Thunderclap headache, which is also known as lone acute severe headache, is an uncommon type of headache that may be the result of an underlying vascular or nonvascular intracranial abnormality or may represent a primary headache syndrome of unknown cause. Common and uncommon causes of thunderclap headache are listed in Table 9.1 . The more benign, though no less painful, primary thunderclap headache occurs over three times more frequently than the serious secondary thunderclap headache. Because of the often-threatening causes of the less common secondary thunderclap headache (e.g., subarachnoid hemorrhage, cerebral venous thrombosis), urgent evaluation including computed tomography (CT) and/or magnetic resonance imaging (MRI) of the brain and cerebrospinal fluid analysis are indicated in all patients suspected of having thunderclap headache.
TABLE 9.1
Main and Rare Causes of Thunderclap Headache
Main Causes
Rare Causes
Vascular Disorders
Subarachnoid hemorrhage
Pituitary apoplexy, arteritis, angiitis
Intracerebral hemorrhage
Unruptured vascular malformation, aneurysm
Cerebral venous thrombosis
Arterial hypertension
Spontaneous intracranial hypotension
Cerebral segmental vasoconstriction
Cervical artery dissection
Nonvascular Disorders
Greater occipital neuralgia
Intermittent hydrocephalus by colloid cyst
Infections
Meningitis, encephalitis
Erve virus
Sinusitis
Primary Headache Disorders
Migraine
Cluster headache
Primary thunderclap headache
Tension headache, new daily persistent headache
Primary exertional headache
Primary cough headache
Modified from Linn FHH. Primary thunderclap headache. In: Aminoff MJ, ed. Handbook of Clinical Neurology. Vol. 97. New York: Elsevier; 2010:473–481.
One of the most severe headaches encountered in clinical practice, thunderclap headache is characterized by a very rapid onset to peak of less than 1 minute. The headache may last from 1 to 10 days and, because of its intensity, almost always provokes an urgent trip to the emergency department, where the headache is invariably initially misdiagnosed as the sentinel headache of acute subarachnoid hemorrhage or other potentially catastrophic headache syndromes ( Tables 9.2 and 9.3 ). This is not surprising in that primary thunderclap headache is virtually indistinguishable clinically from subarachnoid hemorrhage, one of the most neurologically devastating forms of cerebrovascular accident. Thus, because of the serious consequences of misdiagnosis, by necessity primary thunderclap headache is a diagnosis of exclusion.
TABLE 9.2
Comparison of Primary Thunderclap Headache and Subarachnoid Hemorrhage
Comparison Factors
Primary Thunderclap Headache
Subarachnoid Hemorrhage
Severe headache
Yes
Yes
Nausea and vomiting
Yes
Yes
Focal neurological signs
No
Yes
Nuchal rigidity
No
Yes
Photophobia
No
Yes
Vertigo
No
Yes
Neck and back pain
No
Yes
TABLE 9.3
Diseases That May Mimic Primary Thunderclap Headache
Hemorrhagic
Ischemic
Neoplasm
Infection
Meningitis
Encephalitis
Abscess
Parasitic
Hypertensive crisis
Loss of spinal fluid
Postdural puncture headache
Spontaneous spinal fluid leak
Collagen-vascular disease
Lupus cerebritis
Vasculitis
Polymyositis
Headache
Cluster headache
Primary exertional headache
Primary cough headache
Migraine
Ice pick headache
Primary sexual headache
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