Thunderclap Headache
- Article Author:
- Sandeep Sekhon
- Article Author:
- Roopa Sharma
- Article Editor:
- Marco Cascella
- Updated:
- 7/21/2020 6:08:04 PM
- For CME on this topic:
- Thunderclap Headache CME
- PubMed Link:
- Thunderclap Headache
Introduction
The word’ thunderclap headache’ was founded by Day and Raskin in 1986. They discovered it in a patient suffering from an unruptured cerebral aneurysm who presented with multiple episodes of an abrupt onset of a severe form of headache, later labeled as a thunderclap headache.[1] A thunderclap headache is defined as a severe form of headache that occurs abruptly, like a’ clap of thunder’ as suggested by the name, peaks within 30 seconds to 1 minute of the onset, and usually fades in next few hours. It is often defined by the sufferers as the worst headache of their life.[2]
Usually, thunderclap headache is the expression of intracranial vascular disorders (secondary thunderclap headache). On the other hand, it is rarely classifiable among primary headaches. Data that thunderclap headache occurs as a primary condition, indeed, is limited. Hence, primary thunderclap headache is diagnosed when all organic reasons have been ruled out.[3]
A primary headache is compatible with no apparent associating etiology on the assessment of the case nor on imaging studies. According to the third edition of the International Classification of Headache Disorders (ICHD-3), the primary headache disorders are categorized as migraine, tension-type headache (TTH), trigeminal autonomic cephalalgias (TACs), and other primary headache disorders. Thunderclap headache has been documented under the’ other primary headache disorders.’ It is described as an abrupt onset severe headache which reaches a peak within a minute, lasts for at least 5 minutes, and is not better explained by other headache diagnostic criteria listed as per ICHD-3.[4] A primary thunderclap headache can be precipitated by cough, workout, and sexual activity and hence be categorized as primary cough headache, primary exercise headache, and primary headache attributed to sexual activity.[5]
A thunderclap headache is continually attributed to significant vascular intracranial disorders, especially subarachnoid hemorrhage (SAH). Therefore, it should be considered a medical emergency. As a consequence, in a patient who presents in the ED with an abrupt onset of sudden severe headache, it is almost always necessary to rule out secondary causes, including significant underlying intracranial pathology like SAH, meningitis, ischemic stroke, intracerebral hemorrhage.[6] In addition to the secondary forms, primary forms of thunderclap headache can be identified, although much rarer than the secondary ones. Moreover, some authors believe that primary thunderclap headache cases may represent missed diagnoses of an underlying cause.[7][3]
This chapter is aimed at presenting an overview of the features and management of the thunderclap headache. The role of the multi-professional team for improving care and patients’ outcomes is also discussed.
Etiology
The diagnosis of primary thunderclap headache is made when no underlying etiology is found.[8] Numerous etiologies for secondary thunderclap headaches have been recognized.
- SAH. It is found to be the most common cause.
- Reversible cerebral vasoconstriction syndrome (RCVS). After SAH, it is the most frequent cause.[1] The disease is more common in women, typically in their 40s-50s with a history of migraine. While RCVS cases usually result in a favorable clinical course, this clinical condition can lead to important complications such as intracranial hemorrhage, seizures, or cerebral infarcts.[9]
- Infectious-associated vasoconstriction disorders. Cerebral vasoconstriction syndromes like central nervous system infections, comprising sinusitis, meningitis, and viral infection.[10]
- Non-vasoconstrictive disorders. A vast extent of non-vasoconstrictive disorders could also incite abrupt and intense headaches, encompassing all kinds of intracranial hemorrhage in addition to SAH, arterial dissection, venous sinus thrombosis, expanding berry aneurysm.[11]
- Ischemic stroke
- Hypertensive crisis
- Vasculitis
- Spontaneous intracranial hypotension
- Medication-induced headache. Numerous serotonergic medications (like lysergic acid diethylamine, selective serotonin reuptake inhibitors, ergot derivatives, triptans) can cause abrupt onset severe headache. Sympathomimetics like cocaine, amphetamine, nicotine patches can also precipitate a thunderclap headache.
- Catecholamine secreting tumors. Pheochromocytoma and carcinoid tumors have been found to be associated with a thunderclap headache secondary to vasoconstriction.
- Pregnancy and delivery (hypertensive crisis)[12]
- Others. Binge drinking of alcohol and smoking cannabis can precipitate vasoconstriction leading to a thunderclap headache.
