Atypical presentations of idiopathic intracranial hypertension

• Type of Material: Article
• Authors: Benson Chen, Emory University; Nancy Newman, Emory University; Valerie Biousse, Emory University
• Language: English
• Date: 2021-01-01
• Publisher: Wolters Kluwer
• Publication Version: Final Published Version
• Copyright Statement: © 2020 Taiwan J Ophthalmol
• License: Creative Commons Attribution-NonCommercial-ShareAlike 4.0 International
• Final Published Version (URL): http://dx.doi.org/10.4103/tjo.tjo_69_20
• Title of Journal or Parent Work: Taiwan Journal of Ophthalmology
• Volume: 11
• Issue: 1
• Start Page: 25
• End Page: 38
• Grant/Funding Information: None.
• Keywords: pseudotumor cerebri; Idiopathic intracranial hypertension; magnetic resonance imaging
• Research Categories: Health Sciences, Medicine and Surgery; Health Sciences, Opthamology

Abstract

Idiopathic intracranial hypertension (IIH) is a disorder of unknown etiology that results in isolated raised intracranial pressure. Classic symptoms and signs of IIH include headache, papilledema, diplopia from sixth nerve palsy and divergence insufficiency, and pulsatile tinnitus. Atypical presentations include: (1) highly asymmetric or even unilateral papilledema, and IIH without papilledema; (2) ocular motor disturbances from third nerve palsy, fourth nerve palsy, internuclear ophthalmoplegia, diffuse ophthalmoplegia, and skew deviation; (3) olfactory dysfunction; (4) trigeminal nerve dysfunction; (5) facial nerve dysfunction; (6) hearing loss and vestibular dysfunction; (7) lower cranial nerve dysfunction including deviated uvula, torticollis, and tongue weakness; (8) spontaneous skull base cerebrospinal fluid leak; and (9) seizures. Although atypical findings should raise a red flag and prompt further investigation for an alternative etiology, clinicians should be familiar with these unusual presentations.