Thunderclap headache
Encyclopedia
A thunderclap headache is a headache
Headache
A headache or cephalalgia is pain anywhere in the region of the head or neck. It can be a symptom of a number of different conditions of the head and neck. The brain tissue itself is not sensitive to pain because it lacks pain receptors. Rather, the pain is caused by disturbance of the...

 that is severe and sudden-onset. It is defined as a severe headache that takes seconds to minutes to reach maximum intensity. It can be indicative of a number of medical problems, most importantly subarachnoid hemorrhage
Subarachnoid hemorrhage
A subarachnoid hemorrhage , or subarachnoid haemorrhage in British English, is bleeding into the subarachnoid space—the area between the arachnoid membrane and the pia mater surrounding the brain...

, which can be potentially life-threatening. Usually, further investigations are performed to identify the underlying cause.

Signs and symptoms

A headache is called "thunderclap headache" if it is severe in character and reaches maximum severity within seconds to minutes of onset. In many cases, there are no other abnormalities, but the various causes of thunderclap headaches may lead to a number of neurological symptoms. The most important causes are subarachnoid hemorrhage
Subarachnoid hemorrhage
A subarachnoid hemorrhage , or subarachnoid haemorrhage in British English, is bleeding into the subarachnoid space—the area between the arachnoid membrane and the pia mater surrounding the brain...

, cerebral venous sinus thrombosis
Cerebral venous sinus thrombosis
Cerebral venous sinus thrombosis is a rare form of stroke that results from thrombosis of the dural venous sinuses, which drain blood from the brain. Symptoms may include headache, abnormal vision, any of the symptoms of stroke such as weakness of the face and limbs on one side of the body, and...

 and cervical artery dissection.

In subarachnoid hemorrhage
Subarachnoid hemorrhage
A subarachnoid hemorrhage , or subarachnoid haemorrhage in British English, is bleeding into the subarachnoid space—the area between the arachnoid membrane and the pia mater surrounding the brain...

, there may be syncope
Syncope (medicine)
Syncope , the medical term for fainting, is precisely defined as a transient loss of consciousness and postural tone characterized by rapid onset, short duration, and spontaneous recovery due to global cerebral hypoperfusion that most often results from hypotension.Many forms of syncope are...

 (transient loss of consciousness), seizures, meningism
Meningism
Meningism is the triad of nuchal rigidity , photophobia and headache. It is a sign of irritation of the meninges, such as seen in meningitis, subarachnoid hemorrhages and various other diseases...

 (neck pain and stiffness), visual symptoms, and vomiting
Vomiting
Vomiting is the forceful expulsion of the contents of one's stomach through the mouth and sometimes the nose...

. 50-70% of people with subarachnoid hemorrhage have an isolated headache without decreased level of consciousness. The headache typically persists for several days.

Cerebral venous sinus thrombosis
Cerebral venous sinus thrombosis
Cerebral venous sinus thrombosis is a rare form of stroke that results from thrombosis of the dural venous sinuses, which drain blood from the brain. Symptoms may include headache, abnormal vision, any of the symptoms of stroke such as weakness of the face and limbs on one side of the body, and...

, thrombosis of the veins of the brain, usually causes a headache that reflects raised intracranial pressure
Intracranial pressure
Intracranial pressure is the pressure inside the skull and thus in the brain tissue and cerebrospinal fluid . The body has various mechanisms by which it keeps the ICP stable, with CSF pressures varying by about 1 mmHg in normal adults through shifts in production and absorption of CSF...

 and is therefore made worse by anything that makes the pressure rise further, such as cough
Cough
A cough is a sudden and often repetitively occurring reflex which helps to clear the large breathing passages from secretions, irritants, foreign particles and microbes...

ing. In 2-10% of cases, the headache is of thunderclap character. In most cases there are other neurological abnormalities, such as seizures and weakness of part of the body, but in 15-30% the headache is the only abnormality.

Carotid artery dissection
Carotid artery dissection
Carotid artery dissection is a separation of the layers of the artery wall supplying oxygen-bearing blood to the head and brain, and is the most common cause of stroke in young adults...

 and vertebral artery dissection
Vertebral artery dissection
Vertebral artery dissection is a dissection of the inner lining of the vertebral artery, which is located in the neck and supplies blood to the brain. After the tear, blood enters the arterial wall and forms a blood clot, thickening the artery wall and often impeding blood flow...

 (together cervical artery dissection), in which a tear forms inside the wall of the blood vessels that supply the brain, often causes pain on the affected side of the head or neck. The pain usually precedes other problems that are caused by impaired blood flow through the artery into the brain; these may include visual symptoms, weakness of part of the body, and other abnormalities depending on the vessel affected.

Causes

Thunderclap headaches can be caused by a number of different primary conditions including:
  • Subarachnoid hemorrhage
    Subarachnoid hemorrhage
    A subarachnoid hemorrhage , or subarachnoid haemorrhage in British English, is bleeding into the subarachnoid space—the area between the arachnoid membrane and the pia mater surrounding the brain...

     (10-25% of all cases of thunderclap headache)
  • Cerebral venous sinus thrombosis
    Cerebral venous sinus thrombosis
    Cerebral venous sinus thrombosis is a rare form of stroke that results from thrombosis of the dural venous sinuses, which drain blood from the brain. Symptoms may include headache, abnormal vision, any of the symptoms of stroke such as weakness of the face and limbs on one side of the body, and...

