is not associated with obvious neurologi-cal or musculoskeletal disease. More than 80% of patients withbenign cough headache are males, in contradistinction to symp-tomatic cough headache, in which no gender predilection is seen.The onset of benign cough headache is abrupt, occurring immedi-ately after coughing or other activities that cause a Valsalva maneuver. Although the intensity of pain is severe and peaks rapidly, it

lasts only seconds to minutes. The character of the pain associated with benign cough headache is splitting or sharp, and the pain islocated in the occipital region bilaterally and occasionally the ver-tex of the skull. No accompanying neurological or systemic symp-toms are seen, as with cluster and migraine headaches. The age ofonset of benign cough headache is generally in the late fifth or sixthdecade of life. If such headaches occur before age 50, there shouldbe strong clinical suspicion that the patient either has symptom-atic cough headache or a pathological condition in the posteriorfossa, such as Arnold-Chiari malformation or tumor. Tumors ofthe foramen magnum also may mimic the presentation of benigncough headache even if no neurological symptoms are present.

Symptomatic Cough Headache

Symptomatic cough headache is almost always associated wistructural abnormalities of the cranium, such as Arnold-Chiamalformation I and II or intracranial tumors (Figure 6-2). Tsymptoms associated with symptomatic cough headache athought to be due to herniation of the cerebellar tonsil througthe foramen magnum into the space normally occupied by tupper portion of the cervical spinal cord. Similar to benign cou

headache, the onset of pain associated with symptomatic cougheadache is abrupt, occurring immediately after coughing or othactivities that cause a Valsalva maneuver. Although the intensiof pain is severe and peaks rapidly, it lasts only seconds to miutes. In contrast to benign cough headache, associated neurologcal symptoms may be present, including difficulty swallowinfaintness, and numbness in the face and upper extremities. Theassociated symptoms should be taken very seriously because thare indicative of increased intracranial pressure and herniation the intracranial contents.

The character of the pain associated with symptomatic couheadache is splitting or sharp, and pain is located in the occipitregion bilaterally and occasionally the vertex of the skull. Tage of onset of symptomatic cough headache is generally in th

third decade of life, although, depending on the amount of nerological compromise, it may occur at any age. In contrast benign cough headache, which occurs predominantly in mesymptomatic cough headache occurs with equal prevalence both genders.

TESTING

Magnetic resonance imaging (MRI) of the brain provides the beinformation regarding the cranial vault and its contents. MRIhighly accurate and helps identify abnormalities that may pthe patient at risk for neurological disasters secondary to intrcranial and brainstem pathological conditions, including tumoand demyelinating disease. Special attention to the foramen ma

num may help identify more subtle abnormalities responsibfor posterior fossa neurological signs and symptoms. MRI helidentify bleeding associated with leaking intracranial aneurysm which may mimic the symptoms of both types of cough heaache. Magnetic resonance angiography (MRA) may be useful helping identify aneurysms responsible for the patient’s neurlogical symptoms. In patients who cannot undergo MRI, such patients with pacemakers, computed tomography (CT) is a resonable second choice. Lumbar puncture should be performedintracranial hemorrhage is suspected, even if blood is not preseon MRI or CT. Plain radiographs of the cervical spine also mbe useful in the evaluation of Arnold-Chiari malformations an

Chapter 6

COUGH HEADACHE

ICD-9 CODE 784.0

ICD-10 CODE R51



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should be included in the evaluation of all patients with coughheadache.

Screening laboratory tests consisting of complete blood cellcount, erythrocyte sedimentation rate, and automated bloodchemistry testing should be performed if the diagnosis of cough

headache is in question. Intraocular pressure should be measuredif glaucoma is suspected.


Cough headache is a clinical diagnosis supported by a combinationof clinical history, physical examination, radiography, MRI, andMRA. Pain syndromes that may mimic cough headache includebenign exertional headache, ice pick headache, sexual headache,trigeminal neuralgia involving the first division of the trigeminalnerve, demyelinating disease, cluster headache, and chronic par-oxysmal hemicrania. Trigeminal neuralgia involving the first divi-sion of the trigeminal nerve is uncommon and is characterizedby trigger areas and tic-like movements. Demyelinating disease

is generally associated with other neurological findings, includingoptic neuritis and other motor and sensory abnormalities. Thepain of chronic paroxysmal hemicrania and cluster headache isassociated with redness and watering of the ipsilateral eye, nasalcongestion, and rhinorrhea during the headache. These findingsare absent in all types of cough headache. Migraine headachemay or may not be associated with painless neurological find-ings known as aura, but the patient almost always reports somesystemic symptoms, such as nausea or photophobia, not typicallyassociated with cough headache.

TREATMENT

Indomethacin is the treatment of choice for benign cough head-

ache. A starting dose of 25 mg daily for 2 days and titrating to25 mg three times per day is a reasonable treatment approach.This dose may be carefully increased up to 150 mg per day. Indo-methacin must be used carefully, if at all, in patients with pepticulcer disease or impaired renal function. Headache specialists havenoted anecdotal reports of a positive response to cyclooxygenase-2(COX-2) inhibitors in the treatment of benign cough headache.Underlying sleep disturbance and depression are best treated witha tricyclic antidepressant compound, such as nortriptyline, whichcan be started at a single bedtime dose of 25 mg.

