Headache in patients with neurofibromatosis type 1

A study of headache in a homogeneously ascertained population of 181 subjects suffering from neurofibromatosis type 1 is described. All subjects underwent a diagnostic protocol including imaging studies (for subjects over 5 years old up until 1992). Headache data were collected by means of a questionnaire. Headache was present in 55 of 181 subjects (25 males). Overall headache frequency was 30%, which is not significantly different from the frequency of headache reported in the general population. Headache was primary in 52 cases (5 migraine and 47 tension-type) and secondary to obstructive hydrocephalus with brain tumor-induced intracranial hypertension in 3 with a tension-type pattern. It was concluded that headache is not a specific feature of neurofibromatosis type 1, it is not significantly related to central nervous system abnormalities, and in itself, it is not an indication for neuroradiological examination.