Increased risk of cluster headache in relatives of cluster headache patients

M. Leone, M. B. Russell, A. Rigamonti, A. Attanasio, D. D'Amico, L. Grazzi, G. Bussone

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Cluster headache is an uncommon, highly debilitating primary headache, characterized by brief, severe, unilateral headaches, accompanied by autonomic manifestation (eye reddening, lacrimation, rhinorrhea, ptosis, etc.). These attacks occur in cluster periods lasting weeks or months. The etiology and pathogenesis of this headache have not been clearly established. Until fairly recently the disease was considered sporadic. However, a family history of cluster headache is now reported in 2-7% of patients. A Danish genetic epidemiological survey showed an increased risk of cluster headache among relatives of cluster headache patients. We studied the familial risk of cluster headache in an Italian population. The families of 191 consecutive cluster headache patients (118 males, 73 females; mean age 45.9 years) from the Milan Headache Centre were included. All patients (probands) were of Italian origin, alive, and diagnosed with cluster headache according to IHS criteria. All underwent physical and neurological examination, and cerebral CT or MRI in order to exclude other general or neurological conditions. Information on relatives was collected by direct interview of the probands (n=118) or by questionnaire (n=73). In both cases information on age of onset, duration of illness, frequency of attacks, duration of cluster period and attacks, character, location and intensity of the pain and accompanying autonomic signs was collected. Living relatives reported as having cluster headache by the probands were examined at the Milan Headache Centre or interviewed by telephone. In 11 deceased relatives information provided by the probands was detailed enough to fulfil IHS criteria for cluster headache. The relative risk of cluster headache was calculated in first- and second-degree relatives of probands with cluster headache. Information on 3589 persons was obtained from the 191 cluster headache patients: 1036 were first degree (488 women and 548 males) and 2553 were second degree (1234 women and 1319 males). A positive family history was found in 19% (37/191) of the families. A total of 32 first-degree (15 women and 17 males) and 15 second-degree relatives (5 males and 10 women) were affected. The frequencies of cluster headache were 3.1% (32/1036) and 0.6% (15/2553) among first- and second-degree relatives, respectively. The relative risk of cluster headache was 26.89 (95% CI 17.57-36.21) in first-degree and 4.35 (95% CL 2.13-5.21) in second-degree relatives. This study shows increased familial risk of cluster headache in an Italian population, giving further support to the hypothesis that cluster headache is, in some families, an inherited disorder.

Original languageEnglish
JournalNeurological Sciences
Issue number4 SUPPL.
Publication statusPublished - 2000

ASJC Scopus subject areas

  • Neuroscience(all)
  • Clinical Neurology


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