Cluster Headache, Familial

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WHO-ICD-10 version:2010

Diseases of the nervous system

Episodic and paroxysmal disorders

OMIM Number


Mode of Inheritance

Autosomal dominant


Cluster headaches (CH) are a rare form of severe disabling headache. CH is characterized by episodes of severe unilateral pain in the orbital, supraorbital and/or temporal areas that last from 15 to 180 minutes, with recurrence up to 8 times daily. It is accompanied by local signs and symptoms of autonomic dysfunction occurring on the side, such as conjunctival redness, tearing eyes, nasal congestion, rhinorrhea, sweating of the face and forehead, myosis, ptosis, and edema of the eyelid. The attacks occur in series lasting weeks or months. The attacks are extremely painful, so that CH is also commonly called suicide headache. There are two forms of CH: episodic CH, characterized by alternating active periods, with daily or almost daily attacks lasting from 7 days to one year, and remission periods lasting one month or more that are totally symptom free; and chronic CH, in which attacks recur for greater than one year without remission or with remissions lasting less than one month. CH mainly affects young adults, predominantly males (M/F sex ratio = 4) and available evidence suggests that it is a lifelong disorder in the majority of patients, but with longer remission periods over time.

Although, the exact pathogenesis of this condition remains unclear, the underlying abnormality is in the posterior hypothalamus with subsequent trigeminovascular and cranial autonomic activation. Alcohol, nicotine, exercise, and elevated environmental temperature are recognized as triggers of CH. The treatment of CH is only symptomatic. The only two treatments of attacks with proven efficacy are subcutaneous sumatriptan and oxygen inhalation.

CH has traditionally been considered a sporadic disease. Recently, however, a familial recurrence has been appreciated as several CH cases have been observed in family pedigrees. Furthermore, family studies indicate that first-degree relatives of CH probands carry a 5- to 18-fold and the second degree relatives carry a 1- to 3-fold increased relative risk of the disease. CH has been considered a probable autosomal dominant disease with a penetrance of 0.3-0.34 in males and 0.17-0.21 in females. However, autosomal recessive pattern has been suggested in certain families. In addition, the hypocretin receptor 2 (HCRTR2) G1246A polymorphism has been associated with the risk for cluster headache.

Epidemiology in the Arab World

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Other Reports


Alzoubi et al. (2009) investigated the prevalence of headache among 4,836 adults in Jordan encountered in the period between January 2007 to November 2008. As much as 82.3% of participants complained from headache at least once per year. Nearly, 36.1% were tension-type headache and 59% of the participants had other family members who suffered from headache.


Bessisso et al. (2005) studied the prevalence of headache and migraine and associated factors among school children in the State of Qatar through a cross-sectional study conducted over a period from March 2001 to April, 2003. A total of 1000 school children ranging from 6-17 years old, selected by a multi-stage stratified cluster sampling method, were interviewed by means of a questionnaire. Of these, 851 school children (27.7% males, and 72.3% females) responded to the questionnaire. This study showed that the prevalence rate of headache was 85%. A family history of headache was observed among the study subject (45.1% of females and 36.6% of males had a family history of headache). In the study group, Bessisso et al. (2005) found different types of headache: 28% had unclassified headache, 20% had chronic daily headache, 14.6 had episodic tension headache, and 11.9% had migraine. Of the total subjects, 43.6% experienced unilateral headache and 4.2% of them had migraine with aura. Headache was found to be more prevalent in females (86.5%) than males (81%). Also, the study showed that girls (86.5%) had more frequent headache episodes than boys. It was also observed that the oldest children had more frequent episodes of headache; the highest rate was seen in the age group of 11-15 years old (49%). The most common triggers for headache were found to be fatigue (35.8%) and lack of sleep (17.6%). The most common symptoms prior to headache were found to be change in mood for female students (39.1%) and blurred vision for males (34.6%). The impact of headache on children was found to be frequent absence from school (80%), which affected their school performance.

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