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Overview of thunderclap headache

Overview of thunderclap headache
Literature review current through: Jan 2024.
This topic last updated: Apr 18, 2023.

INTRODUCTION — Thunderclap headache (TCH) refers to a severe headache of sudden onset. Its explosive and unexpected nature is likened to a "clap of thunder." Multiple etiologies may cause TCH (table 1). This topic will review the clinical presentation, etiologies, and diagnostic evaluation of TCH.

DEFINITION — A TCH is a very severe headache of abrupt onset that reaches its maximum intensity within one minute or less of onset [1]. The key feature that differentiates TCH from other headaches is the rapidity with which it develops; extreme severity alone is insufficient [2].

However, "sudden" may be to some extent subjective; patients or physicians are not always so precise. In a series of 2131 patients with acute-onset headache peaking within one hour, including 132 with subarachnoid hemorrhage, the interobserver agreement about sudden onset was only fair, and 6 of 132 patients with subarachnoid hemorrhage had a time to peak headache intensity of up to one hour [3]. Therefore, care must be taken in defining sudden onset. Other severe headaches may be worrisome and compel a diagnostic evaluation but would not qualify as TCH unless reaching maximum intensity quickly.

CLINICAL PRESENTATION — By definition, TCH is a very severe headache that begins abruptly and reaches maximum intensity within one minute or less of onset. Patients may present with TCH either in isolation or accompanied by additional symptoms and signs (eg, meningismus, fever, tinnitus, orthostatic worsening of headache, altered mental state, seizure, motor or sensory deficits, or cranial nerve palsies) that reflect the underlying cause.

Given the serious and potentially life-threatening nature of underlying intracranial etiologies (table 1), TCH is a medical emergency requiring immediate evaluation. (See 'Diagnostic evaluation' below.)

Although the cause of TCH cannot be determined with certainty based upon the presentation alone, some clues to the possible etiology can be gleaned from the history and from accompanying clinical features [2,4].

TCH associated with altered consciousness, seizures, or focal neurologic symptoms and signs can be due to multiple possible causes, including subarachnoid hemorrhage, other intracranial hemorrhage, reversible cerebral vasoconstriction syndrome (RCVS), posterior reversible encephalopathy syndrome (PRES), hypertensive crisis, cerebral sinus thrombosis, cervical artery dissection, or ischemic stroke.

Other clinical presentations may suggest specific causes of TCH:

Recurrent TCH over days to weeks suggest RCVS.

TCH associated with orthostatic headaches suggests spontaneous intracranial hypotension.

Postpartum setting suggests RCVS or cerebral venous thrombosis.

Recent minor trauma suggests cervical artery dissection or spontaneous intracranial hypotension.

Horner syndrome or pulsatile tinnitus suggests dissection of the ipsilateral internal carotid artery.

Papilledema and visual symptoms suggest intracranial hypertension related to cerebral venous thrombosis.

Fever or meningismus suggests meningitis.

Facial pain, ear, nose, and throat symptoms suggest complicated sinusitis.

COMMON CAUSES — TCH has multiple causes (table 1). The most frequent are subarachnoid hemorrhage and reversible cerebral vasoconstriction syndromes (RCVS).

Subarachnoid hemorrhage — The primary symptom of aneurysmal subarachnoid hemorrhage is a sudden, severe headache. Approximately one-half of patients with subarachnoid hemorrhage present with TCH [4]. While there are no symptoms or signs that can reliably differentiate primary from secondary TCH, the following features are associated with increased odds of subarachnoid hemorrhage in a patient with TCH [5-7]:

Impaired consciousness

Neck stiffness

Nausea, vomiting

Exertion or Valsalva immediately preceding onset of TCH

Elevated blood pressure

Occipital headache

History of smoking

Whether or not these features are present, all patients with TCH need to be evaluated for subarachnoid hemorrhage and other underlying causes (see 'Diagnostic evaluation' below). Misdiagnosis or delay in diagnosis of subarachnoid hemorrhage is not uncommon. Misdiagnosis occurs because of failure to recognize the spectrum of possible presentations of subarachnoid hemorrhage, lack of knowledge regarding the limitations of head computed tomography (CT), and failure to perform lumbar puncture and interpret cerebrospinal fluid tests correctly. (See "Aneurysmal subarachnoid hemorrhage: Clinical manifestations and diagnosis", section on 'Evaluation and diagnosis'.)

Initial evaluation for subarachnoid hemorrhage must include noncontrast CT of the brain (algorithm 1). Lumbar puncture is indicated when there is clinical suspicion of subarachnoid hemorrhage and the head CT is normal.

The diagnosis of subarachnoid hemorrhage as well as the utility of head CT and lumbar puncture are discussed in greater detail separately. (See "Aneurysmal subarachnoid hemorrhage: Clinical manifestations and diagnosis", section on 'Evaluation and diagnosis' and 'Diagnostic evaluation' below.)

Reversible cerebral vasoconstriction syndromes — RCVS are a group of conditions characterized by reversible segmental narrowing of the cerebral arteries. The clinical presentation of RCVS is usually dramatic with sudden, severe recurrent thunderclap headaches over the span of a few days that simulate aneurysmal subarachnoid hemorrhage; however, in patients with RCVS, thunderclap headaches often recur over a span of one to four weeks, and the duration of headache in RCVS is generally shorter (ie, several hours with resolution between attacks). Headaches of RCVS are frequently triggered by diverse conditions such as sexual activity, heat or cold exposure, various therapeutic and recreational drugs, and the postpartum state [8]. (See "Reversible cerebral vasoconstriction syndrome", section on 'Clinical presentation and course'.)

