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Lifetime prevalence and concordance risk of cluster headache in the Swedish twin population

From the Departments of Neurology (K.E., E.W.), Clinical Neuroscience (D.A.S.), and Medical Epidemiology and Biostatistics (N.L.P.), Karolinska Institutet, Stockholm, Sweden.

Address correspondence and reprint requests to Dr Svensson, Division of Neurology, Department of Clinical Neuroscience, Karolinska Institutet, Karolinska University Hospital, Huddinge, R54, SE-14186 Stockholm, Sweden; e-mail: Dan.Svensson{at}ki.se

Objective: To examine the lifetime prevalence and the concordance risk of cluster headache (CH) in a twin sample representative of the Swedish general population.

Methods: The authors assessed CH as defined by the second edition of the International Classification of Headache Disorders in 31,750 registered twins born from 1935 to 1958. Structured lay screening interviews were followed by neurologist interviews of possible cases. Co-twins of affected index twins were follow-up interviewed regardless of their screening outcome.

Results: A total of 250 screening-positives (0.8%) were found, of which 218 (88%) were follow-up interviewed. Forty-five (21%) had the CH diagnosis verified. Among screen-negatives, hospitalization records pointed at two more verified cases and index twins at one more verified case. A total of 48 CH cases provided a crude lifetime prevalence of 151 per 100,000 (95% CI: 108, 194). The male-to-female sex ratio corresponded to 4.8 (95% CI: 2.3, 9.9). The crude lifetime prevalence of CH was higher in the twins born from 1945 to 1958 than in the twins born from 1935 to 1944 (190 vs 90/100,000). CH recurred in 2 of 12 co-twins of monozygous index twins (including 1 nonparticipant twin), whereas all co-twins of 25 dizygous index twins proved to be unaffected.

Conclusion: As many as 1 per 500 of the general population are affected by cluster headache. Twin concordance seems low, but genetic factors may play a role for familial clustering.

Additional material related to this article can be found on the Neurology Web site. Go to www.neurology.org and scroll down the Table of Contents for the September 12 issue to find the title link for this article.

Dan A. Svensson was supported by a stipend from GlaxoWellcome. The Swedish Twin Registry is supported by grants from the Department of Higher Education, the Swedish Scientific Council, and AstraZeneca. The SALT Study was supported by grants from NIH (AG08724 to M. Gatz), the Swedish Council for the Planning and Coordination of Research, and Merck, Sharp & Dohme (the headache part).

Disclosure: The authors report no conflicts of interest.

Received January 25, 2006. Accepted in final form May 4, 2006.

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September 12 Highlights

Neurology 2006 67: 734-735. [Full Text]  

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