AIMS: The aims of this article are to characterize the headache in idiopathic intracranial hypertension (IIH) and to field-test the ICHD diagnostic criteria for headache attributed to IIH.

MATERIALS AND METHODS: We included 44 patients with new-onset IIH. Thirty-four patients with suspected but unconfirmed IIH served as controls. Headache and other IIH-related symptoms were assessed by a detailed standardized interview. In participants referred before diagnostic lumbar puncture (n = 67), we recorded headache intensity before and after cerebrospinal fluid (CSF) withdrawal.

RESULTS: Headache in patients with IIH was daily occurring in 86%, focal in 84% and pulsating in 52%. Aggravation of headache by coughing or straining and relief after CSF withdrawal were significantly more frequent in patients than in controls (p ≤ 0.002). From the ICHD-2 to the ICHD-3 beta classification the sensitivity increased from 60% to 86% whereas the specificity decreased from 86% to 53%. Based on our data the headache criteria can be revised to increase sensitivity to 95% and specificity to 65%.

CONCLUSION: Aggravation of headache by coughing or straining, relief after CSF withdrawal, retrobulbar pain and pulsatile tinnitus may suggest intracranial hypertension. Based on data from a well-defined IIH cohort, we propose a revision of the ICDH-3 beta diagnostic criteria with improved clinical applicability and increased sensitivity and specificity.