Cluster headache

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Associate Editor(s)-in-Chief: Sabeeh Islam, MBBS[2] Saumya Easaw, M.B.B.S.[3]

Cluster headaches are rare, extremely painful and debilitating headaches that occur in groups or clusters. Often appearing during seasonal changes. They may also be described as suicide headaches, a reference to the excruciating pain and resulting desperation that has culminated in actual suicide. Cluster headaches are classified as vascular headaches. The intense pain is caused by the dilation of blood vessels which creates pressure on the trigeminal nerve. While this process is the immediate cause of the pain, the etiology (underlying cause or causes) is not fully understood. Differential of cluster headache usually involves syndromes that manifest as unilateral headache, brief but frequent attacks. Such syndromes include the following: Chronic paroxysmal hemicrania (CPH) , Short-lasting unilateral neuralgiform headache attacks, Trigeminal neuralgia, primary stabbing headache, and headache associated with an underlying intracranial lesion. Patients with cluster headaches usually have an underlying secondary cause of headaches such as structural brain lesion, TBI and genetic predisposition that may increase the risk of cluster headache. Cluster headache is one of the most common trigeminal autonomic cephalalgias. It is a life long condition and usually results in reduced quality of life and marked functional disability. Although, the frequency of episodes usually decrease with age, 80% of patients continue to experience the attacks even after 15 years of onset. Diagnostic criteria for cluster headache require the following as per ICHD-3: International Classification of Headache Disorders, 3rd edition. Cluster headache sufferers typically experience very severe headaches of a piercing quality near one eye or temple that last for fifteen minutes to three hours with some lasting days (rarely more than three days). The headaches are typically unilateral and rarely change sides during the same cycle (see episodic). Cluster headaches have a characteristic circadian periodicity and usually present with autonomic symptoms. Neuro-imaging with an magnetic resonance imaging (MRI) scan with and without contrast is preferred to non-contrast computed tomography (CT) scan. MRI is usually indicated to rule out underlying structural brain lesion or to evaluate brain and pituitary gland in patient presenting with typical features or highly suspicious of cluster headache. Cluster headache treatment is generally divided into acute therapy or abortive therapy focused at aborting individual attacks and preventive or prophylactic therapy aimed at preventing recurrent attacks during the cluster period. Cluster headaches often go undiagnosed for many years, being confused with migraine or other causes of headache. Because of the relative rareness of the condition and ambiguity of the symptoms, some sufferers may not receive treatment in the emergency room and patients may even be mistaken as exhibiting drug-seeking behavior. Over-the-counter pain medications (such as aspirin, paracetamol, and ibuprofen) typically have no effect on the pain from a cluster headache. Unlike other headaches such as migraines and tension headaches, cluster headaches do not respond to biofeedback. Some have reported partial relief from narcotic pain killers. Percocet (Oxycodone with paracetamol) has had widespread success amongst some cluster headache patients, especially males. Anecdotal evidence indicates that cluster headaches can be so excruciating that even morphine does little to ease the pain. However, some newer medications like fentanyl (and Percocet) have shown promise in early studies and use. The drug of choice for primary prevention of cluster headache is Verapamil. Other agents that can be used also include glucocorticoids, topiramate and lithium.

Cluster headaches have been called by several other names in the past including Erythroprosopalgia of Bing, ciliary neuralgia, migrainous neuralgia, erythromelagia of the head, Horton’s headache, histaminic cephalalgia, petrosal neuralgia, sphenopalatine neuralgia, Vidian neuralgia, Sluder’s neuralgia, and hemicrania angioparalyticia. In 1952, cluster headache was named by American physician E. Charles Kunkle. In 1998, cluster headache was established as a separate entity by International Classification of Headache Disorders (ICHD-I). In 2004, restlessness/agitation was added with the ICHD-II revision. In 2013, two new autonomic features were added in the ICHD-III-beta revision in 2013.2.

The clear pathogenesis of cluster headache is still unknown. Various thought processes and pathophysiologic mechanism are considered to be involved in the pathogenesis. Generally 3 brain systems are mostly found to be involved or associated with the pathophysiology of cluster headache. Three brain systems are thought to be involved: hypothalamus, autonomic system and trigeminal nucleus. Orexin/hypocretin receptor 2 (HCRTR2) gene mutations are found to be particularly associated with cluster headaches in a couple of separate independent studies. Cluster headaches may also be associated with or secondary to other conditions such as: Hypothalamic and pituitary tumors, meningiomas (anywhere from the cavernous sinus to the upper cervical spine), carotid artery dissections, vascular malformations and sleep apnea.