The only uniformly effective treatment for symptomatic coughheadache is surgical decompression of the foramen magnum.

Figure 6-2 Low-lying cerebellar tonsils (straight arrows) of a Chiari malformation are shown deforming the medulla (curved arrow) in a sagittalT1-weighted spin echo image. 4, Fourth ventricle. (From Stark DD, Brad-ley WG Jr, editors: Magnetic resonance imaging, 3rd ed, St Louis, 1999,Mosby, p 1841.)

Herniation ofcerebellar tonsil

Spinal cordFigure 6-1 Symptomatic cough headache is oftenassociated with structural abnormalities, such as Arnold-Chiari malformation, and usually occurs in thethird decade of life.


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6 • Cough Headache

This surgery is usually done via suboccipital craniectomy. Surgi-cal decompression prevents the low-lying cerebellar tonsils fromobstructing the flow of spinal fluid from the cranium to the spinalsubarachnoid space during a Valsalva maneuver.


Failure to diagnose cough headache correctly may put the patientat risk if intracranial pathology or demyelinating disease (whichmay mimic the clinical presentation of cough headache) is over-looked. MRI and MRA are indicated in all patients thought tohave cough headache. Failure to diagnose glaucoma, which alsomay cause intermittent ocular pain, may result in permanent lossof sight.

SUGGESTED READINGS

Berciano J, Poca M-A, García A, Sahuquillo J: Paroxysmal cervicobrachial couginduced pain in a patient with syringomyelia extending into spinal cord posrior gray horns, J Neurol 54:678–681, 2007.

Chen YY, Lirng JF, Fuh JL, et al: Primary cough headache is associated with pterior fossa crowdedness: a morphometric MRI study, Cephalalgia 24:694–692004.

Pascual J: Primary cough headache, Curr Pain Headache Rep 9:272–276, 2005Pascual J, Rubén Martín A, Oterino A: Headaches precipitated by cough, p

longed exercise or sexual activity: a prospective etiological and clinical stud

J Headache Pain 9:259–266, 2008. Waldman SD: Arnold Chiari malformation type I. In Waldman SD, Campb

RS, editors: Imaging of pain, Philadelphia, 2011, Saunders, pp 27–28. Waldman SD: Arnold Chiari malformation type II. In Waldman SD, Campb

RS, editors: Imaging of pain, Philadelphia, 2011, Saunders, pp 29–30.

Clinical Pearls

Any patient presenting with headaches associated withexertion or Valsalva maneuver should be taken very seri-ously. Although statistically most of these headaches ulti-mately are proved to be of benign cause, a few patients havepotentially life-threatening disease. The diagnosis of cough

headache is made by obtaining a thorough, targeted head-ache history and performing a careful physical examination.The clinician must separate patients suffering from benigncough headache from patients suffering from symptom-atic cough headache. Patients with benign cough headacheshould have a normal neurological examination. If the neu-rological examination is abnormal, the diagnosis of benigncough headache should be discarded and a careful searchfor the cause of the patient’s neurological findings shouldbe undertaken.


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16


Sudden unilateral neuralgiform conjunctival injection tearing(SUNCT) headache is an uncommon primary headache disorderthat is one of a group of three headache syndromes known as thetrigeminal autonomic cephalgias (Table 7-1). Whether SUNCTheadache is in fact a distinct headache entity or simply a con-stellation of symptoms that occurs on a continuum along withthe other trigeminal autonomic cephalgias is a point of ongoingdebate among headache and pain management specialists (Figure 7-1). As with most headache syndromes, the exact cause of thepain of SUNCT headache is unknown; however, the pathogenesisof this uncommon cause of head and face pain is thought to bedysfunction of the trigeminal autonomic reflex.

The pain of SUNCT headache has a rapid onset to peak, with

attacks lasting 5 seconds to 4 minutes and the frequency of attacksranging from 20 to 200 attacks per day. In some patients, theseattacks can be triggered by sensory stimulation of the affectedareas, such as when washing the face, brushing the teeth, and soforth. Although in many ways similar to cluster headache (e.g.,unilateral, periorbital and frontal location of pain, sclera injec-tion, rapid onset to peak, short duration of attacks, and pain-freeperiods between attacks), SUNCT exhibits many dissimilarities as well. In contrast to cluster headache, alcohol consumption doesnot seem to trigger attacks of SUNCT headache, and there do notseem to be the seasonal and chronobiological patterns so charac-teristic of cluster headache, although SUNCT headache occursmost frequently in the morning and afternoon (Table 7-2).

Blockade of the sphenopalatine ganglion, which is so effec-

tive in the treatment of cluster headache, is of little value in thetreatment of SUNCT headache. Patients suffering from SUNCTheadache may respond to daily trigeminal nerve blocks with localanesthetic, as described subsequently.

SIGNS AND S YMPTOMS

Patients with SUNCT headache present with the complaint ofsevere paroxysms of ocular or periorbital pain that radiate intothe ipsilateral temple, forehead, nose, cheek, throat, and maxillaryregion. This pain is associated with significant inflammation of theaffected eye (Figure 7-2). The pain is neuralgiform and severe to

excruciating in intensity (Table 7-3). SUNCT occurs on the rightside 70% of the time in a manner analogous to trigeminal neu-ralgia. Like trigeminal neuralgia, rare cases of bilateral SUNCTheadache have been reported. Also like trigeminal neuralgia, thepain of SUNCT headache rarely switches sides. SUNCT head-ache occurs slightly more frequently in males. It can occur at anyage, with a peak incidence in the fifth decade.

