Idiopathic intracranial hypertension without papilledema (IIHWOP) in chronic refractory headache

Valentina Favoni, Giulia Pierangeli, Francesco Toni, Luigi Cirillo, Chiara La Morgia, Samir Abu-Rumeileh, Monica Messia, Raffaele Agati, Pietro Cortelli, Sabina Cevoli

Research output: Contribution to journalArticlepeer-review


Background: To determine the prevalence of Idiopathic intracranial hypertension without papilledema (IIHWOP) testing revised diagnostic criteria by Friedman in refractory chronic headache (CH) patients. Methods: This is a prospective observational study. Each patient underwent ophthalmologic evaluation and Optical Coherence Tomography; brain magnetic resonance venography (MRV) and a lumbar puncture (LP) with opening pressure (OP) measurement. CSF withdrawal was performed in patients with CSF OP > 200 mmH20. IIHWOP was defined according Friedman's diagnostic criteria. Effect of CSF withdrawal was evaluated clinically in a 6-month follow-up and with a MRV study at 1 month. Results: Forty-five consecutive patients were enrolled. Five were excluded due to protocol violations. Analyses were conducted in 40 patients (32 F, 8 M; mean age 49.4 ± 10.8). None had papilledema. Nine patients (22.5%) had OP greater than 200 mmH2O, two of them above 250 mmH2O. Two (5%) had neuroimaging findings suggestive of elevated intracranial pressure. One of them (2.5%) met the newly proposed diagnostic criteria by Friedman for IIHWOP. After CSF withdrawal seven (77.8%) of the nine patients improved. No changes in neuroimaging findings were found. Conclusions: We found a low prevalence (2.5%) of IIHWOP in refractory CH patients according to current diagnostic criteria. In agreement with Friedman's criteria, our results confirm that a diagnosis of IIHWOP should be based on CSF OP and the combination of neuroradiological findings. However, where to set the CSF OP upper limit in IIHWOP needs further field testing. Although IIHWOP is a rare clinical condition, it should be considered and treated in refractory CH patients.

Original languageEnglish
Article number503
JournalFrontiers in Neurology
Issue numberJUN
Publication statusPublished - Jun 26 2018


  • Chronic headache
  • Idiopathic intracranial hypertension
  • Lumbar puncture
  • MRI
  • Refractory headache

ASJC Scopus subject areas

  • Neurology
  • Clinical Neurology

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