Approach to the patient with thunderclap headache
- Todd J Schwedt, MD, MSCI
Todd J Schwedt, MD, MSCI
- Professor of Neurology
- Mayo Clinic
- David W Dodick, MD
David W Dodick, MD
- Professor of Neurology
- Mayo Clinic College of Medicine
Thunderclap headache (TCH) refers to a severe headache of sudden onset. Its explosive and unexpected nature is likened to a "clap of thunder." Although TCH initially referred to pain associated with an unruptured intracranial aneurysm , multiple etiologies are now recognized  (table 1).
This topic will review the clinical presentation, etiologies, and diagnostic evaluation of TCH.
A thunderclap headache (TCH) is a very severe headache of abrupt onset that reaches its maximum intensity within one minute or less of onset. The key feature that differentiates TCH from other headaches is the rapidity with which it develops; extreme severity alone is insufficient . Other severe headaches may be worrisome and compel a diagnostic evaluation, but would not qualify as TCH unless reaching maximum intensity quickly.
The underlying pathophysiology of primary thunderclap headache (TCH) and reversible cerebral vasoconstriction syndromes (RCVS) is unclear. Excessive sympathetic activity or an abnormal vascular response to circulating catecholamines may be involved. This would explain the occurrence of TCH during physical activity, in patients with pheochromocytoma, acute hypertensive crises, and in patients who take sympathomimetic drugs or tyramine containing foods concurrently with monoamine oxidase (MAO) inhibitors. In further support of this hypothesis, reversible cerebral vasoconstriction like that which can occur with some cases of TCH has been documented in pheochromocytoma, eclampsia, sympathomimetic drug intoxication, and autonomic dysreflexia [4-8].
Although vasoconstriction can be initiated by mechanical and biochemical stimuli, a neurogenic mechanism is implied when vasoconstriction is associated with the abrupt onset of headache. Sympathetic afferents that innervate the intracranial vasculature contain neuropeptide Y and noradrenaline, both vasoconstrictors [9,10]. Vascular caliber may be a reflection of sympathetic tone and sympathetic receptor sensitivity, and TCH may be a result of spontaneous and abnormal central sympathetic response. This theory is supported by models of vasospasm in subarachnoid hemorrhage as well as the clinical finding of multifocal vasospasm in patients with pheochromocytoma and sympathomimetic drug intoxication [4,6,7,11,12].
- Day JW, Raskin NH. Thunderclap headache: symptom of unruptured cerebral aneurysm. Lancet 1986; 2:1247.
- Ducros A, Bousser MG. Thunderclap headache. BMJ 2013; 346:e8557.
- Schwedt TJ. Thunderclap Headache. Continuum (Minneap Minn) 2015; 21:1058.
- ARMSTRONG FS, HAYES GJ. Segmental cerebral arterial constriction associated with pheochromocytoma: report of a case with arteriograms. J Neurosurg 1961; 18:843.
- Trommer BL, Homer D, Mikhael MA. Cerebral vasospasm and eclampsia. Stroke 1988; 19:326.
- Margolis MT, Newton TH. Methamphetamine ("speed") arteritis. Neuroradiology 1971; 2:179.
- Kaye BR, Fainstat M. Cerebral vasculitis associated with cocaine abuse. JAMA 1987; 258:2104.
- Furlan JC. Headache attributed to autonomic dysreflexia: an underrecognized clinical entity. Neurology 2011; 77:792.
- 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; 19:1213.
- Edvinsson L, Owman C. Cerebrovascular nerves and vasomotor receptors. In: Cerebral arterial spasm, Wilkins RH (Ed), Williams and Wilkins, Baltimore 1980. p.30.
- Endo S, Suzuki J. Experimental cerebral vasospasm after subarachnoid hemorrhage. Participation of adrenergic nerves in cerebral vessel wall. Stroke 1979; 10:703.
- Edvinsson L, Egund N, Owman C, et al. Reduced noradrenaline uptake and retention in cerebrovascular nerves associated with angiographically visible vasoconstriction following experimental subarachnoid hemorrhage in rabbits. Brain Res Bull 1982; 9:799.
- Broner S, Lay C, Newman L, Swerdlow M. Thunderclap headache as the presenting symptom of myocardial infarction. Headache 2007; 47:724.
- Evans RW. Thunderclap headache associated with a nonhemorrhagic anaplastic oligodendroglioma. MedGenMed 2007; 9:26.
- 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.
- Mucchiut M, Valentinis L, Tuniz F, et al. Adult aqueductal stenosis presenting as a thunderclap headache: a case report. Cephalalgia 2007; 27:1171.
- García-García J, Ayo-Martín O, Segura T. A case of giant cell arteritis presenting as thunderclap headache. Headache 2013; 53:546.
- Heo YE, Kwon HM, Nam HW. Thunderclap headache as an initial manifestation of phaeochromocytoma. Cephalalgia 2009; 29:388.
- Im SH, Kim NH. Thunderclap headache after micturition in bladder pheochromocytoma. Headache 2008; 48:965.
- Schievink WI, Thompson RC, Loh CT, Maya MM. Spontaneous retroclival hematoma presenting as a thunderclap headache. Case report. J Neurosurg 2001; 95:522.
- 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.
- Barritt AW, Vundavalli S, Hughes PJ. Varicella vasculopathy presenting with thunderclap headache. JRSM Open 2017; 8:2054270416675081.
- Cho JH, Ahn JY, Byeon SH, Huh JS. Thunderclap headache as initial manifestation of Vogt-Koyanagi-Harada disease. Headache 2008; 48:153.