TESTINGMagnetic resonance imaging (MRI) of the brain provides the cli-nician with the best information regarding the cranial vault andits contents. MRI is highly accurate and helps identify abnor-malities that may put the patient at risk for neurological disasterssecondary to intracranial and brainstem pathological conditions,including tumors and demyelinating disease. Magnetic resonanceangiography (MRA) also may be useful in helping identify aneu-rysms, which may be responsible for the patient’s neurologicalfindings. In patients who cannot undergo MRI, such as patients with pacemakers, computed tomography (CT) is a reasonable sec-ond choice. Radionuclide bone scanning and plain radiographyare indicated if fracture or bony abnormality such as metastatic

disease is considered in the differential diagnosis.Screening laboratory tests consisting of complete blood cellcount, erythrocyte sedimentation rate, and automated bloodchemistry testing should be performed if the diagnosis of SUNCT

Chapter 7

SUDDEN UNILATERAL

NEURALGIFORM CONJUNCTIVALINJECTION TEARING HEADACHE

TABLE 7-1

Trigeminal Autonomic Cephalgias

Cluster headache

Chronic paroxysm hemicrania

SUNCT headache

SUNCT, Sudden unilateral neuralgiform conjunctival injection tearing.

SUNCTParoxysmalhemicrania

Clusterheadache

Time15–180 min2–30 min5 s–4 min

Overlapbetween duration

Overlapbetween duration

Figure 7-1 Overlap between attack duration in trigeminal autonomiccephalalgias. SUNCT, Sudden unilateral neuralgiform conjunctivalinjection tearing. (From Leone M, Bussone G: Pathophysiology of trigemi-nal autonomic cephalalgias, Lancet Neurol 8:755–774, 2009.)

ICD-9 CODE 350.1

ICD-10 CODE G50.0

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7 • Sudden Unilateral Neuralgiform Conjunctival Injection Tearing Headache

headache is in question. Intraocular pressure should be measuredif glaucoma is suspected.


SUNCT headache is a clinical diagnosis supported by a combina-tion of clinical history, normal physical examination, radiography,and MRI. Pain syndromes that may mimic SUNCT headacheinclude cluster headache, temporal arteritis, trigeminal neuralgia

involving the first division of the trigeminal nerve, demyelinatindisease, primary stabbing headache, hypnic headache, and chronparoxysmal hemicranias, although because of the overlapping fetures of all headache and facial pain syndromes, SUNCT heaache can be easily mistaken for another type of headache or facpain (Figure 7-3; Table 7-4). Trigeminal neuralgia involving tfirst division of the trigeminal nerve is uncommon and is charaterized by trigger areas and tic-like movements. Demyelinatindisease is generally associated with other neurological findingincluding optic neuritis and other motor and sensory abnormaties. The pain of chronic paroxysmal hemicrania lasts much longthan the pain of SUNCT headache.

TREATMENT

The treatment of SUNCT headache is analogous to the treatmeof trigeminal neuralgia, although the pharmacological managment of this uncommon headache disorder is disappointing. Tuse of anticonvulsants such as lamotrigine and gabapentin reprsents a reasonable starting point. High-dose steroids tapered ov10 days also have been anecdotally reported to provide relief. Fpatients who do not respond to the previously mentioned trea

ments, daily trigeminal nerve block with a local anesthetic ansteroid is a reasonable next step.Occasionally, retrogasserian injection of glycerol, balloon com

pression of the Gasserian ganglion, and microvascular decompresion of the trigeminal nerve root are required to provide palliatioof pain. Underlying sleep disturbance and depression associat with the pain of SUNCT headache are best treated with a tricclic antidepressant compound, such as nortriptyline, which can started at a single bedtime dose of 25 mg.


Failure to diagnose SUNCT headache correctly may put tpatient at risk if an intracranial pathological condition or dem

elinating disease, which may mimic the clinical presentation SUNCT headache, is overlooked. MRI is indicated in all patienthought to have SUNCT headache. Failure to diagnose glaucomor temporal arteritis, which also may cause intermittent oculpain, may result in permanent loss of sight.

TABLE 7-2

Comparison of Cluster Headache and Sudden UnilateralNeuralgiform Conjunctival Injection Tearing Headache

Comparison Factors ClusterHeadache

SUNCTHeadache

Ocular and retroorbital location Yes Yes

Unilateral Yes Yes

Rapid onset to peak Yes Yes

Severe intensity Yes Yes

Attacks occur in paroxysms Yes Yes

Duration of attacks short Yes YesPain free between attacks Yes Yes

Significant rhinorrhea during attacks Yes No

Alcohol triggers attacks Yes No

Tactile trigger areas No Yes

Seasonal pattern of attacks Yes No

Chronobiological pattern of attacks Yes No

Significant eye inflammation No Yes

Responds to sphenopalatine ganglionblock

Yes No

Responds to trigeminal nerve block No Yes

SUNCT, Sudden unilateral neuralgiform conjunctival injection tearing.

Figure 7-2 Patients with SUNCT headache present with severe paroxysms of ocular or periorbital pain that radiates into the ipsilateral temple, forehead, nose, cheek, throat, and maxillary region that is associatedwith significant inflammation of the affected eye.