Clinical suspicion for RCVS is warranted for patients who present with the following manifestations (see "Reversible cerebral vasoconstriction syndrome", section on 'Evaluation'):

Recurrent TCH; or

Single TCH, particularly if combined with border zone infarcts or vasogenic edema on neuroimaging; or

No TCH but abnormal angiography and no brain lesions on neuroimaging; the absence of brain lesions virtually rules out primary angiitis of the central nervous system

Recurrent thunderclap headache and the presence of a vasoconstrictive trigger are key components of clinical scoring systems developed to help identify RCVS as the cause to TCH (table 2 and table 3) [9,10]. (See "Reversible cerebral vasoconstriction syndrome".)

Despite the presence of widespread cerebral vasoconstriction, the admission brain scan is normal in 30 to 70 percent of patients with RCVS. (See "Reversible cerebral vasoconstriction syndrome", section on 'Brain imaging'.)

Cerebral angiographic abnormalities may be absent during the first days after symptom onset; the changes are dynamic and progress proximally, resulting in a "sausage on a string" appearance of the circle of Willis arteries and their branches. These abnormalities begin to resolve spontaneously (without specific therapy) over a few weeks [4]. (See "Reversible cerebral vasoconstriction syndrome", section on 'Neurovascular imaging'.)

There is no proven therapy for RCVS. Supportive care is directed toward managing blood pressure, severe headaches, and other complications such as seizures. (See "Reversible cerebral vasoconstriction syndrome", section on 'Management'.)

The clinical outcome is benign in 95 percent of patients. Rare patients develop severe irreversible deficits or death from progressive strokes or cerebral edema. Recurrence of an episode of RCVS may occur in about 5 percent of patients [11]. (See "Reversible cerebral vasoconstriction syndrome", section on 'Clinical course and prognosis'.)

LESS COMMON CAUSES — Conditions that are less common causes of TCH include the following:

Cerebral infection (eg, meningitis, acute complicated sinusitis)

Cerebral venous thrombosis

Cervical artery dissection

Spontaneous intracranial hypotension

Posterior reversible encephalopathy syndrome (PRES)

Ischemic stroke

These are discussed in the sections that follow.

Cerebral venous thrombosis — Patients with cerebral venous thrombosis may present with TCH. Headache is the most common presenting symptom in cerebral venous thrombosis, occurring in approximately 90 percent of patients. In addition to headaches, patients with cerebral venous thrombosis usually present with some combination of papilledema, seizures, bilateral focal deficits, and/or altered level of consciousness. However, about 15 percent of patients may present with isolated headache. (See "Cerebral venous thrombosis: Etiology, clinical features, and diagnosis", section on 'Headache'.)

TCH accompanies cerebral venous thrombosis in 2 to 15 percent of patients [12-14]. Most commonly, however, the headaches of cerebral venous thrombosis have a gradual subacute onset; they may be localized or diffuse, persistent, exacerbated by Valsalva, and positional with worsening on recumbency.

In cases of cerebral venous thrombosis associated with TCH, the initial presentation of cerebral venous thrombosis may be clinically indistinguishable from that of subarachnoid hemorrhage. Magnetic resonance imaging (MRI) with venography should be considered when cerebral venous thrombosis is suspected, as initial testing for a suspected diagnosis of subarachnoid hemorrhage with head CT and lumbar puncture will not always detect cerebral venous thrombosis. The cerebrospinal fluid abnormalities in cerebral venous thrombosis are nonspecific, but the opening pressure is often elevated, especially when the sagittal sinus is involved. In patients with cerebral venous thrombosis who have normal neurologic exams, the head CT is normal in approximately 25 percent, whereas in patients with cerebral venous thrombosis who have focal neurologic signs, the head CT is normal in less than 10 percent [15,16]. (See "Cerebral venous thrombosis: Etiology, clinical features, and diagnosis", section on 'Diagnosis'.)

Cervical artery dissection — Headache and/or neck pain is the most frequent initial symptom of cervicocephalic dissection, found in 60 to 90 percent of cases. (See "Cerebral and cervical artery dissection: Clinical features and diagnosis", section on 'Local symptoms'.)

Although the onset of headache in cervical artery dissection is usually gradual, a minority present with TCH [17-19]. In a series of 970 consecutive patients with dissection, approximately 5 percent had a presentation consistent with a TCH (approximately 4 percent in carotid dissections and 9 percent in vertebral dissections) [19]. Diagnostic criteria from the International Classification of Headache Disorders, 3rd edition (ICHD-3) for headache secondary to cervical artery dissection stipulate that the headache must be ipsilateral to the dissected artery [1].

Cervical artery dissection can cause ischemic stroke, transient ischemic attack, or, rarely, subarachnoid hemorrhage. In addition to neck pain and headache, other local manifestations of dissection can include a Horner syndrome, pulsatile tinnitus, an audible bruit, or cranial neuropathies. Ischemia from vertebral dissections may present with vestibular symptoms and signs that may be subtle. (See "Cerebral and cervical artery dissection: Clinical features and diagnosis", section on 'Ischemic stroke or TIA' and "Cerebral and cervical artery dissection: Clinical features and diagnosis", section on 'Subarachnoid hemorrhage'.)

Common causes of dissection include various degrees of trauma or spontaneous events, with underlying predispositions in some cases. Dissection can also result from major head and neck trauma, but most dissections occur spontaneously or after minor or trivial injury. (See "Cerebral and cervical artery dissection: Clinical features and diagnosis", section on 'Etiology'.)

