Headaches in patients with neurofibromatosis-1

An analysis of patients followed with a diagnosis of neurofibromatosis-1 and headache was conducted. Characterization of headache type was done after chart review of 81 patients with neurofibromatosis-1 and headache. Consent was obtained for subsequent telephone interviews using a standardized questionnaire concerning the onset, characteristics, timing, triggers, and associated symptoms of the patients' headaches. Data was summarized and tabulated. Of 132 patients with neurofibromatosis-1, 81 were identified with any headache by screening history. Recurrent headaches were present in 77% of patients and in 47% of our neurofibromatosis-1 clinic population. Fifty-three of 81 patients were accessible for and agreeable to telephone interview. There were 23 male patients and 30 female patients aged 5 6/12 to 49 6/12 years, with a mean age of 20.9 years. Eighty-one percent reported having experienced recurrent headaches within the year. The majority reported onset of headache prior to the age of 10 years. Headache characteristics included the following: frequency of monthly or less, frontotemporal location, pulsating or pressing quality, and moderate severity (pain scale 4 to 5 out of 10). Headaches interfered with daily activities, had weekend occurrence, and had a duration less than 2 hours. Common headache triggers included stress, "change in weather," menstruation, fatigue, and certain foods. A high percentage of patients reported associated symptoms of nausea with or without vomiting (37%), phonophobia, photophobia, pallor, and visual scotoma. We classified 34% of the patients as having migraine (25% with aura, 9% without aura), 45% with nonmigrainous headache only, and 15% with mixed headache types (either intermittently), and 7.5% with other head pains. We conclude that patients with neurofibromatosis-1 are at greater risk for headaches than the general population. While the prevalence of both migraine and nonnigraine headache is somewhat greater than in the general population, the proportion of tension-type headache, especially in young children, is greater than expected.