Epidemiology
A thunderclap headache is a rare kind of headache. The incidence in the age group of 18 years and above is 38-43 cases per 100,000 adults per year.[13] The lifetime prevalence of a thunderclap headache is found to be 0.3 percent in the community and 2-5 percent in cases who visit the emergency department.[14]
Pathophysiology
Thunderclap headache has multifactorial pathophysiology and is poorly understood.[15] In primary thunderclap headache, the sympathetic nervous system possibly plays a crucial function as it regulates the vascular tone. Pain occurs due to acute vasoconstriction and changes in the vascular tone as an outcome of exacerbated sympathetic tone. Vasospasm indicates a localized smooth muscle constriction, which leads to the narrowing of the blood vessel leading to abrupt onset severe headache. It can be induced by chemical or neurogenic stimulants.[2] Damage to the underlying vessel wall and release of inflammatory cytokines is another possible mechanism of acute-onset severe headache.[16]
History and Physical
Headache is a common sign and is documented by nearly 1% to 3% of cases presenting to the casualty. Several anomalies present with headache as the primary symptom, and it is significant to collect a detailed history including the onset, duration, progression, and severity of the headache for an accurate diagnosis and management of the patient. In the case of thunderclap headache, the patient would describe the pain as abrupt onset, severe headache, which would progress within minutes from the onset and lasts for five or more minutes or a few hours. Usually, it can be accompanied by nausea or vomiting.
Often times, the patients would describe it as the ’worst headache of their life.’ The patient should be enquired about risk factors like high blood pressure, low immunity, or cancer. Further history about pregnancy, hypercoagulable states, medications, or prior similar episodes should be documented.[14] Special emphasis should be given to certain warning signs in patients in a secondary headache, which helps narrow down the diagnosis. These are:
- New or changed pattern of headache in a person above 50 years of age or immunosuppressed
- Abrupt onset and severe intensity headache (it reaches the maximum intensity in < 1 minute)
- Diurnal variation in the pain with more pain early in the morning after waking up
- Headache secondary to exertion or with changes in the posture
- Headache associated with stiffness in the neck muscles or jaw claudication
- Associated focal/nonfocal neurological signs or abnormal neurological examination
In addition to a detailed history (e.g., recurrent thunderclap headaches can be suggestive for RCVS), a thorough examination including a detailed neurological assessment is required to formulate the correct diagnosis and treatment. A thunderclap headache is a medical emergency, and its presence should prompt early investigation. The presence of focal/nonfocal neurological signs and symptoms helps physicians to determine whether the imaging is required and which modality to select to confirm the diagnosis.
These neurological signs can be:
- Speech deficit
- Abnormal pupillary signs
- Cranial nerve abnormalities
- Ataxia[17]
- Motor deficits
- Photophobia (e.g., in RCVS)
- Altered mental state
- Seizures
Evaluation
Diagnostic assessment of the thunderclap headache should be done on an urgent basis to rule out the possibility of harmful etiologies, for example, SAH. There is, indeed, an overlap in the clinical presentation between thunderclap headache and other conditions such as SAH and migraine headaches. The evaluation requires:
- Computed tomography (CT) scan is a highly sensitive test for evaluating intracranial hemorrhage, edema, mass, or signs of herniation.[18]
- If a CT scan is negative, a lumbar puncture is recommended to rule out meningitis and SAH.[19]
- If these tests are negative, the next best step is to perform a magnetic resonance angiography (MRA) and venography (MRV) of the head and neck blood vessels.
- CT angiogram can be helpful for the diagnosis of vasospasm.[20][21]
The diagnosis of a primary thunderclap headache is made when a detailed analysis could not find out a particular reason for a thunderclap headache.[22]
Treatment / Management
Several strategies have been proposed for the treatment of the thunderclap headache.
- Calcium channel blockers: Nimodipine is most commonly used for the treatment of a thunderclap headache. It crosses the blood-brain barrier easily and inhibits activation of glial cells and tumor necrosis factor (TNF) alpha production. TNF alpha is a cytokine involved in the pathogenesis of thunderclap headache. It produces CNS inflammation and vasoconstriction thus precipitates a thunderclap headache onset.[23] Nimodipine also has a blocking effect on certain neurotransmitters like serotonin, histamine, etc, thus it can prevent the vasospasm. It has also been found that treatment with nimodipine decreases the recurrence rate of thunderclap headaches to many folds.[24] Commonly found side effects of nimodipine are dizziness and flushing.
