Complete bilateral ophthalmoplegia in malignant intracranial hypertension in a child

Eur J Ophthalmol. 2020 Oct 20:1120672120966562. doi: 10.1177/1120672120966562. Online ahead of print.

Abstract

Purpose: To describe a case of fulminant idiopathic intracranial hypertension (IIH) in a child with "malignant" presentation.

Case report: A 16-year-old, previously healthy, girl presented with bilateral visual loss and bilateral global limitation of eye movements in the absence of headache. Extensive laboratory evaluation for infectious, inflammatory, autoimmune, and neoplastic conditions was negative. Magnetic resonance imaging (MRI) of the brain and lumbar puncture findings were consistent with a diagnosis of IIH. Extraocular motility improved in the next few days as well as optic disc edema but visual acuity remained poor.

Conclusion: The authors believe that the acute, severe, and fulminant ("malignant") presentation with markedly elevated intracranial pressure may produce the unique presentation of severe vision loss and bilateral complete ophthalmoplegia. Interestingly, there was no headache. To our knowledge this is the first such case to be reported in the English language ophthalmic literature.

Keywords: Neuro imaging; eye movement disorders; neuro-ophthalmic disease; neuro-ophthalmology; ocular motility disorders; optic neuropathy; pediatric ophthalmology; strabismus.