Seizures as the Initial Manifestation of Idiopathic Intracranial Hypertension Spectrum Disorder

ABSTRACT

Idiopathic intracranial hypertension (IIH) is a syndrome of isolated elevated intracranial pressure of unknown aetiology. The IIH spectrum has evolved over the past decade making the diagnosis and management more challenging. The neurological examination in IIH is typically normal except for papilloedema and possible cranial nerve 6 palsy. Recent publications have highlighted skull base thinning and remodelling in patients with chronic IIH. Resulting skull base defects can cause meningo-encephalocoeles, which are potential epileptogenic foci. We describe the clinical and radiological characteristics of five IIH patients with seizures and meningo-encephalocoeles as the presenting manifestations of IIH spectrum disorder.

KEYWORDS:

• Idiopathic intracranial hypertension
• seizures
• meningocele
• encephalocele
• meningoencephalocele

Contributors

NAB, WB, AB, and RM collected the data, designed the manuscript, prepared the first draft, figure, table, and panel. VB, AMS and NJN designed the manuscript, contributed to the discussion, critically edited the review, and approved its final version.

Disclosure statement

None of the authors have any competing interest. VB is consultant for GenSight Biologics and Neuro-phoenix. NJN is consultant for GenSight Biologics, Santhera/Chiesi, Stoke, and Neurophoenix; receives research support from GenSight Biologics and Santhera/Chiesi.

Additional information

Funding

VB, NJN are supported by the National Institutes of Health’s National Eye Institute core grant P30-EY06360 (Department of Ophthalmology, Emory University School of Medicine) and by a departmental grant from Research to Prevent Blindness (New York, NY). WB received a scholarship from Geneva University Hospitals, Geneva, Switzerland.