Thunderclap Headache

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Thunderclap Headache

David W. Dodick, MD

Address

Department of Neurology, Mayo Clinic, 13400 East Shea Blvd.,

Scottsdale, AZ 85259, USA.

E-mail: dodick.david@mayo.edu

Current Pain and Headache Reports 2002, 6:226–232

Current Science Inc. ISSN 1531–3433

Introduction

Thunderclap headache (TCH) refers to an excruciating

headache of instantaneous onset. It is as sudden and

unexpected as a “clap of thunder.” Although the term was

used initially to describe a headache that had character-

istics of an unruptured cerebral aneurysm [1], a number of

other disorders have symptoms similar to TCH, including

subarachnoid hemorrhage (SAH), cerebral venous sinus

thrombosis (CVST), pituitary apoplexy, spontaneous

intracranial hypotension, hypertensive encephalopathy,

and retroclival hematomas [2–8]. In some patients with

TCH, there may be no underlying secondary cause; how-

ever transient diffuse vasospasm in the absence of SAH is

detected on an angiography in others. TCH has been

considered to be a migraine variant (crash migraine) in

patients with a history of migraine. Patients with TCH or

serious underlying pathology frequently may have normal

neurologic examination results and normal computed

tomographic (CT) brain scans. This has created confusion

regarding nosology and the nature and extent of the

diagnostic evaluation.

Pathophysiology

The pathogenesis of secondary TCH is related to the nature

of the underlying pathology. The mechanism usually

involves an acute insult of pain-sensitive intracranial

structures (eg, meninges in SAH or dural venous sinuses in

acute thrombosis). The pathophysiology or mechanisms

that underlie idiopathic TCH with segmental vasospasm is

unclear. The occurrence of TCH during physical activity in

patients with pheochromocytoma or acute hypertensive

crisis, and in patients who ingest sympathomimetic drugs

or tyramine-containing foods and concurrently use

monoamine oxidase inhibitors, suggests that excessive

sympathetic activity or an abnormal vascular response to

circulating catecholamines may exist. To support the

hypothesis that TCH is a radiographic entity, reversible

cerebral vasospasm has also been associated with pheo-

chromocytoma, eclampsia, and sympathomimetic drug

(amphetamine, cocaine) intoxication [9–12].

Vasospasm (as opposed to arterial stenosis) usually

reflects a reversible process that consists of a localized

smooth muscle contraction of the blood vessel wall that

results in segmental luminal narrowing. Mechanical, bio-

chemical, and neurogenic stimuli can provoke vasospasm.

The abrupt onset of the headache and vasospasm may

imply a neurogenic mechanism. The perivascular and

intramural portions of the intracranial arteries are filled

with neuropeptide Y, which contains sympathetic nerves. It

elicits concentration-dependent contractions in human

cerebral and meningeal blood vessels [13]. The vascular

caliber may reflect directly sympathetic tone. Sympathetic

receptor sensitivity is evident from experimental and

animal models of vasospasm in SAH [14] and the clinical

observation of multifocal vasospasm in patients with

pheochromocytoma and sympathomimetic drug intoxi-

cation. Therefore, it is possible for idiopathic TCH to

represent a spontaneous and aberrant central sympathetic

response. This is supported by the sudden and severe head-

ache in patients with paroxysmal hypertension because of

baroreceptor dysfunction and loss of inhibition of central

sympathetic reflexes [15].

Differential Diagnosis

Although idiopathic TCH may represent a distinct primary

headache syndrome, an indistinguishable headache profile

may occur in the setting of sinister intracranial and

extracranial vascular pathology. In addition to SAH (a well-

recognized cause for TCH), similar sudden peak-intensity

headaches, which may be undetectable in neurologic

examinations, can be a symptom of CVST, pituitary

apoplexy, cervicocephalic arterial dissection, acute hyper-

tensive crisis, spontaneous retroclival hematomas, and

spontaneous intracranial hypotension [2–8]. These dis-

orders, with the exception of SAH, may not be detected by

CT or lumbar puncture (LP).

Thunderclap headache refers to an excruciating headache

of instantaneous onset. It occurs as suddenly and unexpect-

edly as a “clap of thunder.” Patients with thunderclap

headache may have normal neurologic examination results

and normal computed tomographic brain scans, even if

they have serious underlying pathology. This has created

confusion regarding nosology and the nature and extent

of the diagnostic evaluation, which this article discusses.

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Cerebral Venous Sinus Thrombosis

Headache is the most common symptom of CVST and the

most frequent symptom in approximately 75% of patients.

The headache may be diffuse or localized, persistent, worse

on recumbancy, and aggravated by valsalva maneuver.