In the absence of ischemic stroke, routine CT scanning of the brain and lumbar puncture are typically unrevealing in patients with dissection. Additional diagnostic tests are necessary if there is clinical suspicion for cervical artery dissection; these could include ultrasound, CT angiography, magnetic resonance angiography, conventional angiography, or MRI of the neck with a fat saturation protocol. The diagnosis is confirmed by neuroimaging findings, particularly the demonstration of a long, tapered arterial stenosis, a tapered occlusion, a dissecting aneurysm (pseudoaneurysm), an intimal flap, a double lumen, or an intramural hematoma. (See "Cerebral and cervical artery dissection: Clinical features and diagnosis", section on 'Choice of neuroimaging study'.)

The management of cervicocephalic dissection is reviewed elsewhere. (See "Cerebral and cervical artery dissection: Treatment and prognosis".)

Infections — Bacterial and viral meningitis are most commonly associated with headaches of a gradual onset but may rarely present with TCH. A prospective analysis of 148 patients presenting to their primary care physician with TCH found four (3 percent) with infectious etiologies [20]. Lumbar puncture for cerebrospinal fluid examination is required for diagnosis.

Headaches related to acute sinusitis may also rarely be of the thunderclap variety. Although headaches and facial pain are frequent in patients with acute rhinosinusitis, most commonly these are acute to subacute in presentation and not consistent with TCH. However, TCH may occasionally be the presenting manifestation of sinusitis when related intracranial complications have occurred, such as with extension through the sphenoid sinus [21].

Spontaneous intracranial hypotension — Patients with spontaneous intracranial hypotension usually present with orthostatic headaches and some combination of nausea/vomiting, dizziness, auditory changes, diplopia, visual blurring, interscapular pain, and/or upper extremity radicular pain. This syndrome is also known as spontaneous low cerebrospinal fluid (CSF) pressure headache.

CSF leakage from spinal meningeal defects or dural tears may be the most common causes of this syndrome [22]. The headache is caused by displacement of pain-sensitive structures due to the low CSF pressure. (See "Spontaneous intracranial hypotension: Pathophysiology, clinical features, and diagnosis".)

A minority of patients with headache attributed to spontaneous intracranial hypotension present with TCH [23,24]. As an example, in a series of 28 patients with low CSF pressure headache due to CSF leaks from dural tears, TCH was the presenting sign in 14 percent [23].

The classic features of spontaneous intracranial hypotension are orthostatic headache, low CSF pressure, and diffuse meningeal enhancement on brain MRI. (See "Spontaneous intracranial hypotension: Pathophysiology, clinical features, and diagnosis", section on 'Introduction' and "Spontaneous intracranial hypotension: Pathophysiology, clinical features, and diagnosis", section on 'Clinical features'.)

Confirmation of the diagnosis requires evidence of low CSF pressure by MRI or lumbar puncture and/or evidence of a CSF leak on myelography or radioisotope cisternography. (See "Spontaneous intracranial hypotension: Pathophysiology, clinical features, and diagnosis", section on 'Evaluation and diagnosis' and "Spontaneous intracranial hypotension: Pathophysiology, clinical features, and diagnosis", section on 'Diagnostic criteria'.)

Typical features on brain MRI include diffuse meningeal enhancement, subdural hematomas or hygromas, sagging of the brain, engorgement of cerebral venous sinuses, and pituitary enlargement. Brain MRI remains normal in up to 20 percent of patients with spontaneous intracranial hypotension. (See "Spontaneous intracranial hypotension: Pathophysiology, clinical features, and diagnosis", section on 'Brain MRI' and "Spontaneous intracranial hypotension: Pathophysiology, clinical features, and diagnosis", section on 'Spine MRI'.)

Ischemic stroke — TCH may rarely be the presenting feature of ischemic stroke. In most cases, headaches associated with stroke are not consistent with TCH. However, several cases of ischemic stroke associated with TCH have been reported, including cases in which TCH was the primary clinical feature [5,25-27]. Neurologic exam will show a stroke-related deficit but requires careful examination for cerebellar signs. CT and lumbar puncture may be nondiagnostic in patients with TCH and recent stroke, suggesting the necessity of MRI in such cases.

Posterior reversible encephalopathy syndrome — The posterior reversible encephalopathy syndrome, also known as reversible posterior leukoencephalopathy syndrome (RPLS), usually presents with the insidious onset over hours to days with symptoms that may include headache, visual changes, altered mental status, seizures, or focal neurologic signs. PRES most often occurs in the setting of hypertensive crisis, preeclampsia, or cytotoxic immunosuppressive therapy; however, it can also occur in many other clinical settings. Hypertension is frequent but not invariable. Hypertensive crisis may precede the neurologic syndrome by 24 hours or longer. (See "Reversible posterior leukoencephalopathy syndrome".)

TCH may be a rare presenting feature of acute hypertensive crisis/PRES [28]. Evaluation with head CT and lumbar puncture may miss the diagnosis of PRES. MRI is more sensitive than head CT for revealing the white matter and cortical edema associated with PRES. These changes typically involve the parietal and occipital lobes and may also involve the basal ganglia, brainstem, and cerebellum. Since the imaging changes are secondary to vasogenic edema and not ischemia or infarction, prompt treatment of the hypertension is essential and should result in correction of the associated clinical syndrome.