TABLE 7-3

Descriptors of Pain Associated with Sudden UnilateralNeuralgiform Conjunctival Injection Tearing Headache

Stabbing

Shooting

Lancinating

Shocklike

Sharp

Piercing

Pricking

Staccato-like



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Cluster headache

Neck

Shoulder,neck, arm

Paroxysmal hemicrania

SUNCT

Hemicrania continua

Migraine

NVOP

TABLE 7-4

Differential Diagnosis: Sudden Unilateral NeuralgiformConjunctival Injection Tearing Headache

Cluster headache

Temporal arteritis

Trigeminal neuralgia

Demyelinating disease

Primary stabbing headache

Hypnic headache

Chronic paroxysmal hemicrania

Clinical Pearls

Trigeminal nerve block with local anesthetic is especiallyuseful in the diagnosis and palliation of pain secondary toSUNCT headache. Given the uncommon nature of thisheadache syndrome and its overlap with the symptoms ofcluster headache and other neurological problems, includ-ing cavernous sinus thrombosis and intracranial and retroor-bital tumors, SUNCT headache must remain a diagnosis ofexclusion. All patients suspected to have SUNCT headacherequire MRI of the brain with and without gadolinium con-trast material and thorough ophthalmological and neurolog-ical evaluation. Trigeminal nerve block should be performedonly by clinicians familiar with the regional anatomy.

Figure 7-3 Pain location in the trigeminal autonomic cephalgias (TACs)and neurovascular orofacial pain. The TACs are characterized by orbitaland periorbital pain. In paroxysmal hemicrania and hemicrania conti-nua, large adjacent areas are affected. Migraine is largely unilateral butmay be bilateral in up to 30% of cases (this has been marked by a lightershaded area contralaterally). Neurovascular orofacial pain is character-ized by its location in the lower two thirds of the face with intraoral andperioral areas frequently involved as primary sites. Two-headed arrow above diagram indicates the side shift that occurs in specific headache.NVOP, Neurovascular orofacial pain. (Modified from Benoliel R, Sharav Y:The trigeminal autonomic cephalgias (TACs). In: Sharav Y, editor: Orofacialpain and headache, Edinburgh, 2008, Elsevier, pp 225–254.)

SUGGESTED READINGS

Leone M, Bussone G: Pathophysiology of trigeminal autonomic cephalalgias, Lan-cet Neurol 8:755–774, 2009.

Levin M: Nerve blocks and nerve stimulation in headache disorders, Tech Reg Anesth Pain Manage 13:42–49, 2009.

Levin M: Nerve blocks in the treatment of headache, Neurotherapeutics 7:197–203, 2010.

Klasser GD, Balasubramaniam R: Trigeminal autonomic cephalalgias. III. Short-lasting unilateral neuralgiform headache attacks with conjunctival injection and

tearing, Oral Surg Oral Med Oral Pathol Oral Radiol Endodontol 104: 773–771.2007.

Rozen TD: Trigeminal autonomic cephalalgias, Neurol Clin 27:537–557, 2009. Waldman SD: The trigeminal nerve. In Waldman SD, editor: Pain Review , Phila-

delphia, 2009, Saunders, pp 15–17. Waldman SD: Gasserian ganglion block. In Waldman SD, editor: Atlas of inter-

ventional pain management , ed 3, Philadelphia, 2009, Saunders, pp 32–38. Waldman SD: Gasserian ganglion block: balloon compression technique. In

Waldman SD, editor: Atlas of interventional pain management , ed 3, Philadel-phia, 2009, Saunders, pp 43–47.

Waldman SD: Trigeminal nerve block: coronoid approach. In Waldman SD, edi-tor: Atlas of interventional pain management , ed 3, Philadelphia, 2009, Saunders,pp 47–50.

Williams MH, Broadley SA: SUNCT and SUNA: clinical features and medicaltreatment, J Clin Neurosci 15:527–534, 2008.


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1


Thunderclap headache is an uncommon type of headache thatmay be the result of an underlying vascular or nonvascular intra-cranial abnormality or may represent a primary headache syn-drome of unknown cause. Common and uncommon causes ofthunderclap headache are listed in Table 8-1. The more benign,though no less painful, primary thunderclap headache occurs overthree times more frequently than the serious secondary thunder-clap headache. Because of the often-threatening causes of the lesscommon secondary thunderclap headache (e.g., subarachnoidhemorrhage, cerebral venous thrombosis), urgent evaluationincluding computed tomography (CT) and/or magnetic resonanceimaging of the brain and cerebrospinal fluid analysis are indicatedin all patients suspected of having thunderclap headache.

One of the most severe headaches encountered in clinicalpractice, thunderclap headache is characterized by a very rapidonset to peak of less than 1 minute. The headache may lastfrom 1 to 10 days and, because of its intensity, almost alwaysprovokes an urgent trip to the emergency department, wherethe headache is invariably initially misdiagnosed as the sentinelheadache of acute subarachnoid hemorrhage or other potentiallycatastrophic headache syndromes (Tables 8-2 and 8-3). This isnot surprising in that primary thunderclap headache is virtuallyindistinguishable clinically from subarachnoid hemorrhage, oneof the most neurologically devastating forms of cerebrovascularaccident. Thus, because of the serious consequences of misdiag-nosis, by necessity primary thunderclap headache is a diagnosisof exclusion.