Cluster headaches are generally associated with underlying neurologic conditions and structural abnormalities of brain and the pituitary gland. Common causes include; Chronic paroxysmal hemicrania (CPH), Subarachnoid hemorrhage, Trigeminal Neuralgia, Cyclical migraine, Hemicrania continua, Intermittent hydrocephalus, Sleep apnea, Acute optic neuritis, Acute glaucoma, Acute hypertension, Cerebral venous thrombosis, and Interior carotid artery dissection.

Differential of cluster headache usually involves syndromes that manifest as unilateral headache, brief but frequent attacks. Such syndromes include the following: Chronic paroxysmal hemicrania (CPH) , Short-lasting unilateral neuralgiform headache attacks, Trigeminal neuralgia, primary stabbing headache, and headache associated with an underlying intracranial lesion.

The prevalence of cluster headache is <1 percent and mostly affects men. A meta-analysis of 16 population-based epidemiologic studies showed that the lifetime prevalence of cluster headache for adults of all ages was 0.1 percent and the 1 year prevalence of cluster headache was 53 per 100,000.

Patients with cluster headaches usually have an underlying secondary cause of headaches such as structural brain lesion, TBI and genetic predisposition that may increase the risk of cluster headache.

Cluster headache is one of the most common trigeminal autonomic cephalalgias. It is a life long condition and usually results in reduced quality of life and marked functional disability. Although, the frequency of episodes usually decrease with age, 80% of patients continue to experience the attacks even after 15 years of onset.

Diagnostic criteria for cluster headache require the following as per ICHD-3: International Classification of Headache Disorders, 3rd edition.

Cluster headache sufferers typically experience very severe headaches of a piercing quality near one eye or temple that last for fifteen minutes to three hours with some lasting days (rarely more than three days). The headaches are typically unilateral and rarely change sides during the same cycle (see episodic). Cluster headaches have a characteristic circadian periodicity and usually present with autonomic symptoms.

Examination findings in cluster headache are usually autonomic related; suggesting parasympathetic hyperactivity and sympathetic impairment. Exam findings may include ptosis, lacrimation, conjuctival injection, miosis, nasal congestion, rhinorrhea, neck stiffness and photophobia.

Laboratory investigations, such as electrophysiologic testing (eg, evoked potential, electroencephalography) and examination of the cerebrospinal fluid, are not found to be helpful and needs more studies for further evaluation.

Neuro-imaging with a non-contrast computed tomography (CT) scan is usually indicated to rule out underlying structural brain lesion or to evaluate brain and pituitary gland in patient presenting with typical features or highly suspicious of cluster headache.

Neuro-imaging with an magnetic resonance imaging (MRI) scan with and without contrast is preferred to non-contrast computed tomography (CT) scan. MRI is usually indicated to rule out underlying structural brain lesion or to evaluate brain and pituitary gland in patient presenting with typical features or highly suspicious of cluster headache.

Cluster headache treatment is generally divided into acute therapy or abortive therapy focused at aborting individual attacks and preventive or prophylactic therapy aimed at preventing recurrent attacks during the cluster period. Cluster headaches often go undiagnosed for many years, being confused with migraine or other causes of headache. Because of the relative rareness of the condition and ambiguity of the symptoms, some sufferers may not receive treatment in the emergency room and patients may even be mistaken as exhibiting drug-seeking behavior. Over-the-counter pain medications (such as aspirin, paracetamol, and ibuprofen) typically have no effect on the pain from a cluster headache. Unlike other headaches such as migraines and tension headaches, cluster headaches do not respond to biofeedback. Some have reported partial relief from narcotic pain killers. Percocet (Oxycodone with paracetamol) has had widespread success amongst some cluster headache patients, especially males. Anecdotal evidence indicates that cluster headaches can be so excruciating that even morphine does little to ease the pain. However, some newer medications like fentanyl (and Percocet) have shown promise in early studies and use.

The drug of choice for primary prevention of cluster headache is Verapamil. Other agents that can be used also include glucocorticoids, topiramate and lithium.

Template:WikiDoc Sources

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Associate Editor(s)-in-Chief: Sabeeh Islam, MBBS[2], Saumya Easaw, M.B.B.S.[3]

Cluster headaches have been called by several other names in the past including Erythroprosopalgia of Bing, ciliary neuralgia, migrainous neuralgia, erythromelagia of the head, Horton’s headache, histaminic cephalalgia, petrosal neuralgia, sphenopalatine neuralgia, Vidian neuralgia, Sluder’s neuralgia, and hemicrania angioparalyticia. In 1952, cluster headache was named by American physician E. Charles Kunkle. In 1998, cluster headache was established as a separate entity by International Classification of Headache Disorders (ICHD-I). In 2004, restlessness/agitation was added with the ICHD-II revision. In 2013, two new autonomic features were added in the ICHD-III-beta revision in 2013.2.