UNCOMMON OR RARE CAUSES — A number of conditions are uncommon or rare causes of TCH, including:

Pituitary apoplexy

Colloid cyst of the third ventricle

Acute myocardial infarction [29]

Anaplastic oligodendroglioma [30]

Aortic arch dissection [31]

Aqueductal stenosis [32]

Giant cell arteritis [33]

Pheochromocytoma [34,35]

Pneumocephalus [31]

Retroclival hematoma [36]

Spinal epidural hematoma [37]

Varicella zoster virus vasculopathy [38]

Vogt-Koyanagi-Harada syndrome [39]

Pituitary apoplexy and colloid cyst of the third ventricle are discussed in the sections that follow.

Pituitary apoplexy — Pituitary apoplexy is caused by hemorrhage or infarction of the pituitary gland in the setting of a pituitary adenoma [40]. The clinical presentation of pituitary apoplexy ranges from relatively mild symptoms to more severe symptoms including acute headache, ophthalmoplegia, decreased visual acuity, visual loss, change in mental status, adrenal crisis, coma, or even sudden death. TCH may be a predominant feature. (See "Causes of hypopituitarism", section on 'Pituitary apoplexy'.)

Pituitary apoplexy can present with TCH in patients who have normal physical examinations, head CT scans, and cerebrospinal fluid analyses [41,42]. Pituitary tumors that are isodense to normal brain tissue may be easily overlooked on CT studies, even if hemorrhage is present. Brain MRI has a much higher sensitivity than CT for detecting the tumor and associated blood.

Colloid cyst of the third ventricle — Patients with colloid cysts of the third ventricle may also present with TCH when the cyst, which can act like a ball valve, suddenly impedes the flow of cerebrospinal fluid, causing acute obstructive hydrocephalus. Headache is reported by 68 to 100 percent of patients with third ventricular colloid cysts, making it the most commonly associated symptom.

Typically, the headache has an acute onset and resolution, with a duration of seconds to one day [43]. The pain is often severe and may be relieved by taking the supine position. Headaches may be located in the bilateral frontal, fronto-parietal, or fronto-occipital head regions [44]. Approximately one-half of patients have associated nausea and vomiting. Loss of consciousness, mental status changes, seizures, coma, and death can occur [45].

Third ventricular colloid cysts are diagnosed via CT or MRI of the brain (image 1). (See "Uncommon brain tumors", section on 'Colloid cyst'.)

UNCERTAIN ASSOCIATION — Sentinel headache (due to an unruptured intracranial aneurysm) and primary TCH are possible causes of TCH, but supporting data are weak.

Sentinel headache — Sentinel headache due to an unruptured intracranial aneurysm is a possible cause of TCH, but supporting data are weak. A sentinel headache is an episode of headache similar to that accompanying subarachnoid hemorrhage but occurring days to weeks prior to aneurysm rupture. Sentinel headaches develop over seconds and reach maximal intensity within minutes; features of subarachnoid hemorrhage, such as stiff neck, altered consciousness, and focal neurologic symptoms and signs, are absent. (See "Aneurysmal subarachnoid hemorrhage: Clinical manifestations and diagnosis", section on 'Clinical presentation'.)

Sentinel headaches may be caused by small aneurysmal leaks (ie, "warning leaks") of blood into the subarachnoid space or physical changes within the aneurysm wall (eg, acute dissection, thrombosis, or expansion) [46]. In mainly retrospective studies, 10 to 43 percent of patients with aneurysmal subarachnoid hemorrhage report a history of a sentinel or warning headache [47]. However, retrospective data may be confounded by recall bias, and a number of reports question the existence of "warning leaks" as the cause of sentinel headaches [1,20,48,49].

As an example, a meta-analysis of seven different longitudinal studies followed 813 patients who presented with TCH and had a normal neurological exam, normal cranial CT, and normal cerebrospinal fluid analysis [49]. No patient developed a subarachnoid hemorrhage or died unexpectedly in the next year, suggesting that acutely symptomatic unruptured aneurysms are rarely the cause of acute headache and that routine cerebrovascular angiography looking for unruptured aneurysms may be unnecessary.

In another meta-analysis of 729 patients with acute severe headache (defined as a "headache that developed within five minutes"), normal neurologic examination, and normal noncontrast head CT, a vascular abnormality on CT angiography was found in approximately 7 percent, including aneurysm in approximately 5 percent [50]. However, a clear relation between the headache and the CT angiography findings was evident for only 1.6 percent; most of the aneurysms were likely incidental findings.

If, in the evaluation of a patient with TCH, an aneurysm is found, a neurosurgeon or other cerebrovascular specialist should be consulted. However, some experts believe that routine cerebral angiography is not necessary in patients with a single TCH who have a normal neurologic examination, normal cranial CT, and normal cerebrospinal fluid findings [8]. The unruptured aneurysm should be managed according to an individualized analysis that incorporates factors including the risk of aneurysm rupture according to size and location, comorbid medical illness, patient age, and risks of treatment. These issues are discussed in detail separately. (See "Unruptured intracranial aneurysms".)

Following the standard evaluation for TCH will avoid most misdiagnosis (algorithm 1).

Primary TCH — There is controversy as to whether TCH can occur as a benign and potentially recurrent headache disorder in the absence of underlying organic intracranial pathology [2]. Reported cases of primary TCH may represent missed diagnosis of the underlying causes. Thus, it cannot be overstated that primary TCH must be a diagnosis made only after exclusion of all possible underlying causes [1,51].

For the diagnosis of primary TCH, the International Classification of Headache Disorders, 3rd edition (ICHD-3) requires fulfilling the following criteria [1]:

Severe head pain

Abrupt onset, reaching maximum intensity in <1 minute

Lasting for ≥5 minutes

Not better accounted for by another ICHD-3 beta diagnosis

In addition, the diagnosis requires normal brain imaging, including the brain vessels, and normal cerebrospinal fluid findings [1].