Although its onset is usually subacute over several days,

TCH may be the main symptom in 10% of patients with

CVST [3]. Overall, CT scan results are normal in approxi-

mately 25% of patients with CVST and in 50% or more of

patients with isolated intracranial hypertension; however,

it is normal in fewer than 10% of patients who have focal

neurologic signs [16,17]. Although some combination of

lymphocytic pleocytosis, red blood cells, and elevated pro-

tein levels is present in 30% to 50% of patients, 40% of

patients with CVST may have elevated opening pressure

without alteration in cytochemistry studies [18,19]. If there

is clinical suspicion of CVST, the CT scan is supplanted by

magnetic resonance imaging (MRI), which should be the

initial investigation [20].

Carotid Artery Dissection

Headache is the earliest and most common clinical

manifestation of symptomatic internal carotid artery (ICA)

dissection and can occur in 75% of patients [21]. Oculosym-

pathetic paresis (Horner’s syndrome) and unilateral head-

ache (especially in the anterior head region) is strongly

suggestive of internal carotid artery dissection. Acute hemic-

ranial headache with delayed focal cerebral ischemic events

is another common symptom of internal carotid artery

dissection. The onset of headache is instantaneous and

severe in approximately 13% of the patients [22]. CT

scanning and LPare unrevealingmodesoftestingunlessthey

are accompanied by an ischemic stroke; magnetic resonance

angiography (MRA) is becoming the imaging modality of

choice for demonstrating the arterial dissection. The out-

come of internal carotid artery dissection is generally favor-

able, but permanent neurologic deficits or death may result

[23,24]. Although controlled trials are unavailable, early

detection of arterial dissection may allow early initiation of

antiplatelet or anticoagulation therapy, possibly preventing a

more serious cerebral ischemic complication.

Spontaneous Intracranial Hypotension

Schievink et al. [5] observed 28 consecutive patients with

spontaneous intracranial hypotension (SIH) secondary to

spinal cerebrospinal fluid (CSF) leaks and found that 14%

(four of 28) had TCH. Nuchal rigidity was observed in

three of the patients who sought early medical attention.

These patients underwent CT scanning, LP, and cerebral

angiography to exclude an aneurysmal SAH. Despite the

negative results of these initial studies, the patients showed

evidence of brain or cerebellar tonsillar descent, and

pachymeningeal thickening and enhancement, which are

characteristic findings of this syndrome on MRIs.

Pituitary Apoplexy

Pituitary apoplexy has been reported to present with TCH

in the absence of positive findings on clinical, CT, and CSF

examinations [2,6]. Pituitary apoplexy is an uncommon

clinical syndrome usually characterized by acute headache,

ophthalmoplegia, diminished visual acuity, and altered

mental status. It is caused by the sudden infarction or

hemorrhage of the pituitary gland that invariably harbors

an adenoma. However, the clinical manifestations of pitu-

itary apoplexy are protean, ranging froma clinically benign

event to a catastrophic illness with adrenal crisis, coma, or

sudden death. This illustrates that the pituitary tumor,

which is isodense to brain tissue, can be overlooked easily

on routine CT, even if a hemorrhage is present in the gland.

MRI readily identifies the tumor and the associated hemor-

rhage. These cases emphasize the importance of consider-

ing this potentially devastating disorder as a cause of TCH,

particularly if initial investigations seem unrevealing and

underscore the value of MRI in detecting underlying causes

for TCH, which may not be obvious on the initial CT or LP.

Retroclival Hemorrhage

Spontaneous retroclival hematomas have been reported to

be symptoms of TCH [8]. Although clival hematomas are

unusual complications of severe head and neck injuries

and can be accompanied by atlantoaxial dislocation,

reports of spontaneous hemorrhage have been reported. In

the study by Schievink et al. [5], the neurologic examina-

tion results and CT brain scans were normal, but the MRI

disclosed an abnormality. This report highlights the need

for an MRI and a high index of suspicion for patients with

TCH if the initial CT and examination results are normal.

Unruptured or Thrombosed Intracranial

Aneurysms

An instantaneous severe headache has been recognized as

the signature feature of aneurysmal SAH, but approxi-

mately 20% to 50% of patients report a distinctive and

unusually severe headache in the days or weeks preceding

an aneurysmal SAH. This headache is often referred to as a

warning or sentinel headache [7,25–27] and has been

thought to represent a “warning leak” or small SAH. Linn

et al. [28] have questioned this hypothesis by demon-

strating a frequency of severe headaches in only 11% of

patients in the month preceding an SAH. They suggested

the possibility that the severity of a prior headache episode

is overinterpreted (recall bias) in the context of serious

brain disease; this may account for the higher frequency of

sentinel headaches in other studies.

