Idiopathic intracranial hypertension: Clinical nosography and field-testing of the ICHD diagnostic criteria. A case-control study

Cephalalgia. 2015 Jun;35(7):553-62. doi: 10.1177/0333102414550109. Epub 2014 Sep 16.


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.

Keywords: Idiopathic intracranial hypertension; case-control studies; diagnostic; headache; pseudotumor cerebri.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Adult
  • Case-Control Studies
  • Cough / complications
  • Cough / diagnosis
  • Cough / therapy
  • Female
  • Headache / complications
  • Headache / diagnosis
  • Headache / therapy
  • Humans
  • Male
  • Prospective Studies
  • Pseudotumor Cerebri / complications
  • Pseudotumor Cerebri / diagnosis*
  • Pseudotumor Cerebri / therapy*
  • Spinal Puncture / methods
  • Spinal Puncture / standards*
  • Young Adult