Primary TCH may occur spontaneously, while a patient is at rest, or may be precipitated by exertion or Valsalva. Exercise, weight lifting, or sexual activity is a precipitating event in up to one-third of cases.

DIAGNOSTIC EVALUATION — Given the potentially serious nature of possible underlying intracranial etiologies, TCH must be evaluated and treated as a medical emergency, beginning with an evaluation for subarachnoid hemorrhage (algorithm 1). The clinical features, imaging characteristics, cerebrospinal fluid (CSF) findings of the more common causes of TCH (table 4) should be kept in mind.

The clinician must initially presume that the patient presenting with TCH has a secondary TCH and a comprehensive search for the etiology should occur immediately.

Evaluation for subarachnoid hemorrhage — A sudden-onset headache, though not necessarily a TCH, is characteristic of subarachnoid hemorrhage. The evaluation for subarachnoid hemorrhage is reviewed here briefly and discussed in detail elsewhere. (See "Aneurysmal subarachnoid hemorrhage: Clinical manifestations and diagnosis", section on 'Evaluation and diagnosis' and "Aneurysmal subarachnoid hemorrhage: Clinical manifestations and diagnosis", section on 'Identifying the source of bleeding'.)

Initial imaging evaluation — All patients with TCH should have a head CT without contrast as soon as possible after the onset of TCH (algorithm 1). Head CT may show subarachnoid hemorrhage or other potential TCH causes such as ischemic stroke.

For patients who present with a history of two or more episodes of thunderclap headache occurring within one week, we also suggest neurovascular imaging to assess for reversible cerebral vasoconstriction syndrome (RCVS). CT- or magnetic resonance (MR)-angiography of the head and neck may identify the multifocal segmental narrowing and dilation seen with RCVS (image 2 and image 3). Neurovascular imaging may also show other potential TCH causes such as ischemic stroke or cervical artery dissection.

Lumbar puncture — If initial imaging does not reveal the cause, a lumbar puncture should be performed urgently to evaluate the CSF for evidence of subarachnoid hemorrhage or other causes of TCH. A negative head CT and lumbar puncture effectively eliminate the diagnosis of subarachnoid hemorrhage as long as both tests are performed within a few days of the event. In addition, lumbar puncture may help to diagnose other causes of TCH (eg, meningitis, spontaneous intracranial hypotension).

Lumbar puncture should include measurement of opening pressure (which may be elevated in patients with subarachnoid hemorrhage or cerebral venous thrombosis or low in patients with spontaneous intracranial hypotension), routine CSF analyses including cell counts, and visual inspection for xanthochromia.

Some experts recommend the use of CSF spectrophotometry, if available, to measure xanthochromia, especially when a traumatic tap makes CSF interpretation difficult or when the specimen has been improperly processed. The sensitivity of spectrophotometry approaches 100 percent when lumbar puncture is performed 12 hours to two weeks after subarachnoid hemorrhage onset [52]. Despite its high sensitivity, spectrophotometry has only low to moderate specificity for the diagnosis of subarachnoid hemorrhage and is not universally recommended. As a practical matter, spectrophotometry is rarely available in North American hospitals. (See "Aneurysmal subarachnoid hemorrhage: Clinical manifestations and diagnosis", section on 'Lumbar puncture'.)

Alternative approaches — Some experts believe routine lumbar puncture is not necessary if a technically adequate CT is done within six hours from headache onset is read as negative by an attending-level radiologist [8,53]. Brain CT without contrast is highly sensitive and specific for the diagnosis of subarachnoid hemorrhage when performed very early after headache onset. During the first 12 hours following onset of headache, third-generation CT scanners have a sensitivity nearing 100 percent and a specificity of 98 percent [54]. After six hours, in general practice, however, clinicians may easily overlook subtle imaging abnormalities on CT that are suggestive of subarachnoid hemorrhage. The sensitivity of head CT in subarachnoid hemorrhage declines rapidly over time, decreasing to 86 percent on day 2, 76 percent after two days, and 58 percent after five days [55]. Furthermore, head CT alone does not adequately evaluate for other causes of TCH. (See "Aneurysmal subarachnoid hemorrhage: Clinical manifestations and diagnosis", section on 'Need for lumbar puncture when early CT is negative'.)

CTA of the head may be used to identify the source of subarachnoid hemorrhage, but it is less sensitive than digital subtraction angiography for identifying small (≤2 mm) aneurysms. Some experts use CTA instead of lumbar puncture in the initial evaluation of patients with TCH and a negative head CT [56,57]. However, using CTA may both miss patients with small, ruptured aneurysms and misattribute others with TCH due to another cause and expose them to risks of surgical treatment for an unruptured cerebral aneurysm. (See "Aneurysmal subarachnoid hemorrhage: Clinical manifestations and diagnosis", section on 'CT followed by CTA'.)

Evaluation for other causes — For patients with an uncertain cause of TCH after evaluation to exclude subarachnoid hemorrhage, we suggest obtaining additional brain and neurovascular imaging, such as brain MRI with contrast, MR angiography, and MR venography (algorithm 1). If MR imaging is not an option, we suggest imaging the cerebral vasculature with CT angiography and CT venography.

However, some experts believe that patients who present with a single TCH but are otherwise asymptomatic and have a normal neurologic examination, normal cranial CT, and normal CSF findings on lumbar puncture do not require noninvasive angiography or additional work-up unless there is recurrent TCH [8].

Cerebral venous and sinus imaging should be obtained with MR venography or CT venography in patients presenting with TCH to exclude the presence of cerebral venous sinus thrombosis.