In 1986, the term thunderclap headache was intro-

duced to describe a similar headache as a symptom of an

unruptured cerebral aneurysm [1]. The authors observed a

42-year-old woman who suffered three TCHs within 1

week. The results of a CT brain scan and CSF examinations

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Uncommon Headache Syndromes

were normal. Despite the absence of blood within the CSF,

conventional angiography revealed a diffuse multifocal and

segmental cerebral vasospasm and a saccular aneurysm at

the point of origin of the right posterior cerebral artery.

However, no evidence of hemorrhage was observed during

an aneurysmal surgery. Four weeks later, the results of the

follow-up angiogram were normal. The authors concluded

that unruptured intracranial aneurysms can have symptoms

of thunderclap headache. Cerebral angiography is necessary

for these patients, even if the CT and CSF results are normal.

A similar case was reported 2 years later when a 50-year-old

woman had recurrent TCHs in a 13-day period and the CT

or CSF showed no evidence of blood [29]. An angiography

revealed a wide-neck (44-mm) aneurysm of the ICA with

evidence of vasospasm in the right ICA and middle cerebral

arteries; however, there was no evidence of rupture during

surgery. Recently, a 50-year-old woman who had TCH and

normal CT and CSF results in the setting of a posterior

communicating artery aneurysm (with daughter sac) was

reported [30]. At surgery, there was no evidence of hemor-

rhage or rupture, although the aneurysm dome was “thin-

walled.” Because the prevalence of unruptured intracranial

aneurysms in the general population is 3.6% to 6% [31], it

is debatable if the aneurysms experienced by the patients

mentioned earlier were an incidental finding or an unrup-

tured saccular aneurysm, which can have symptoms of TCH

and can produce normal CT, CSF, and neurologic examina-

tion results [32,33]. Because approximately 12%of patients

with SAH have the “worst headache of their life” and

normal neurologic examination results, this issue takes on

singular importance [7]. The answer to this question would

also answer the question: should any patient undergo

conventional angiography?

The most compelling evidence that supports the idea

that TCH is symptomatic of an unruptured aneurysm was a

report of a 53-year-old woman with a sudden severe head-

ache whose CT scan and CSF test results were normal [34].

Two days later, the patient was unresponsive and died

shortly after an angiogram revealed a right internal carotid

aneurysm. Unfortunately, this report was presented in an

abstract. The cause of death or possible evidence of SAH

was not disclosed.

In one retrospective study, seven of 111 patients with

symptomatic unruptured saccular aneurysms had TCH [35].

Some of the patients did not have CSF examinations. In

addition, there were abnormal neurologic findings or other

clinical and imaging features that posed little diagnostic

confusion regarding the presence of an underlying cause for

the headache. Therefore, this study did not address patients

with TCH who have normal CT, CSF, and neurologic exami-

nation results. In another retrospective study [36], 562

patients had sudden severe headache, normal CT results,

and “colorless”CSF. Cerebral aneurysms werereportedin52

patients (9.3%). Of the 46 patients who were taken to

surgery, eight (1.4%) had a “minor leak,” although it is

unclear how this was determined. Collectively, these data

have prompted the recommendation that angiography be

obtained in patients with TCH who have normal CT, CSF,

and neurologic examination results because of the

potentially devastating consequences of overlooking a

symptomatic saccular aneurysm.

Evidence against an association between unruptured

inctracranial aneurysms and TCH is derived from several

prospective studies, which have concluded that angio-

graphy is not indicated in patients with TCH and a blood-

less CT and CSF examination [37‧,41]. In one prospective

study [37‧], 71 patients with TCH and negative CT scan

and CSF results were followed for a mean of 3.3 years.

None of these patients suffered SAH during the follow-up

period, although 27% experienced similar headaches

before or subsequent to the index event. Similarly, other

studies have reported recurrent TCH in 8% to 44% of

patients after the first occurence [38–41], suggesting that it

may be a unique and recurrent, benign primary headache

disorder. Thus most patients who have TCH and normal

CT and CSF examination results will not harbor a symp-

tomatic cerebral aneurysm. The outcome and prognosis is

benign in the absence of other intracranial causes. To deter-

mine which patients require further investigation and

which diagnostic studies should be pursued, the clinical

index of suspicion of other disorders that may occur with

TCH and normal neurologic, CT, and CSF examination

results should be considered.

Benign (Idiopathic) Thunderclap Headache

Thunderclap headache may occur as a benign and poten-

tially recurrent idiopathic headache disorder in the

absence of organic intracranial pathology [32,37–41].