• In 2004, the first anti-CGRP treatment (intravenous CGRP-receptor antagonist or gepant, olcegepant) was established as an effective treatment for Primary headaches.
• In May 17, 2018, Erenumab, an anti-CGRP receptor monoclonal antibody, for prevention of migraine.

• Translational research models were actually transformed into actual treatments, were also FDA approved.
• The ongoing discoveries has opened a new era in the acute and preventive treatment of primary headache disorders.

Template:WikiDoc Sources

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Associate Editor(s)-in-Chief: Sabeeh Islam, MBBS[2], Saumya Easaw, M.B.B.S.[3]

The clear pathogenesis of cluster headache is still unknown. Various thought processes and pathophysiologic mechanism are considered to be involved in the pathogenesis. Generally 3 brain systems are mostly found to be involved or associated with the pathophysiology of cluster headache. Three brain systems are thought to be involved: hypothalamus, autonomic system and trigeminal nucleus. Orexin/hypocretin receptor 2 (HCRTR2) gene mutations are found to be particularly associated with cluster headaches in a couple of separate independent studies. Cluster headaches may also be associated with or secondary to other conditions such as: Hypothalamic and pituitary tumors, meningiomas (anywhere from the cavernous sinus to the upper cervical spine), carotid artery dissections, vascular malformations and sleep apnea.

The clear pathogenesis of cluster headache is still unknown. Various thought processes and pathophysiologic mechanism are considered to be involved in the pathogenesis. Generally 3 brain systems are mostly found to be involved or associated with the pathophysiology of cluster headache.^{[1][2][3][4][5][6]}

• Hypothalamus
  • The site of the circadian pacemaker in the suprachiasmatic nucleus
  • Posterior hypothalamus activation is most commonly involved with the onset of cluster headache
  • Alterations in hypothalamic and pituitary molecules (orexin, melatonin, and luteinizing hormone)
• Autonomic system
  • The superior salivatory nucleus and the sphenopalatine ganglion, which includes molecules such as vasoactive intestinal peptide (VIP)
  • Depending upon the setting, stimulation of ganglion may stimulate or abort an episode of cluster headache
• Trigeminal nucleus
  • Trigeminovascular system
    • Trigeminal nucleus plus Large cranial blood vessels and meninges
  • The Trigeminocervical complex
    • Trigeminal nucleus plus upper cervical dorsal horns
  • Trigeminal nucleus is usually associated with the pain component of cluster headaches
  • This system includes molecules such as calcitonin gene-related peptide, pituitary adenylate cyclase-activating peptide

• First-degree relatives of sufferers are more likely to have the condition than the population at large.^[7]
• Orexin/hypocretin receptor 2 (HCRTR2) gene mutations are found to be particularly associated with cluster headaches in a couple of separate independent studies.

The following trigger factor may be involved in the pathogenesis of cluster headache:

• Nitroglycerin (glyceryl trinitrate)
• Alcohol
• Nicotine dependence or smoking
• Exposure to hydrocarbons (petroleum solvents, perfume)
• Decreased tolerance to heat, and becoming overheated may act as a trigger.

Cluster headaches may also be associated with or secondary to other conditions such as:

• Hypothalamic and pituitary tumors
• Meningiomas (anywhere from the cavernous sinus to the upper cervical spine)
• Carotid artery dissection
• Vascular malformations
• Sleep apnea

Template:WikiDoc Sources

_

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief:

Disease name] may be caused by [cause1], [cause2], or [cause3].

OR

Common causes of [disease] include [cause1], [cause2], and [cause3].

OR

The most common cause of [disease name] is [cause 1]. Less common causes of [disease name] include [cause 2], [cause 3], and [cause 4].

OR

The cause of [disease name] has not been identified. To review risk factors for the development of [disease name], click here.

• Life-threatening causes include conditions which may result in death or permanent disability within 24 hours if left untreated. There are no life-threatening causes of disease name, however complications resulting from untreated disease name is common.
• Life-threatening causes of [symptom/manifestation] include [cause1], [cause2], and [cause3].
• [Cause] is a life-threatening cause of [disease].

Common causes of [disease name] may include:

• [Cause1]
• [Cause2]
• [Cause3]

OR

• [Disease name] is caused by an infection with [pathogen name].
• [Pathogen name] is caused by [pathogen name].

Less common causes of [disease name] include:

• [Cause1]
• [Cause2]
• [Cause3]

• [Disease name] is caused by a mutation in the [gene name] gene.

List the causes of the disease in alphabetical order:

Template:WH Template:WS

Please help WikiDoc by adding more content here. It’s easy! Click here to learn about editing.