  • Cervical artery dissection
  • Hypertensive emergency
    Hypertensive emergency
    Malignant hypertension or hypertensive emergency is severe hypertension with acute impairment of an organ system and the possibility of irreversible organ-damage...

     (severely raised blood pressure)
  • Spontaneous intracranial hypotension (low pressure on the cerebrospinal fluid
    Cerebrospinal fluid
    Cerebrospinal fluid , Liquor cerebrospinalis, is a clear, colorless, bodily fluid, that occupies the subarachnoid space and the ventricular system around and inside the brain and spinal cord...

     without an explanation)
  • Stroke
    Stroke
    A stroke, previously known medically as a cerebrovascular accident , is the rapidly developing loss of brain function due to disturbance in the blood supply to the brain. This can be due to ischemia caused by blockage , or a hemorrhage...

     (headache occurs in about 25% of strokes but usually not thunderclap character)
  • Retroclival hematoma (hematoma behind the clivus
    Clivus (anatomy)
    The clivus is a part of the cranium, a shallow depression behind the dorsum sellæ that slopes obliquely backward. It forms a gradual sloping process at the anterior most portion of the basilar occipital bone at its junction with the sphenoid bone. On axial planes, it sits just posterior to the...

     in the skull, usually due to physical trauma but sometimes spontaneous)
  • Pituitary apoplexy
    Pituitary apoplexy
    Pituitary apoplexy or pituitary tumor apoplexy is bleeding into or impaired blood supply of the pituitary gland at the base of the brain. This usually occurs in the presence of a tumor of the pituitary, although in 80% of cases this has not been diagnosed previously...

     (infaction or hemorrhage of the pituitary gland
    Pituitary gland
    In vertebrate anatomy the pituitary gland, or hypophysis, is an endocrine gland about the size of a pea and weighing 0.5 g , in humans. It is a protrusion off the bottom of the hypothalamus at the base of the brain, and rests in a small, bony cavity covered by a dural fold...

    )
  • Colloid cyst
    Colloid cyst
    A colloid cyst is a cyst containing gelatinous material in the brain. It is almost always found just posterior to the foramen of Monro in the anterior aspect of the third ventricle, originating from the roof of the ventricle. Because of its location, it can cause obstructive hydrocephalus and...

     of the third ventricle
    Third ventricle
    The third ventricle is one of four connected fluid-filled cavities comprising the ventricular system within the human brain. It is a median cleft between the two thalami, and is filled with cerebrospinal fluid ....

  • Meningitis
    Meningitis
    Meningitis is inflammation of the protective membranes covering the brain and spinal cord, known collectively as the meninges. The inflammation may be caused by infection with viruses, bacteria, or other microorganisms, and less commonly by certain drugs...

     (rarely features thunderclap headache)
  • Reversible cerebral vasoconstriction syndrome (previously Call-Fleming syndrome, several subtypes)
  • Primary cough headache, primary exertional headache and primary sexual headache
  • Primary thunderclap headache

Diagnosis

The most important initial investigation is computed tomography
Computed tomography
X-ray computed tomography or Computer tomography , is a medical imaging method employing tomography created by computer processing...

 of the brain, which is very sensitive for subarachnoid hemorrhage. If this is normal, a lumbar puncture
Lumbar puncture
A lumbar puncture is a diagnostic and at times therapeutic procedure that is performed in order to collect a sample of cerebrospinal fluid for biochemical, microbiological, and cytological analysis, or very rarely as a treatment to relieve increased intracranial pressure.-Indications:The...

 is performed, as a small proportion of SAH is missed on CT and can still be detected as xanthochromia.

If both investigations are normal, the specific description of the headache and the presence of other abnormalities may prompt further tests, usually involving magnetic resonance imaging
Magnetic resonance imaging
Magnetic resonance imaging , nuclear magnetic resonance imaging , or magnetic resonance tomography is a medical imaging technique used in radiology to visualize detailed internal structures...

 (MRI). Magnetic resonance angiography
Magnetic Resonance Angiography
Magnetic resonance angiography is a group of techniques based on Magnetic Resonance Imaging to image blood vessels. Magnetic resonance angiography is used to generate images of the arteries in order to evaluate them for stenosis , occlusion or aneurysms...

 (MRA) may be useful in identifying problems with the arteries (such as dissection), and magnetic resonance venography (MRV) identifies venous thrombosis. It is not usually necessary to proceed to cerebral angiography
Cerebral angiography
Cerebral angiography is a form of angiography which provides images of blood vessels in and around the brain, thereby allowing detection of abnormalities such as arteriovenous malformations and aneurysms....

, a more precise but invasive investigation of the brain's blood vessels, if MRA and MRV are normal.

History

The importance of severe headaches in the diagnosis of subarachnoid hemorrhage has been known since the 1920s, when London neurologist Charles Symonds
Charles Symonds
Sir Charles Putnam Symonds KBE CB was an English neurologist.His initial medical training was at Guy's Hospital, followed by specialised training at the National Hospital for Neurology and Neurosurgery...

 described the clinical syndrome. The term "thunderclap headache" was introduced in 1986 in a report by John Day and Neil Raskin, neurologists at the University of California, San Francisco
University of California, San Francisco
The University of California, San Francisco is one of the world's leading centers of health sciences research, patient care, and education. UCSF's medical, pharmacy, dentistry, nursing, and graduate schools are among the top health science professional schools in the world...

, in a report of a 42-year old woman who had experienced several sudden headaches and was found to have an aneurysm that had not ruptured.
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