Before the introduction of the term in 1986, benign TCH

was referred to as a variety of terms or clinical circum-

stances such as benign vascular headache, migrainous

vasospasm, crash migraine, benign sexual (‘coital’) head-

ache type II, benign isolated cerebral vasculitis, and benign

angiopathy of the nervous system [42–49]. Although some

patients with idiopathic TCH may have a history of

migraine, the clinical presentation and temporal profile of

TCH is unique. A characteristic clinical and angiographic

pattern has emerged and diagnostic criteria have been pro-

posed [15,32,43,50]. The headache, which is sudden and

reaches maximum intensity within 30 seconds, usually

lasts several hours; however, a lingering, less severe head-

ache may persist for weeks. Episodes of TCH may occur

repeatedly over a period of 7 to 14 days. TCH may recur

over subsequent months to years in one-third of patients.

From the available literature, it appears that idiopathic

TCH may be associated with angiographic evidence of

diffuse segmental vasospasm [32,50]. These headaches may

occur spontaneously when patients are at rest or during light

activities, or may be precipitated by intense exertion, valsalva

maneuvers, or sexual activity. Exercise, weight-lifting, or

sexual intercourse is a precipitating event in up to one-third

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of patients with idiopathic TCH. Benign sexual headache

type II likely represents idiopathic TCH, and reflects the

influence of exertion on this headache disorder rather than

having a unique relationship with sexual activity.

Thunderclap headache, which is associated with diffuse

vasospasm in the absence of SAH, may be associated with

fluctuating focal neurologic signs or symptoms, seizures,

and stroke [51,52]. Angiography demonstrates alternating

segments of arterial constriction and dilation involving

large cerebral arteries at the circle of Willis and their

second- and third-order branches. These cases, which occur

in the absence of an obvious precipitating cause other than

exertion, should be distinguished from “benign angio-

pathy of the central nervous system (CNS).” In these

reported cases, a number of associations have been identi-

fied including the ingestion of ergotamine derivatives and

the postpartum period (postpartum angiopathy); the latter

has been related to bromocriptine in some patients

[53,54]. Similar to the idiopathic headache, this disorder is

often self-limited and resolves within 2 months without

treatment. However, when cerebral vasospasm is present,

careful clinical monitoring is important. Although the

evidence is purely anecdotal, nimodipine may be used to

prevent or ameliorate the risk of a delayed ischemic deficit

[51]. Although there is limited knowledge regarding their

pathogenesis, these two entities may be synonymous.

However, idiopathic TCH should remain a separate dis-

order. The use of the termangiopathy should be avoided in

cases of spontaneous TCH until evidence of abnormal

vascular histopathology is demonstrated.

Idiopathic TCH with vasospasm must be distinguished

from inflammatory CNS vasculitis because the outcome,

management, and prognosis of each are different. Isolated

CNS vasculitis is not known to occur with TCH in the

absence of SAH [55], and affected patients often have abnor-

mal spinal fluid examination results. Isolated CNS vasculitis

is invariably restricted to blood vessels that are less than 0.5

mm in diameter and not in the large blood vessels that

constitute the circle of Willis and their second-order

branches, as observed in patients with TCHand vasospasm.

The striking similarity between the clinical presenta-

tion and angiographic features of idiopathic TCH with

vasospasm and the reported cases of unruptured

aneurysms that resemble TCH suggest that the unruptured

aneurysms observed in these patients were incidental and

unrelated to the headache or the vasospasm [1,29,30]. It

would be difficult to account for a mechanism through

which an unruptured aneurysm could precipitate diffuse,

segmental, or cerebral vasospasm in the absence of

subarachnoid blood.

Crash Migraine

It has been suggested that idiopathic TCH is a migraine

variant and may be a harbinger for future migraine attacks

[37,44]. Indeed, the occurrence of an unusual sudden severe

headache that was self-limited and accompanied by nausea

and vomiting in patients with a history of migraine was the

basis for using the term crash migraine [44]. The original

description of crash migraine resembles that of idiopathic

TCH. Fisher [44] referred to crash migraine as a headache

that reaches a high intensity “out of the blue” in 10 or 20

seconds, suggesting SAH; however, LP and angiography

results are normal. The headache may be situated posteriorly

or generalized. Vomiting occurs in approximately 50% of

patients. There may be a recurrence 2 or 3 days later. In 10

patients, long-termfollow-up revealed no disaster.