SIGNS AND S YMPTOMS

As mentioned earlier, primary thunderclap headache is char-acterized by a very rapid onset to peak of less than 1 minute without obvious inciting factors (e.g., sexual activity, cough-ing, straining at stool). The patient with primary thunderclapheadache is almost always convinced that he or she is havinga stroke and often appears frightened and anxious. The head-ache of primary thunderclap headache can be located anywherein the head or neck. Nausea and vomiting is present approxi-mately 75% of the time. However, the nuchal rigidity and other

focal neurological signs often associated with acute subarachnohemorrhage and other neurologically devastating syndromes which severe headache of acute onset are a prominent feature auniformly absent.

TESTING

Testing in patients suspected of having primary thundercl

headache has two immediate goals: (1) to identify occult intrcranial pathological conditions or other diseases that may mimprimary thunderclap headache and may require specific urgetreatment and (2) to identify the presence of subarachnohemorrhage. All patients with a recent onset of severe headacthought to be secondary to primary thunderclap headache shouundergo emergent CT of the brain to rule out any pathologiccondition that could be responsible for the patient’s symptom

Chapter 8

PRIMARY THUNDERCLAP

HEADACHE

TABLE 8-1

Main and Rare Causes of Thunderclap Headache

Main Causes Rare Causes

Vascular disorders

Subarachnoid hemorrhage Pituitary apoplexy, arteritis,angiitis

Intracerebral hemorrhage Unruptured vascularmalformation, aneurysm

Cerebral venous thrombosis Arterial hypertension

Spontaneous intracranialhypotension

Cerebral segmentalvasoconstriction

Cervical artery dissection

Nonvascular disorders

Greater occipital neuralgia

Intermittent hydrocephalus bycolloid cyst

Infections

Meningitis, encephalitis Erve virus

Sinusitis

Primary headache disorders

Migraine Cluster headache

Primary thunderclap headache Tension headache, new dailypersistent headache

Primary exertional headache

Primary cough headache

Linn FHH: Primary thunderclap headache. In: Aminoff MJ, editor: Handbook ofclinical neurology, vol 97, New York, 2010, Elsevier, pp 473–481.

ICD-9 CODE 339.43

ICD-10 CODE G 44.53



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(Figure 8-1). Modern multidetector CT scanners have a diagnos-tic accuracy approaching 100% for subarachnoid hemorrhage ifCT angiography of the cerebral vessels is part of the scanningprotocol. Cerebral angiography may also be required if surgicalintervention is being considered and the site of bleeding cannotbe accurately identified.

Magnetic resonance imaging (MRI) of the brain and magneticresonance angiography may be useful if an aneurysm is not identi-fied on CT studies and may be more accurate in the diagnosis ofarteriovenous malformations (Figure 8-2). Screening laboratorytests, including an erythrocyte sedimentation rate, complete bloodcount, coagulation studies, and automated blood chemistry,

should be performed in patients with subarachnoid hemorrhage.Blood typing and crossmatching should be considered in anypatient in whom surgery is being contemplated or who has pre-existing anemia. Careful serial ophthalmological examinationshould be performed on all patients with subarachnoid hemor-rhage to chart the course of papilledema.

Lumbar puncture is useful in revealing the presence or absenceof blood in the spinal fluid, but its utility may be limited by thepresence of increased intracranial pressure, making lumbar punc-ture too dangerous. Electrocardiographic abnormalities are com-mon in patients with subarachnoid hemorrhage and are thoughtto be due to abnormally high levels of circulating catecholamines

TABLE 8-2

Comparison of Primary Thunderclap Headache andSubarachnoid Hemorrhage

Comparison FactorsPrimary ThunderclapHeadache

SubarachnoidHemorrhage

Severe headache Yes Yes

Nausea and vomiting Yes Yes

Focal neurological signs No YesNuchal rigidity No Yes

Photophobia No Yes

Vertigo No Yes

Neck and back pain No Yes

TABLE 8-3

Diseases That May Mimic Primary ThunderclapHeadache

Hemorrhagic

Ischemic

NeoplasmInfection

Meningitis

Encephalitis

Abscess

Parasitic

Hypertensive crisis

Loss of spinal fluid

Postdural puncture headache

Spontaneous spinal fluid leak

Collagen-vascular disease

Lupus cerebritis

Vasculitis

Polymyositis

Headache

Cluster headache

Primary exertional headache

Primary cough headache

Migraine

Ice pick headache

Primary sexual headache

A

B

C

Figure 8-1 Computed tomography scan showing subarachnoid hem-orrhage (SAH). Right middle cerebral artery aneurysm in a 58-year-oldman with SAH and intracranial hematoma (IH). A, Volume renderingimage from computed tomography angiography (CTA) clearly displaysthe relationship of the aneurysm to bone structures, adjacent branchvessels, and aneurysmal neck (arrow). B, Maximum intensity projec-tion (MIP) image from CTA clearly demonstrates the relationship of theaneurysm (arrow). C, Thin-MIP image from CTA shows the relationshipof the aneurysm to IH (arrowhead), and the ruptured aneurysm has asmall “nipple” (arrow). (From Chen W, Yang Y, Xing W, et al: Applicationsof multislice CT angiography in the surgical clipping and endovascular coil-ing of intracranial aneurysms, J Biomed Res 24:467–473, 2010.)