MRI is more sensitive than head CT for many of the possible underlying causes of TCH, such as cerebral venous thrombosis, cervical artery dissection, spontaneous intracranial hypotension, and reversible posterior leukoencephalopathy syndrome.

Likelihood of identifying an underlying cause — An underlying cause for TCH is identified in 27 to 71 percent of patients [5,7,20,31,58]. The wide range of these findings is attributable to the use of different criteria for defining and diagnosing TCH among the various studies and to the inclusion of patients from variable settings (eg, emergency department versus outpatient). While all of the cited studies used brain CT and lumbar puncture for diagnosis, angiography was either not used or was used sparingly. This may have led to missed diagnoses of certain conditions, such as the reversible cerebral vasoconstriction syndromes, and to underestimation of secondary TCH frequency.

Subarachnoid hemorrhage is consistently the most frequent cause for TCH in these series [5,7,20,58]. Other vascular conditions (ie, cervical artery dissection, cerebral venous thrombosis, and reversible cerebral vasoconstriction syndromes) are the next most frequent secondary causes of TCH; these disorders can present with TCH in isolation [4,59]. In a multicenter study of 644 adults presenting to an emergency department with TCH, serious pathology was identified in 10.9 percent, including 3.6 percent with subarachnoid hemorrhage, 2 percent with other intracranial hemorrhage, 1.7 percent with ischemic stroke, 1.2 percent with viral meningitis, and 1.1 percent with transient ischemic attack [60].

Supporting evidence comes from a series of 56 consecutive patients with recurrent TCH of unknown etiology, including no evidence of subarachnoid hemorrhage on both CT head and cerebrospinal fluid examination, who were referred to a headache clinic in Taiwan [61]. MR angiography performed at a mean interval of 11 days after the first headache attack showed segmental vasoconstriction involving Circle of Willis vessels in 39 percent (see 'Reversible cerebral vasoconstriction syndromes' above). This finding must be interpreted cautiously, since the utility of MR angiography for determination of segmental vasoconstriction is not established. Nevertheless, this report illustrates the potential for serious causes of TCH other than subarachnoid hemorrhage and the importance of a thorough investigation, including vascular imaging of the extracranial and intracranial circulation, in patients presenting with TCH who have no evidence of subarachnoid hemorrhage.

MANAGEMENT — The management of TCH depends on the underlying etiology. This is discussed separately for the following conditions:

Cerebral aneurysm (see "Aneurysmal subarachnoid hemorrhage: Treatment and prognosis" and "Unruptured intracranial aneurysms")

Reversible cerebral vasoconstriction syndromes (see "Reversible cerebral vasoconstriction syndrome", section on 'Management')

Cerebral venous thrombosis (see "Cerebral venous thrombosis: Treatment and prognosis")

Spontaneous intracranial hypotension (see "Spontaneous intracranial hypotension: Treatment and prognosis")

Cervical artery dissection (see "Cerebral and cervical artery dissection: Treatment and prognosis")

Ischemic stroke (see "Initial assessment and management of acute stroke" and "Initial evaluation and management of transient ischemic attack and minor ischemic stroke")

Posterior reversible encephalopathy syndrome and acute hypertensive crisis (see "Drugs used for the treatment of hypertensive emergencies" and "Moderate to severe hypertensive retinopathy and hypertensive encephalopathy in adults" and "Reversible posterior leukoencephalopathy syndrome", section on 'Management')

Pituitary apoplexy (see "Causes of hypopituitarism", section on 'Pituitary apoplexy')

SUMMARY AND RECOMMENDATIONS

Definition – Thunderclap headache (TCH) refers to a severe headache of sudden onset that reaches its maximum intensity within one minute or less of onset. (See 'Definition' above.)

Clinical features – Patients may present with TCH either in isolation or accompanied by additional symptoms and signs (eg, meningismus, fever, tinnitus, orthostatic worsening of headache, altered mental state, seizure, motor or sensory deficits) that reflect the underlying cause. (See 'Clinical presentation' above.)

Underlying etiologies – TCH may be a symptom of a wide range of etiologies (table 1 and table 4). These include (see 'Common causes' above and 'Less common causes' above and 'Uncommon or rare causes' above):

Subarachnoid hemorrhage from ruptured cerebral aneurysm

Reversible cerebral vasoconstriction syndromes (RCVS)

Cerebral venous thrombosis

Cervical artery dissection

Infections, including viral and bacterial meningitis or acute complicated sinusitis

Spontaneous intracranial hypotension

Ischemic stroke

Intracerebral and other types of intracranial hemorrhage

Posterior reversible encephalopathy syndrome

Pituitary apoplexy

Colloid cysts of the third ventricle

Diagnostic evaluation – All patients with TCH should have a head CT without contrast as soon as possible after the onset of TCH (algorithm 1). For patients who present with a history of two or more episodes of thunderclap headache occurring within one week, we also suggest neurovascular imaging to assess for reversible cerebral vasoconstriction syndrome. If initial imaging does not reveal the cause, a lumbar puncture should be performed urgently to evaluate the CSF for evidence of subarachnoid hemorrhage or other causes of TCH. (See 'Evaluation for subarachnoid hemorrhage' above.)

For patients with an uncertain cause of TCH after evaluation to exclude subarachnoid hemorrhage, we suggest obtaining additional brain and neurovascular imaging, such as brain MRI with contrast, magnetic resonance (MR) angiography, and MR venography. (See 'Evaluation for other causes' above.)

Management – The management of TCH depends on the underlying etiology. (See 'Management' above.)

