Headache in Sturge-Weber syndrome.

Based on reports of headache in arteriovenous malformations and epilepsy, one would predict that the prevalence of migraine headache in the Sturge-Weber syndrome would be higher than in the general population. A questionnaire designed to determine the prevalence and type of headache by IHS criteria was sent to the 500 members of the Sturge-Weber Foundation. The average age of the patients in this sample (71 patients) was 15 years. The sex ratio was approximately one to one. Headache meeting IHS criteria for migraine occurred in 28% (equally among males and females) compared to 17% in females and 5% in males in the general population. Neurologic deficits occurred in 58% during migraine. The prevalence of migraine in children under the age of 10 (31%) was higher than that reported in the general population (5%) and the age of onset was younger. The mechanism of migraine headache in Sturge-Weber syndrome may be similar to that found in epilepsy and vascular malformations.