The clinical features, temporal pattern, mode of onset,

and associated features of idiopathic TCH are unique and

distinct from the well-established clinical criteria of

migraine and tension-type headache. The International

Headache Society Classification has established a uniform

terminology for headache disorders in which the diagnosis

is based on clinical features [56]. By providing homo-

genous patient population samples, these criteria have

facilitated an enormous advance in our knowledge of the

epidemiology and pathophysiology of several primary

headache disorders. This has led to remarkably effective

treatments for some of these disorders. To gain insights

into the epidemiology, clinical spectrum, pathophysiology,

appropriate management, and prognosis of patients with

this disorder, clinical criteria must be established to create

homogenous patient populations to observe in a prospec-

tive fashion. If idiopathic TCH was considered a migraine

or tension-type headache variant, it could lead to an

erroneous clinical diagnosis and complacency regarding

the need for investigations, especially in patients with a

history of migraine headache. Therefore, until the patho-

genesis of idiopathic TCH is elucidated, the term crash

migraine should be abandoned to avoid the misunder-

standing that this is a legitimate migraine.

Diagnostic Evaluation

Idiopathic TCH is a diagnosis of exclusion. This statement

cannot be overemphasized because of the serious nature of

the potential intracranial causes. Sudden severe headache

is the most common symptom of an SAH. It may precede

an imminent SAH in 10%to 50% of patients or may be the

only manifestation of an SAH. Unfortunately, there are no

characteristics of the headache that reliably distinguish

between SAH and idiopathic TCH; therefore, a thorough

diagnostic evaluation is mandatory [57]. CT scanning is

obligatory in all patients. Retrospective and prospective

studies, which have evaluated third-generation CT scanners

in SAH, reveal a sensitivity of 100% and 98%, respectively,

within the first 12 hours after the onset of headache, and

93% within the first 24 hours [7]. Although these figures

are promising, expert neuroradiologists interpreted the CT

scans in most of these studies. In general practice, physi-

cians are less experienced in reading CT scans, so subtle

abnormalities are undoubtedly overlooked. Furthermore,

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the sensitivity of CT scanning decreases to 86% 1 day after

the onset of headache, 76% after 2 days, and 58% after 5

days [58]. Therefore, an LP is required for patients whose

initial CT scan results are negative, equivocal, or technically

inadequate. The CSF pressure should always be measured

because high intracranial pressure may indicate the

presence of CVST for which approximately 25% of CT scan

results will be negative. Elevated opening pressure may

help distinguish between a traumatic LP and an SAH. It is

vital that this distinction be made because approximately

20% of LPs are traumatic, and observing three successive

tubes for a decrement in the erythrocyte count is not

entirely reliable. In addition, erythrocytes resulting from a

traumatic tap may undergo lysis in vitro if the fluid is not

promptly centrifuged and examined, producing xanth-

achromia from the formation of oxyhemoglobin. Visual

inspection for xanthachromia can miss discoloration in

50% of specimens. The detection of xanthachromia by

spectophotometry is the most accurate test for SAH

because it has a sensitivity of 100% if an LP is performed

12 hours to 2 weeks after the ictus [59].

The contentious issue has been whether intra-arterial

digital subtraction angiography (IADSA) is required to

exclude an unruptured aneurysm in patients with TCH,

anormal neurologic examination results, and normal CT

and CSF examination results. The evidence that unrup-

tured intracranial aneurysms can occur in this fashion is

scarce and incomplete. The aneurysm was likely an

incidental finding in most patients. The evidence from

prospective studies indicate that this presentation is

benign. In a retrospective study of 71 patients with TCH

whose CT and LP results were negative, none of them had

SAH during an average follow-up period of 3.3 years [37].

Furthermore, 225 patients with TCH and negative CT and

LP results were followed for 1 year or more in four prospec-

tive studies, and none of them suffered an SAH or sudden

death [38–41].

If the CSF or clinical findings are difficult to interpret in

patients, or if the index of suspicion is unusually high

(family or personal history of SAH), MRA is an appropriate

procedure for detecting a saccular aneurysm. MRA and CT

angiography (CTA) offer a noninvasive method of evaluat-

ing patients for the presence of an intracranial aneurysm.