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Associate Editor(s)-in-Chief: Saumya Easaw, M.B.B.S.[2]

International Headache Society’s classification of cluster headache:^[1]

1. Headache is associated with at least one of the following signs that have to be present on the pain side:

   • Miosis
   • Eyelid edema
   • Conjunctival injection (red eyes with lacrimation)
   • Rhinorrhea
   • Forehead and facial sweating
   • Nasal congestion
   • Ptosis
   • Lacrimation

2. Severe unilateral, orbital, supraorbital and/or temporal pain lasting 15 to 180 min if untreated
3. Frequency of attacks: from one every other day to eight in a day
4. At least five attacks fulfilling number 2 and 3

Template:WikiDoc Sources

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Associate Editor(s)-in-Chief: Sabeeh Islam, MBBS[2]

Differential of cluster headache usually involves syndromes that manifest as unilateral headache, brief but frequent attacks. Such syndromes include the following: Chronic paroxysmal hemicrania (CPH) , Short-lasting unilateral neuralgiform headache attacks (SUNCT and SUNA), Trigeminal neuralgia, primary stabbing headache, and headache associated with an underlying intracranial lesion.

Differential of cluster headache usually involves syndromes that manifest as unilateral headache, brief but frequent attacks. Such syndromes include the following:^[1]

• Chronic paroxysmal hemicrania (CPH) is a condition similar to cluster headache, but CPH responds well to treatment with the anti-inflammatory drug indomethacin. With CPH, the attacks are much shorter, often lasting only seconds.^[2]
• Short-lasting unilateral neuralgiform headache attacks (SUNCT and SUNA)
• Trigeminal neuralgia^[3]
• Primary stabbing headache
• Headache associated with an underlying intracranial lesion^[4]
  • Intracranial large artery aneurysms
  • Meningiomas
  • Brain arteriovenous malformations
  • Pituitary macroadenomas
  • Recurrent nasopharyngeal carcinoma
  • Metallic foreign body in the maxillary sinus
  • Aspergilloma in sphenoid sinus
  • Benign posterior fossa tumor
  • Cavernous hemangioma

Differential diagnosis of headache includes: Migraine, tension-type headache, cluster headache, seizure, meningitis, encephalitis, neurosyphilis, SAH, subdural hematoma, brain tumor, hypertensive encephalopathy, brain abscess, multiple sclerosis, hemorrhagic stroke, Wernickes encephalopathy, and drug toxicity etc.^{[5][6][7][8][9][10][11][12][13][14][15][16][17][18][19][20][21][22][23]}

Diagnosis of meningitis, is based on clinical presentation in combination with CSF analysis. CSF analysis has major role for diagnosis and rule out other possibilities. The following table summarizes the CSF findings in different types of meningitis.^{[6][7][8][9][10]}

Template:WikiDoc Sources

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Associate Editor(s)-in-Chief: Sabeeh Islam, MBBS[2] Saumya Easaw, M.B.B.S.[3]

The prevalence of cluster headache is <1 percent and mostly affects men. A meta-analysis of 16 population-based epidemiologic studies showed that the lifetime prevalence of cluster headache for adults of all ages was 0.1 percent and the 1 year prevalence of cluster headache was 53 per 100,000.

• The prevalence of cluster headache is <1 percent and mostly affects men.^[1][2]
• A meta-analysis of 16 population-based epidemiologic studies showed:^[3][4][5]
  • The lifetime prevalence of cluster headache for adults of all ages was found to be 0.1 percent
  • The one-year prevalence of cluster headache was noted to be 53 per 100,000

Template:WikiDoc Sources

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Associate Editor(s)-in-Chief: Sabeeh Islam, MBBS[2]

Patients with cluster headaches usually have an underlying secondary cause of headaches such as structural brain lesion, TBI and genetic predisposition that may increase the risk of cluster headache.

• Genetic predisposition^[1][2][3]
• Underlying structural brain lesion
• Traumatic brain injury
  

Template:WikiDoc Sources

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Associate Editor(s)-in-Chief: Sabeeh Islam, MBBS[2]

Cluster headache is one of the most common trigeminal autonomic cephalalgias. It is a life long condition and usually results in reduced quality of life and marked functional disability. Although, the frequency of episodes usually decrease with age, 80% of patients continue to experience the attacks even after 15 years of onset.

• Cluster headache is one of the most common trigeminal autonomic cephalalgias.^[1][2][3][4]
• It is a life long condition and usually results in reduced quality of life and marked functional disability.
• Although, the frequency of episodes usually decrease with age, 80% of patients continue to experience the attacks even after 15 years of onset.^[5][6][7]

Cluster headache if left untreated or undiagnosed, is found to be associated with increased risk of depression and suicide.^[1][2]

Template:WikiDoc Sources