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8 • Primary Thunderclap Headache 2

and hypothalamic dysfunction; however, they are rarely present inpatients with primary thunderclap headache.


The differential diagnosis of primary thunderclap headache gen-erally can be thought of as the diagnosis of the lesser of two evilsbecause most of the diseases that mimic primary thunderclapheadache are also associated with significant mortality and mor-bidity. Table 8-3 lists diseases that may be mistaken for primarythunderclap headache. Prominent among them is subarachnoid

hemorrhage, stroke, collagen-vascular disease, infection, neo-plasm, hypertensive crisis, spinal fluid leaks, and a variety of morebenign causes of headache.

TREATMENT

Although no generally accepted treatment for primary thunder-clap headache has been defined, the following guidelines may beuseful for the clinician when faced with a patient thought to havethis uncommon headache syndrome. First and foremost, if testresults reveal no evidence of intracranial pathology or other seri-ous, life-threatening diseases, constant reassurance that the patient

does not have a stroke or brain tumor is indicated. In generdrugs used to treat headaches whose primary mechanism of actiois vasoconstriction (e.g., ergots, triptans) should be avoided. Anedotal reports indicate that intravenous nimodipine may help aboacute attacks and prevent recurrent headache episodes. Gabapetin also has been advocated as a reasonable treatment for primathunderclap headache and given its favorable risk-to-benefit ratmay be a reasonable therapeutic option.


Complications and pitfalls in the diagnosis and treatment of pmary thunderclap headache generally fall into three categorieThe first category involves the failure to recognize a sentinel bleassociated with subarachnoid hemorrhage and evaluate and trethe patient before significant morbidity or mortality occurs. Thsecond category involves misdiagnosis that results in unnecessatesting, in particular, cerebral angiography, which itself is assoated with significant morbidity and rarely death. The third caegory involves iatrogenic morbidity and rarely mortality from tuse of medications to treat primary thunderclap headache (e.triptans, ergots) that not only do not treat this primary headacsyndrome but also have significant side effects.

F

A B C

D E

Figure 8-2 Magnetic resonance imaging showing arteriovenous malformation. Patient with aneurysm-related false aneurysm (FA) in right parieregion. Preangiographic T1-weighted magnetic resonance axial image (A) and T2-weighted magnetic resonance coronal image (B) show round lesi(arrow) with flow void and mixed signal in the center and mixed signal on the periphery. Fluid attenuated inversion recovery image (C) reveals smarea of surrounding edema (arrow). D, Flow in the center of FA (arrow) on two-dimensional time-of-flight magnetic resonance angiography. E, Preebolization digital subtraction angiography image. F, Residual inflow to FA (arrow) on postembolization DSA image. (From Brzozowski K, FrankowskaPiasecki P, et al. The use of routine imaging data in diagnosis of cerebral pseudoaneurysm prior to angiography, Eur J Radiol. 80:e401–e409, 2011.)

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SUGGESTED READINGS

Anderson T: Current and evolving management of subarachnoid hemorrhage,Crit Care Nurs Clin North Am 21:529–539, 2009.

Chih-Ming H, Ya-Ju L, Yang-Kai F, Shih-Pin C, Tzu-Hsien L: Isolated thunder-clap headache during sex: orgasmic headache or reversible cerebral vasoconstric-tion syndrome? J Clin Neurosci 17:1349–1351, 2010.

de Bruijn SFTM, Stam J, Kappelle LJ: CVST Study Group: Thunderclap headache asfirst symptom of cerebral venous sinus thrombosis, Lancet 348:1623–1625, 1996.

Janardhan V, Biondi A, Riina HA, et al: Vasospasm in aneurysmal subarachnoidhemorrhage: diagnosis, prevention, and management, Neuroimaging Clin N Am

6:483–496, 2006.Linn FHH: Primary thunderclap headache. In: Aminoff MJ, editor: Handbook of

clinical neurology, vol 97, New York, 2010, Elsevier, pp 473–481.Manno EM: Subarachnoid hemorrhage, Neurol Clin 22:347–366, 2004.Newfield P: Intracranial aneurysms: vasospasm and other issues. In Atlee JL, editor:

Complications in anesthesia , ed 2, Philadelphia, 2007, Saunders, pp 719–723.Palestrant D, Connolly ES: Subarachnoid hemorrhage, Neurobiol Dis 265–270,

2007.Pouration N, Dumont AS, Kassell NF: Subarachnoid hemorrhage. In Alves W

and Skolnick B, editors: Handbook of neuroemergency clinical trials , New York,2006, Elsevier, pp 17–44.

Clinical Pearls

Primary thunderclap headache is a diagnosis of exclusion.It is frequently misdiagnosed as the sentinel headache ofsubarachnoid hemorrhage, causing the treating physicianto order urgent diagnostic testing, which is associated withits own significant mortality and morbidity. The lack offocal neurological findings in a patient with acute head-ache should point the clinician toward the diagnosis ofbenign primary headaches including primary thunderclapheadache, cough headache, exertional headache atypicalmigraine, and headache associated with sexual activity. Thisdoes not mean that urgent computerized scanning of thebrain and analysis of the patient’s cerebrospinal fluid arenot indicated.