In a recent meta-analysis, 18 adequate quality studies of

MRA for the detection of intracranial aneurysms were

identified [31]. The sensitivity varied from 69% to 100%,

and the specificity from 75% to 100%. Aneurysm size is an

important factor in aneurysm detection; studies of MRA

consistently indicate sensitivity rates of greater than 95%

for aneurysms more than 6 mm in diameter. CTA has been

studied less extensively than MRA, but published data of

CTA versus intra-arterial digital subtraction angiography

(IADSA) indicate that spiral CTA is as good as, if not more

accurate than, MRA; it has an overall detection rate of 85%

to 98% [60,61]. IADSA with selective cerebral arterial

injections and multiple projections may require a hospital

stay (it is invasive and costly), and carries a 1% risk of

transient complications and a 0.5% risk of permanent

neurologic complications. The risk may be higher in

patients with TCH, especially if they have diffuse vaso-

spasm [62,63]. However, CTA has some disadvantages

compared with MRA. It requires an injection of iodine-

based contrast, which exposes patients to radiation ( 2

mSv, which is roughly equivalent to 1 year of background

radiation), and carries a small risk of allergic reaction or

renal function deterioration. Thus if unruptured cerebral

aneurysms can occur with TCH with normal neurologic

examination results, and negative CT and LP results, it

would appear to be an uncommon occurrence and does

not justify performing IADSA on all patients.

Conclusions

Thunderclap headache is a dramatic event that seems cata-

clysmic to the patient. It may appear to be a serious organic

disease or intracranial catastrophe. It can represent a

benign self-limited primary headache for which the patho-

genesis is not clear. The true incidence of this headache as a

primary disorder will not be known until prospective

studies in urgent care or emergency department settings are

performed. These studies should be possible because it is

assumed that most patients seek medical attention because

of the severity and dramatic onset of the headache. The

prevalence of vasospasm in this disorder could be assessed

by appropriate imaging with high-resolution magnetic

resonance or CTA. Larger prospective studies are needed if

a patient’s initial CT and examination results are negative

to determine the frequency in which the various secondary

causes occur with TCHin isolation. Careful autonomic and

biochemical assessments would be useful because they

specifically examine systemic (norepinephrine) and cranial

venous levels of marker peptides (eg, neuropeptide Y) of

sympathetic activation as a possible mechanismin patients

with idiopathic TCH.

References and Recommended Reading

Papers of particular interest, published recently,

have been highlighted as:

‧

Of importance

‧‧ Of major importance

Day JW, Raskin NH: Thunderclap headache: symptom of

unruptured cerebral aneurysm. Lancet 1986, 2:1247–1248.

Dodick DW, Wijdicks EFM: Pituitary apoplexy presenting as

thunderclap headache. Neurology 1998, 50(5):1510–1511.

de Bruijn SFTM, Stam J, Kappelle LJ, for the CVST Study Group:

Thunderclap headache as the first symptom of cerebral

venous sinus thrombosis. Lancet 1996, 348:1623–1625.

Tang-Wai DF, Phan TG, Wijdicks EFM: Hypertensive

encephalopathy presenting with thunderclap headache.

Headache 2001, 41(2):198–200.

Schievink WI, Wijdicks EFM, Meyer FM, et al.: Spontaneous

intracranial hypotension mimicking aneurysmal subarach-

noid hemorrhage. Neurosurgery 2001, 48(3):513–517.

Page 6

Thunderclap Headache ‧ Dodick

231

Embil JM, Matthias K, Kinnear R: A blinding headache.

Lancet 1997, 350:182.

Edlow JA, Caplan LR: Avoiding pitfalls in the diagnosis of

subarachnoid hemorrhage. N Engl J Med 2000, 342(1):29–36.

Schievink WI, Thompson RC, Loh CT, Maya MM: Spontaneous

retroclival hematoma presenting as a thunderclap headache.

J Neurosurg 2001, 95:522–524.

Armstrong FS, Hayes GJ: Segmental cerebral arterial

constriction associated with pheochromocytoma.

J Neurosurg 1961, 18:843–846.

10.

Trommer BL, Homer D, Mikhael MA: Cerebral vasospasm

and eclampsia. Stroke 1988, 19:326–329.

11.

Margolis MT, Newton THL: Methamphetamine (“speed”)

arteritis. Neuroradiology 1971, 2:179–182.

12.

Kaye BR, Fainstat H: Cerebral vasculitis associated with

cocaine abuse. JAMA 1984, 258:2104–2106.

13.

Edvinsson L, Gulbenkian S, Barroso CP, et al.: Innervation of

the human middle meningeal artery: immunohistochemistry,

ultrastructure, and role of endothelium for vasomotility.

Peptides 1998, 19(7):1213–1225.

14.

Endo S, Suzudi J: Experimental cerebral vasospasm after

subarachnoid hemorrhage: participation of adrenergic

nerves in cerebral vessel wall. Stroke 1979, 10:703–711.

15.

Dodick DW: Recurrent short-lasting headache associated

with paroxysmal hypertension: a clonidine responsive

syndrome. Cephalalgia 2000, 20(5):509–514.

16.

Rao KCVG, Knipp HC, Wagner EJ: CT findings in cerebral

sinus and venous thrombosis. Radiology 1981, 140:391–398.