ACKNOWLEDGMENT — The UpToDate editorial staff acknowledges David W Dodick, MD, who contributed to earlier versions of this topic review.

  1. Headache Classification Committee of the International Headache Society (IHS) The International Classification of Headache Disorders, 3rd edition. Cephalalgia 2018; 38:1.
  2. Schwedt TJ. Thunderclap Headache. Continuum (Minneap Minn) 2015; 21:1058.
  3. Perry JJ, Stiell IG, Sivilotti ML, et al. Clinical decision rules to rule out subarachnoid hemorrhage for acute headache. JAMA 2013; 310:1248.
  4. Ducros A, Bousser MG. Thunderclap headache. BMJ 2013; 346:e8557.
  5. Landtblom AM, Fridriksson S, Boivie J, et al. Sudden onset headache: a prospective study of features, incidence and causes. Cephalalgia 2002; 22:354.
  6. Markus HS. A prospective follow up of thunderclap headache mimicking subarachnoid haemorrhage. J Neurol Neurosurg Psychiatry 1991; 54:1117.
  7. Linn FH, Rinkel GJ, Algra A, van Gijn J. Headache characteristics in subarachnoid haemorrhage and benign thunderclap headache. J Neurol Neurosurg Psychiatry 1998; 65:791.
  8. Edlow JA. Managing Patients With Nontraumatic, Severe, Rapid-Onset Headache. Ann Emerg Med 2018; 71:400.
  9. Rocha EA, Topcuoglu MA, Silva GS, Singhal AB. RCVS2 score and diagnostic approach for reversible cerebral vasoconstriction syndrome. Neurology 2019; 92:e639.
  10. Cho S, Lee MJ, Gil YE, Chung CS. RCVS-TCH score can predict reversible cerebral vasoconstriction syndrome in patients with thunderclap headache. Sci Rep 2021; 11:7750.
  11. Boitet R, de Gaalon S, Duflos C, et al. Long-Term Outcomes After Reversible Cerebral Vasoconstriction Syndrome. Stroke 2020; 51:670.
  12. Cumurciuc R, Crassard I, Sarov M, et al. Headache as the only neurological sign of cerebral venous thrombosis: a series of 17 cases. J Neurol Neurosurg Psychiatry 2005; 76:1084.
  13. Wasay M, Kojan S, Dai AI, et al. Headache in Cerebral Venous Thrombosis: incidence, pattern and location in 200 consecutive patients. J Headache Pain 2010; 11:137.
  14. de Bruijn SF, Stam J, Kappelle LJ. Thunderclap headache as first symptom of cerebral venous sinus thrombosis. CVST Study Group. Lancet 1996; 348:1623.
  15. Rao KC, Knipp HC, Wagner EJ. Computed tomographic findings in cerebral sinus and venous thrombosis. Radiology 1981; 140:391.
  16. Chiras J, Bousser MG, Meder JF, et al. CT in cerebral thrombophlebitis. Neuroradiology 1985; 27:145.
  17. Mitsias P, Ramadan NM. Headache in ischemic cerebrovascular disease. Part I: Clinical features. Cephalalgia 1992; 12:269.
  18. Maruyama H, Nagoya H, Kato Y, et al. Spontaneous cervicocephalic arterial dissection with headache and neck pain as the only symptom. J Headache Pain 2012; 13:247.
  19. von Babo M, De Marchis GM, Sarikaya H, et al. Differences and similarities between spontaneous dissections of the internal carotid artery and the vertebral artery. Stroke 2013; 44:1537.
  20. Linn FH, Wijdicks EF, van der Graaf Y, et al. Prospective study of sentinel headache in aneurysmal subarachnoid haemorrhage. Lancet 1994; 344:590.
  21. McGeeney BE, Barest G, Grillone G. Thunderclap headache from complicated sinusitis. Headache 2006; 46:517.
  22. Rando TA, Fishman RA. Spontaneous intracranial hypotension: report of two cases and review of the literature. Neurology 1992; 42:481.
  23. Schievink WI, Wijdicks EF, Meyer FB, Sonntag VK. Spontaneous intracranial hypotension mimicking aneurysmal subarachnoid hemorrhage. Neurosurgery 2001; 48:513.
  24. Tanaka Y, Tosaka M, Fujimaki H, et al. Sex- and Age-Related Differences in the Clinical and Neuroimaging Characteristics of Patients With Spontaneous Intracranial Hypotension: A Records Review. Headache 2016; 56:1310.
  25. Schwedt TJ, Dodick DW. Thunderclap stroke: embolic cerebellar infarcts presenting as thunderclap headache. Headache 2006; 46:520.
  26. Edvardsson BA, Persson S. Cerebral infarct presenting with thunderclap headache. J Headache Pain 2009; 10:207.
  27. Gossrau G, Dannenberg C, Reichmann H, Sabatowski R. [Thunderclap headache caused by cerebellar infarction]. Schmerz 2008; 22:82.
  28. Tang-Wai DF, Phan TG, Wijdicks EF. Hypertensive encephalopathy presenting with thunderclap headache. Headache 2001; 41:198.
  29. Broner S, Lay C, Newman L, Swerdlow M. Thunderclap headache as the presenting symptom of myocardial infarction. Headache 2007; 47:724.
  30. Evans RW. Thunderclap headache associated with a nonhemorrhagic anaplastic oligodendroglioma. MedGenMed 2007; 9:26.
  31. Devenney E, Neale H, Forbes RB. A systematic review of causes of sudden and severe headache (Thunderclap Headache): should lists be evidence based? J Headache Pain 2014; 15:49.