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suspected even if blood is not present on MRI or CT. Plain radio-graphs of the cervical spine also may be useful in the evaluationof Arnold-Chiari malformations and should be included in theevaluation of all patients with hypnic headache.

Screening laboratory tests consisting of complete blood cellcount, erythrocyte sedimentation rate, and automated bloodchemistry testing should be performed if the diagnosis of hypnicheadache is in question. Intraocular pressure should be measuredif glaucoma is suspected.


Hypnic headache is a clinical diagnosis supported by a combina-tion of clinical history, physical examination, radiography, MRI,and MRA. Pain syndromes that may mimic hypnic headacheinclude the uncommon primary headaches benign exertionalheadache, ice pick headache, and sexual headache, although theunique same-time nocturnal occurrence should help the clinicianeasily identify the patient’s symptoms as hypnic headache. Theclinician must consider other types of headache that occur morefrequently at night, including cluster headache and headachesassociated with sleep apnea, nocturnal arterial hypertension, anal-

gesic rebound, and increased intracranial pressure (Table 9-1).Less commonly, hypnic headache may be confused with trigem-inal neuralgia involving the first division of the trigeminal nerve ordemyelinating disease. Trigeminal neuralgia involving the first divi-sion of the trigeminal nerve is uncommon and is characterized bytrigger areas and tic-like movements. Demyelinating disease is gen-erally associated with other neurological findings, including opticneuritis and other motor and sensory abnormalities. The pain ofchronic paroxysmal hemicrania and cluster headache is associated

with redness and watering of the ipsilateral eye, nasal congestion,and rhinorrhea during the headache. These findings are absent inhypnic headache. Migraine headache may or may not be associ-ated with painless neurological findings known as aura, but patientsalmost always report some systemic symptoms, such as nausea or

photophobia, not typically associated with hypnic headache.

TREATMENT

Indomethacin and lithium carbonate are the treatments of choicefor hypnic headache, with indomethacin being slightly moreeffective for the unilateral form of the syndrome. Indomethacinat a starting dose of 25 mg daily for 2 days and titrating to 25 mgthree times per day is a reasonable treatment approach. This dosemay be carefully increased up to 150 mg per day. Indometha-cin must be used carefully, if at all, in patients with peptic ulcerdisease or impaired renal function. Headache specialists havenoted anecdotal reports of a positive response to cyclooxygenase-2(COX-2) inhibitors in the treatment of benign hypnic headache.

Lithium carbonate is used in the same manner as in the treatmentof cluster headache and has its basis in use in its proven efficacy inthe treatment of other diseases thought to have a chronobiologicalbasis, such as cluster headache and bipolar disorders. However,the therapeutic window of lithium carbonate is small and thisdrug should be used with caution. A starting dose of 300 mg atbedtime may be increased after 48 hours to 300 mg twice perday. If no side effects are noted after 48 hours, the dose may beincreased again to 300 mg three times per day. Anecdotal reportsexist that gabapentin and pregabalin also may be useful in decreas-ing the frequency and intensity of attacks of hypnic headache.Unlike with cluster headache, oxygen inhalation has been ineffec-tive in aborting attacks of hypnic headache once the patient hasbeen awakened by the pain.


Failure to diagnose hypnic headache correctly may put thepatient at risk if an intracranial pathological condition or demye-linating disease (which may rarely mimic the clinical presentationof hypnic headache) is overlooked. MRI and MRA are indicatedin all patients thought to have hypnic headache. Failure to diag-

nose glaucoma, which also may cause intermittent ocular pain,may result in permanent loss of sight.

SUGGESTED READINGS

Alberti A: Headache and sleep, Sleep Med Rev 10:431–437, 2006.Berciano J, Poca M-A, García A, Sahuquillo J: Paroxysmal cervicobrachial hypnic-

induced pain in a patient with syringomyelia extending into spinal cord poste-rior gray horns, J Neurol 254:678–681, 2007.

Chen Y-Y, Lirng J-F, Fuh J-L, et al: Primary hypnic headache is associated with

posterior fossa crowdedness: a morphometric MRI study, Cephalalgia 24:694–699, 2004.

Fowler MV, Capobianco DJ, Dodick DW: Headache in the elderly, Semin Pain Med 2:123–128, 2004.

Manni R, Ghiotto N: In Aminoff M, editor: Handbook of clinical neurology , New York, 2010, Elsevier, pp 469–472.

Pascual J: Primary hypnic headache, Curr Pain Headache Rep 9:272–276, 2005.Pascual J, González-Mandly A, Martín R, Oterino A: Headaches precipitated by

cough, prolonged exercise or sexual activity: a prospective etiological and clini-cal study, J Headache Pain 9:259–266, 2008.

Waldman SD: Arnold Chiari malformation type I. In Waldman SD, CampbellRS, editors: Imaging of pain, Philadelphia, 2011, Saunders, pp 27–28.

Waldman SD: Arnold Chiari malformation type II. In Waldman SD, CampbellRS, editors: Imaging of pain, Philadelphia, 2011, Saunders, pp 29–30.

