Idiopathic Intracranial Hypertension Without Papilledema (IIHWOP) in Chronic Refractory Headache.

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

doi:10.3389/fneur.2018.00503

Abstract

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.

Highlights

Chronic headache (CH) is a challenging clinical entity because management is complex and often unsatisfactory
The primary endpoint of the study was to analyze the prevalence of intracranial hypertension without papilledema (IIHWOP) in a series of consecutive refractory chronic headache patients
The diagnosis of headache was chronic migraine in 39 patients (97.5%) and chronic tension-type headache with concomitant episodic migraine in 1 patient

Summary

Introduction

Chronic headache (CH) is a challenging clinical entity because management is complex and often unsatisfactory. Based on a long history of evidence [2, 3], recently, revised diagnostic criteria for pseudotumor cerebri syndrome by Friedman set the upper limit to 250 mmH2O and recognized the importance of MRI [4] According to these criteria, in the absence of papilledema or sixth nerve palsy, the diagnosis of IIHWOP can be suggested by the combination of elevated OP and the presence of at least three of the following neuroimaging findings: empty sella, distention of the perioptic subarachnoid space with or without a tortuous optic nerve, flattening of the posterior sclerae and transverse venous sinus stenosis (Table 1). To determine the prevalence of Idiopathic intracranial hypertension without papilledema (IIHWOP) testing revised diagnostic criteria by Friedman in refractory chronic headache (CH) patients

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