  32. Mucchiut M, Valentinis L, Tuniz F, et al. Adult aqueductal stenosis presenting as a thunderclap headache: a case report. Cephalalgia 2007; 27:1171.
  33. García-García J, Ayo-Martín O, Segura T. A case of giant cell arteritis presenting as thunderclap headache. Headache 2013; 53:546.
  34. Heo YE, Kwon HM, Nam HW. Thunderclap headache as an initial manifestation of phaeochromocytoma. Cephalalgia 2009; 29:388.
  35. Im SH, Kim NH. Thunderclap headache after micturition in bladder pheochromocytoma. Headache 2008; 48:965.
  36. Schievink WI, Thompson RC, Loh CT, Maya MM. Spontaneous retroclival hematoma presenting as a thunderclap headache. Case report. J Neurosurg 2001; 95:522.
  37. Sathirapanya P, Setthawatcharawanich S, Limapichat K, Phabphal K. Thunderclap headache as a presentation of spontaneous spinal epidural hematoma with spontaneous recovery. J Spinal Cord Med 2013; 36:707.
  38. Barritt AW, Vundavalli S, Hughes PJ. Varicella vasculopathy presenting with thunderclap headache. JRSM Open 2017; 8:2054270416675081.
  39. Cho JH, Ahn JY, Byeon SH, Huh JS. Thunderclap headache as initial manifestation of Vogt-Koyanagi-Harada disease. Headache 2008; 48:153.
  40. Ishii M. Endocrine Emergencies With Neurologic Manifestations. Continuum (Minneap Minn) 2017; 23:778.
  41. Dodick DW, Wijdicks EF. Pituitary apoplexy presenting as a thunderclap headache. Neurology 1998; 50:1510.
  42. Embil JM, Kramer M, Kinnear S, Light RB. A blinding headache. Lancet 1997; 350:182.
  43. KELLY R. Colloid cysts of the third ventricle; analysis of twenty-nine cases. Brain 1951; 74:23.
  44. Michels LG, Rutz D. Colloid cysts of the third ventricle. A radiologic-pathologic correlation. Arch Neurol 1982; 39:640.
  45. Young WB, Silberstein SD. Paroxysmal headache caused by colloid cyst of the third ventricle: case report and review of the literature. Headache 1997; 37:15.
  46. Raps EC, Rogers JD, Galetta SL, et al. The clinical spectrum of unruptured intracranial aneurysms. Arch Neurol 1993; 50:265.
  47. Polmear A. Sentinel headaches in aneurysmal subarachnoid haemorrhage: what is the true incidence? A systematic review. Cephalalgia 2003; 23:935.
  48. Linn FH, Rinkel GJ, Algra A, van Gijn J. The notion of "warning leaks" in subarachnoid haemorrhage: are such patients in fact admitted with a rebleed? J Neurol Neurosurg Psychiatry 2000; 68:332.
  49. Savitz SI, Levitan EB, Wears R, Edlow JA. Pooled analysis of patients with thunderclap headache evaluated by CT and LP: is angiography necessary in patients with negative evaluations? J Neurol Sci 2009; 276:123.
  50. Alons IME, Goudsmit BFJ, Jellema K, et al. Yield of Computed Tomography (CT) Angiography in Patients with Acute Headache, Normal Neurological Examination, and Normal Non Contrast CT: A Meta-Analysis. J Stroke Cerebrovasc Dis 2018; 27:1077.
  51. Linn FH. Primary thunderclap headache. Handb Clin Neurol 2010; 97:473.
  52. Vermeulen M, Hasan D, Blijenberg BG, et al. Xanthochromia after subarachnoid haemorrhage needs no revisitation. J Neurol Neurosurg Psychiatry 1989; 52:826.
  53. Dubosh NM, Bellolio MF, Rabinstein AA, Edlow JA. Sensitivity of Early Brain Computed Tomography to Exclude Aneurysmal Subarachnoid Hemorrhage: A Systematic Review and Meta-Analysis. Stroke 2016; 47:750.
  54. Edlow JA, Caplan LR. Avoiding pitfalls in the diagnosis of subarachnoid hemorrhage. N Engl J Med 2000; 342:29.
  55. van Gijn J, van Dongen KJ. The time course of aneurysmal haemorrhage on computed tomograms. Neuroradiology 1982; 23:153.
  56. American College of Emergency Physicians Clinical Policies Subcommittee (Writing Committee) on Acute Headache:, Godwin SA, Cherkas DS, et al. Clinical Policy: Critical Issues in the Evaluation and Management of Adult Patients Presenting to the Emergency Department With Acute Headache. Ann Emerg Med 2019; 74:e41.
  57. Meurer WJ, Walsh B, Vilke GM, Coyne CJ. Clinical Guidelines for the Emergency Department Evaluation of Subarachnoid Hemorrhage. J Emerg Med 2016; 50:696.
  58. Harling DW, Peatfield RC, Van Hille PT, Abbott RJ. Thunderclap headache: is it migraine? Cephalalgia 1989; 9:87.
  59. Schwedt TJ. Clinical spectrum of thunderclap headache. Expert Rev Neurother 2007; 7:1135.
  60. Roberts T, Horner DE, Chu K, et al. Thunderclap headache syndrome presenting to the emergency department: an international multicentre observational cohort study. Emerg Med J 2022; 39:803.
  61. Chen SP, Fuh JL, Lirng JF, et al. Recurrent primary thunderclap headache and benign CNS angiopathy: spectra of the same disorder? Neurology 2006; 67:2164.
Topic 3346 Version 20.0

References

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