- Analgesics and anti-inflammatory drugs: Acetaminophen and NSAIDs (nonsteroidal anti-inflammatory drugs) have a remarkable effect on the treatment of primary thunderclap headache. Indomethacin has been found to be most effective. Etoricoxib is effective in prophylaxis. Common side effects of Acetaminophen are nausea, stomach pain, or loss of appetite. Patients on NSAIDs usually complain of feeling bloated, nausea, vomiting, diarrhea, or constipation.[13]
- Beta-blocker: Propranolol is effective in preventing a thunderclap headache and has been widely used as a prophylactic agent. Commonly seen adverse effects are fatigue and dizziness. It should be used with caution in patients suffering from respiratory system disorders such as chronic obstructive pulmonary disease).[4]
- Topiramate and lithium: According to studies, in lower dosages, these medications help prevent an episode of thunderclap headache and can be safely used for prophylaxis.[13]
- Triptans (sumatriptan, rizatriptan): Due to the risk of precipitating vasospasm, these medications should be avoided in patients with a thunderclap headache.[25]
Differential Diagnosis
Several clinical conditions can be associated with the occurrence of a thunderclap headache episode:
- Subarachnoid hemorrhage (SAH): Patients with subarachnoid hemorrhage present with an abrupt onset severe headache and often describe the headache as the' worst headache' of their lives. Other symptoms are loss of consciousness and the presence of focal neurological signs along with headache. SAH can be precipitated by exertion.[26]
- Reversible cerebral vasoconstriction syndrome (RCVS): Most commonly seen in patients who are postpartum, had a head injury, or in post head surgery cases. It is presented spontaneously as a severe headache.[27]
- Cerebral venous sinus thrombosis (CVST): These patients present with sudden onset severe headache, which worsens in the recumbent position.[28]
- Cervical artery dissection: It is seen in patients with head and neck surgery. Associated symptoms are horner's syndrome, amaurosis fugax.[29]
- Acute hypertensive crisis: It presents with headache simulating a thunderclap accompanied by seizures and altered mental state.[30]
- Sentinel headache: It is precipitated by physical exertion, sexual intercourse.
- Pituitary apoplexy: This condition presents as a severe headache along with visual disturbances.
- Spontaneous intracranial hypotension (SIH): It's a rare entity that presents with headache, cranial nerve III abnormality, and nuchal rigidity.[3]
- Colloid cysts: Colloid cyst of the third ventricle presents with a severe headache, which gets better while in lying down position. Associated symptoms are seizures, loss of consciousness, stupor, and coma.
- Benign hot bath-related headache: It is precipitated by a hot bath or shower and presents with an abrupt onset severe headache.[31]
- Primary cough, sexual and exertional headaches: These are diagnosed when all other possible reasons for the headache have been ruled out. The headache is precipitated by a cough or physical exertion with a normal neurological examination.[11]
Prognosis
A thunderclap headache, if not detected early, can lead to severe complications secondary to the cause. The most common cause of thunderclap headache presenting to the emergency department is a SAH. If presented with loss of consciousness along with abrupt onset severe headache, the prognosis is poor, as it has a 2% to 4% increased risk of death. There are many folds increased chances of stroke in a patient suffering from RCVS, another common cause of secondary thunderclap headache.[32] Poor prognostic factors are:
- New or changed pattern of headache in a person above 50 years of age or immunosuppressed
- Diurnal variation in the pain with more pain early in the morning after waking up
- Headache associated with stiffness in the neck muscles or jaw claudication
- Associated focal/nonfocal neurological signs or abnormal neurological examination
On the other hand, the primary thunderclap headache has a good prognosis. Although the patient can experience the recurrence of headache in a week, it usually subsides in a few months.
Complications
A thunderclap headache is an emergency. It is important to be detected and treated early. If not treated, the headache can cause lethal complications. Several cases have been reported where patients experience dreadful complications like anterior cerebral artery dissection and hemorrhage.[33] In addition to hemorrhage, it can lead to ischemia (6 to 7 percent of posterior reversible encephalopathy syndromes). In some instances, it can develop into migraine headaches.[34] Chronic episodes of thunderclap headache lead to vasospasm and can result in permanent neurological deficiencies.[35] Secondary thunderclap headache, if left untreated, can lead to stroke, stupor, and death.[36]
Deterrence and Patient Education
Headache is a common sign seen in the emergency department. Clinically, the headaches can be varied and atypical. Educating the patients regarding the typical symptom profile of a thunderclap headache is mandatory as it needs immediate management to prevent dreadful complications. Patients should be educated about the early warning signs of the headache, for example, a recent change in the pattern of the headache, diurnal variation, new headache in old age, associated nuchal rigidity, or jaw claudication.
Enhancing Healthcare Team Outcomes
A thunderclap headache episode frequently poses a diagnostic dilemma. The cause of a secondary thunderclap may be due to a number of reasons, including nonvascular and vascular etiologies. While the physical exam may reveal that the patient has a secondary headache, the cause is difficult to know without proper imaging studies. An interprofessional collaborative approach including the physician, neurologist, laboratory technician, and the nursing staff working as a team is required in the emergency department to formulate the correct diagnosis and start timely management of the life-threatening secondary cause of a thunderclap headache.
Collaboration shared decision making and communication are key elements for a good outcome. The interprofessional care provided to the patient must use an integrated care pathway combined with an evidence-based approach. The earlier signs and symptoms of a complication are identified, the better the prognosis and outcome.