17.

Chiras J, Bousser MG, Meder JF, et al.: CT in cerebral

thrombophlebitis. Neuroradiology 1985, 27:145–154.

18.

Bousser MG, Chiras J, Sauron B, et al.: Cerebral venous

thrombosis: a review of 38 cases. Stroke 1985, 16:199–213.

19.

Barinagarrementeria F, Cantu C, Arredondo H: Aseptic cerebral

venous thrombosis: proposed prognostic scale. J Stroke

Cerebrovasc Dis 1992, 2:34–39.

20.

Bousser MG, Russell RR: Cerebral venous thrombosis.

In Major Problems in Neurology. London, UK: WB Saunders; 1999.

21.

Biousse V, D’Anglejan J, Touboui PJ, et al.: Headache in 67

patients with extracranial internal carotid artery dissection.

Cephalalgia 1991, 17:232–233.

22.

Silbert PL, Mokri B, Schievink WI: Headache and neck

pain in spontaneous internal carotid and vertebral artery

dissections. Neurology 1995, 45:1517–1522.

23.

Mokri B: Traumatic and spontaneous extracranial internal

carotid artery dissections. J Neurol 1990, 237:356–361.

24.

Bogousslavsky J, Despland PA, Regli F: Spontaneous carotid

dissection with acute stroke. Arch Neurol 1987, 44:137–140.

25.

Hauerberg J, Andersen BB, Eskesen V, et al.: Importance of the

recognition of a warning leak as a sign of a ruptured intra-

cranial aneurysm. Acta Neurol Scand 1991, 83:61–64.

26.

Edner G, Ronne-Engstrom E: Can early admission reduce

aneurysmal rebleeds? A prospective study on aneurysmal

incidence, aneurysmal rebleeds, admission and treatment

delays in a defined region. Br J Neurosurg 1991, 5:601–608.

27.

Leblanc R: The minor leak preceding subarachnoid

hemorrhage. J Neurosurg 1987, 66:35–39.

28.

Linn FHH, Rinkel GJE, Algra A, van Gjn J: The notion of

“warning leaks” in subarachnoid hemorrhage: are such

patients in fact admitted with a rebleed? J Neurol Neurosurg

Psychiatry 2000, 68:332–336.

29.

Clarke CE, Shepherd DI, Christi K, Victoratos G: Thunderclap

Headache. Lancet 1988, 2:625.

30.

Witham TF, Kaufmann AM: Unruptured cerebral aneurysm

producing a thunderclap headache. Am J Emerg Med 2000,

18(1):1–6.

31.‧‧ Wardlaw JM, White PM: The detection and management of

unruptured intracranial aneurysms. Brain 2000, 123:205–221.

This is a systematic review that provides an evidence-based approach

to the evaluation and management of patients with unruptured

intracranial aneurysms.

32.‧ Dodick DW, Brown RD, Britton JW, Huston J: Nonaneurysmal

thunderclap headache with diffuse, multifocal, segmental,

and reversible vasospasm. Cephalalgia 1999, 19:1–6.

This article illustrates the potential for thunderclap headache to

occur in the setting of diffuse vasospasm without subarachnoid

hemorrhage.

33.

Abbott RJ, van Hille P: Thunderclap headache and unruptured

cerebral aneurysm. Lancet 1986, 2:1459.

34.

Ng PK, Pulst S-M: Not so benign “thunderclap headache”

[abstract]. Neurology 1992, 42(suppl 3):260.

35.

Raps EC, Rogers JD, Galetta SL, et al.: The clinical spectrum

of unruptured intracranial aneurysms. Arch Neurol 1993,

50:265–268.

36.

Takeuchi T, Kasahara E, Iwasaki M, Kojima S: Necessity for

searching for cerebral aneurysm in thunderclap headache

patients who show no evidence of subarachnoid hemor-

rhage: investigation of eight minor leak cases on operation

[Japanese]. No Shinkei Geka 1996, 24:437–441.

37.‧‧ Wijdicks EFM, Kerkhoff H, Van Gijn J: Long term follow-up

of 71 patients with thunderclap headache mimicking

subarachnoid hemorrhage. Lancet 1988, 2:68–70.

This is a prospective longitudinal study of patients with idiopathic

thunderclap headache without subarachnoid hemorrhage that

illustrates a benign outcome.

38.

Harling DW, Peatfield RC, Van Hille PT, Abbott RJ: Thunder-

clap headache: is it migraine? Cephalalgia 1989, 9:87–90.

39.

Markus HS: A prospective follow-up of thunderclap headache

mimicking subarachnoid hemorrhage.

40.