TABLE 9-1

Nocturnal Headaches That May Be Confused withHypnic Headache

Cluster headache

Headache associated with sleep apnea

Headache associated with nocturnal arterial hypertension

Headache associated with increased intracranial pressure

Analgesic rebound headache

Clinical Pearls

Any patient presenting with nocturnal headaches shouldbe taken very seriously. Although statistically most of theseheadaches ultimately are proved to be of benign cause, afew patients have potentially life-threatening disease. Thediagnosis of hypnic headache is made by obtaining a thor-ough, targeted headache history and performing a carefulphysical examination. The clinician must separate patients

with hypnic headache from patients with headaches causedby an intracranial pathological condition such as tumors

or systemic disease such as nocturnal arterial hyperten-sion. Patients with hypnic headache should have a normalneurological examination. If the neurological examina-tion is abnormal, the diagnosis of benign hypnic headacheshould be discarded and a careful search for the cause of thepatient’s neurological findings should be undertaken.

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2


Nummular headache is an uncommon chronic headache syn-drome characterized by constant localized pain with superim-posed paroxysms of stabbing jabs and jolts of mild to moderateintensity that occur in a coin-shaped localized area of the scalp.Most commonly located in the parietal region, the pain of num-mular headache is unilateral and localized to a single area. It rarelyif ever switches sides. The scalp overlying the area may be tenderto touch and stimulation of the area; for example, the brushing ofhair may exacerbate the pain. Nummular headache occurs slightlymore commonly in women and is generally not seen before thefourth decade of life, but rare reports of children suffering fromnummular headache sporadically appear in the literature. Num-mular headache is also known as coin-shaped headache.

SIGNS AND S YMPTOMS

A patient with nummular headache complains of a unifocal regionof pain and sensitivity most commonly occurring in the vertexof the parietal region (Figure 10-1). The pain is almost alwaysunilateral and does not switch sides, although rare reports exist ofbilateral nummular headache. Some patients describe the pain ofnummular headache as a constant dull ache or sensitivity in theaffected area with superimposed paroxysms of lancinating pain.The pain is chronic, although spontaneous remissions have beenrarely reported. Some patients with nummular headache exhibitanxiety and depression because the intensity of the associated painleads many patients to believe they have a brain tumor.

TESTING

Magnetic resonance imaging (MRI) of the brain provides the bestinformation regarding the cranial vault and its contents. MRI ishighly accurate and helps identify abnormalities that may put thepatient at risk for neurological disasters secondary to intracranialand brainstem pathological conditions, including tumors andcalvarial lesions (Figure 10-2). Magnetic resonance angiography(MRA) also may be useful in helping identify aneurysms, whichmay be responsible for the patient’s pain. In patients who can-not undergo MRI, such as patients with pacemakers, computed

tomography (CT) is a reasonable second choice. Radionuclibone scanning and plain radiography are indicated if fracture bony abnormality, such as metastatic disease, is considered in tdifferential diagnosis.

Screening laboratory tests consisting of complete blood ccount, erythrocyte sedimentation rate, and automated bloochemistry should be performed if the diagnosis of nummulheadache is in question.


Nummular headache is a clinical diagnosis supported by a combination of clinical history, normal physical examination, radography, and MRI. Pain syndromes that may mimic nummulheadache include chronic paroxysmal hemicranias and jolts an

jabs headache. Trigeminal neuralgia involving the first divisioof the trigeminal nerve is uncommon and is characterized by triger areas and tic-like movements. Demyelinating disease is geneally associated with other neurological findings, including optneuritis and other motor and sensory abnormalities. The pain chronic paroxysmal hemicrania lasts much longer than the pain nummular headache and is associated with redness and waterin

of the ipsilateral eye.

Chapter 10

NUMMULAR HEADACHE

Figure 10-1 Patients suffering from nummular headache complain unifocal area of pain and scalp sensitivity.

ICD-9 CODE 784.0

ICD-10 CODE R51



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TREATMENT

Nummular headache uniformly responds to treatment with indo-methacin. Failure to respond to indomethacin puts the diagnosisof nummular headache in question. A starting dosage of 25 mgdaily for 2 days and titrating to 25 mg three times a day is a rea-sonable treatment approach. This dose may be carefully increasedto 150 mg/day. Indomethacin must be used carefully, if at all,in patients with peptic ulcer disease or impaired renal function.

Anecdotal reports of a positive response to cyclooxygenase-2

(COX-2) inhibitors in the treatment of nummular headache havebeen noted in the headache literature, as well as a successful treat-ment with gabapentin. Underlying sleep disturbance and depres-sion are best treated with a tricyclic antidepressant compound,such as nortriptyline, which can be started at a single bedtimedose of 25 mg.


Failure to diagnose nummular headache correctly may put thepatient at risk if an intracranial pathological condition or calvarialdisease, which may mimic the clinical presentation of nummularheadache, is overlooked. MRI is indicated in all patients thoughtto have nummular headache.

SUGGESTED READINGS

Cohen GL: Nummular headache: what denomination? Headache 10:1417–1418,2005.

Evens RW, Pareja JA: Nummular headache, Headache 45:164–165, 2005.Mathew NT: Indomethacin responsive headache syndromes, Headache J Head

Face Pain 21:147–150, 1981.Pareja JA, Caminero AB, Serra J, et al: Nummular headache: a coin-shaped ceph-

algia, Neurology 58:1678–1679, 2002.Pareja JA, Pareja J, Barriga FJ,

atlas of uncommon pain syndromes. expert consult - online and print

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