- Landtblom AM, Fridriksson S, Boivie J, et al. Sudden onset headache: a prospective study of features, incidence and causes. Cephalalgia 2002; 22:354.
- Markus HS. A prospective follow up of thunderclap headache mimicking subarachnoid haemorrhage. J Neurol Neurosurg Psychiatry 1991; 54:1117.
- 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.
- Polmear A. Sentinel headaches in aneurysmal subarachnoid haemorrhage: what is the true incidence? A systematic review. Cephalalgia 2003; 23:935.
- 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.
- 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.
- Rao KC, Knipp HC, Wagner EJ. Computed tomographic findings in cerebral sinus and venous thrombosis. Radiology 1981; 140:391.
- Chiras J, Bousser MG, Meder JF, et al. CT in cerebral thrombophlebitis. Neuroradiology 1985; 27:145.
- Mitsias P, Ramadan NM. Headache in ischemic cerebrovascular disease. Part I: Clinical features. Cephalalgia 1992; 12:269.
- 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.
- Headache Classification Committee of the International Headache Society (IHS). The International Classification of Headache Disorders, 3rd edition (beta version). Cephalalgia 2013; 33:629.
- Linn FH, Wijdicks EF, van der Graaf Y, et al. Prospective study of sentinel headache in aneurysmal subarachnoid haemorrhage. Lancet 1994; 344:590.
- McGeeney BE, Barest G, Grillone G. Thunderclap headache from complicated sinusitis. Headache 2006; 46:517.
- Rando TA, Fishman RA. Spontaneous intracranial hypotension: report of two cases and review of the literature. Neurology 1992; 42:481.
- Schievink WI, Wijdicks EF, Meyer FB, Sonntag VK. Spontaneous intracranial hypotension mimicking aneurysmal subarachnoid hemorrhage. Neurosurgery 2001; 48:513.
- 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.
- Schwedt TJ, Dodick DW. Thunderclap stroke: embolic cerebellar infarcts presenting as thunderclap headache. Headache 2006; 46:520.
- Edvardsson BA, Persson S. Cerebral infarct presenting with thunderclap headache. J Headache Pain 2009; 10:207.
- Gossrau G, Dannenberg C, Reichmann H, Sabatowski R. [Thunderclap headache caused by cerebellar infarction]. Schmerz 2008; 22:82.
- Tang-Wai DF, Phan TG, Wijdicks EF. Hypertensive encephalopathy presenting with thunderclap headache. Headache 2001; 41:198.
- Fugate JE, Rabinstein AA. Posterior reversible encephalopathy syndrome: clinical and radiological manifestations, pathophysiology, and outstanding questions. Lancet Neurol 2015; 14:914.
- Dodick DW, Eross EJ, Drazkowski JF, Ingall TJ. Thunderclap headache associated with reversible vasospasm and posterior leukoencephalopathy syndrome. Cephalalgia 2003; 23:994.
- Singhal AB, Bernstein RA. Postpartum angiopathy and other cerebral vasoconstriction syndromes. Neurocrit Care 2005; 3:91.
- Zampaglione B, Pascale C, Marchisio M, Cavallo-Perin P. Hypertensive urgencies and emergencies. Prevalence and clinical presentation. Hypertension 1996; 27:144.
- Ishii M. Endocrine Emergencies With Neurologic Manifestations. Continuum (Minneap Minn) 2017; 23:778.
- Dodick DW, Wijdicks EF. Pituitary apoplexy presenting as a thunderclap headache. Neurology 1998; 50:1510.
- Embil JM, Kramer M, Kinnear S, Light RB. A blinding headache. Lancet 1997; 350:182.
- KELLY R. Colloid cysts of the third ventricle; analysis of twenty-nine cases. Brain 1951; 74:23.
- Michels LG, Rutz D. Colloid cysts of the third ventricle. A radiologic-pathologic correlation. Arch Neurol 1982; 39:640.
- 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.
- Linn FH. Primary thunderclap headache. Handb Clin Neurol 2010; 97:473.
- Edlow JA, Caplan LR. Avoiding pitfalls in the diagnosis of subarachnoid hemorrhage. N Engl J Med 2000; 342:29.
- van Gijn J, van Dongen KJ. The time course of aneurysmal haemorrhage on computed tomograms. Neuroradiology 1982; 23:153.
- Vermeulen M, Hasan D, Blijenberg BG, et al. Xanthochromia after subarachnoid haemorrhage needs no revisitation. J Neurol Neurosurg Psychiatry 1989; 52:826.
- Harling DW, Peatfield RC, Van Hille PT, Abbott RJ. Thunderclap headache: is it migraine? Cephalalgia 1989; 9:87.
- Schwedt TJ. Clinical spectrum of thunderclap headache. Expert Rev Neurother 2007; 7:1135.
- 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.
- CLINICAL PRESENTATION
- Subarachnoid hemorrhage
- - Sentinel headache
- Reversible cerebral vasoconstriction syndromes
- Cerebral venous thrombosis
- Cervical artery dissection
- Spontaneous intracranial hypotension
- Ischemic stroke
- Acute hypertensive crisis
- Pituitary apoplexy
- Colloid cyst of the third ventricle
- Primary thunderclap headache
- DIAGNOSTIC EVALUATION
- Evaluation for subarachnoid hemorrhage
- Evaluation for other causes
- Likelihood of identifying an underlying cause
- SUMMARY AND RECOMMENDATIONS