Linn FHH, Rinkel GJE, Algra A, van Gijn J: Follow-up of

idiopathic thunderclap headache in general practice.

J Neurol 1999, 246:946–948.

41.

Landtblom AM, Boivie J, Fridriksson S, et al.: Thunderclap

headache: final results from a prospective study of consecu-

tive cases. Acta Neurol Scand 1996, 167(suppl 94):23–24.

42.

Snyder BD, McClelland RR: Isolated benign cerebral

vasculitis. Arch Neurol 1978, 35:612–614.

43.

Garnic JD, Schellinger D: Arterial spasm as a finding

intimately associated with the onset of vascular headache.

Neuroradiology 1983, 24:273–276.

44.

Miller Fisher C: Honored guest presentation: painful states:

a neurological commentary. Clin Neurosurg 1984, 31:32–53.

45.

Serdaru M, Chiras J, Cugas M, Lhermitte F: Isolated benign

cerebral vasculitis or migrainous vasospasm? J Neurol

Neurosurg Psychiatry 1984, 47:73–76.

46.

Schon F, Harrison MJH: Can migraine cause multiple

segmental cerebral artery constrictions? J Neurol Neurosurg

Psychiatry 1987, 50(4):492–494.

47.

Masuzawa T, Shinoda S, Furuse M, et al.: Cerebral angio-

graphic changes on serial examination of a patient

with migraine. Neuroradiology 1983, 24:277–281.

48.

Silbert PL, Hankey GJ, Prentice DA, Apsimon HT: Angiographi-

cally demonstrated arterial spasm in a case of benign sexual

headache and benign exertional headache. Aust NZ J Med

1989, 19:466–468.

49.

Strittmatter M, Zimmermann C, Schimrigk K, Hamann GH:

Thunderclap headache: an independent form of headache?

Weiner Klin Wochenschr 1996, 108:326–329.

50.

Slivka A, Philbrook B: Clinical and angiographic features

of thunderclap headache. Headache 1995, 35:1–6.

51.

Sturm JW, MacDonell R: Recurrent thunderclap headache

associated with reversible intracerebral vasospasm causing

stroke. Cephalalgia 2000, 20(2):132–135.

52.

Call GK, Fleming MC, Sealfon S, et al.: Reversible cerebral

segmental vasoconstriction. Stroke 1988, 19:1159–1170.

53.

Bogousslavsky J, Despland PA, Regli F, Dubuis PY: Postpartum

cerebral angiopathy: reversible vasoconstriction assessed

by transcranial doppler ultrasound. Eur Neurol 1989,

29:102–105.

54.

Henry PY, Larre P, Aupy M, et al.: Reversible cerebral arteriopa-

thy associated with the administration of ergot derivatives.

Cephalalgia 1984, 4:171–178.

Page 7

232

Uncommon Headache Syndromes

55.

Kumar R, Wijdicks EFM, Brown RD, et al.: Isolated angiitis of

the CNS presenting as subarachnoid hemorrhage. J Neurol

Neurosurg Psychiatry 1997, 62(6):649–651.

56.

International Headache Society: Classification and diagnostic

criteria for headache disorders, cranial neuralgias, and facial

pain. Cephalalgia 1988, 8(suppl 7):1–96.

57.

Linn FHH, Rinkel GJE, Algra A, vanGijn J: Headache character-

istics in subarachnoid hemorrhage and benign thunderclap

headache. J Neurol Neurosurg Psychiatry 1998, 65:791–793.

58.

Van Gijn J, van Dongen KJ: The time course of aneurysmal

hemorrhage on computed tomograms. Neuroradiology 1982,

23:153–156.

59.

Vermulen M, Hasan D, Blijenberg BG, et al.: Xanthochromia

after subarachnoid hemorrhage needs no revisitation.

J Neurol Neurosurg Psychiatry 1989, 52:826–828.

60.

Alberico RA, Patel M, Casey S, et al.: Evaluation of the circle

of Willis with three-dimensional CT angiography in patients

with suspected intracranial aneurysms. AJNR Am J Neuroradiol

1995, 16:1571–1578.

61.

Hope JK, Wilson JL, Thomson FJ: Three-dimensional CT

angiography in the detection and characterization of

intracranial berry aneurysms. AJNR Am J Neuroradiol 1996,

17:439–445.

62.

Raynor RB, Ross GR: Arteriography and vasospasm.

J Neurosurg 1969, 17:1055–1060.

63.

Warnock NG, Gandhi MR, Bergvall U, Powell T: Complications

of intra-arterial digital subtraction angiography in patients

investigated for cerebral vascular disease. Br J Radiol 1993,

66:855–885