Idiopathic Intracranial Hypertension With Sustained Normal Opening Pressure: A Case Report

TL;DR

A 30-year-old woman with symptoms of increased intracranial pressure was diagnosed with idiopathic intracranial hypertension despite normal opening pressure, highlighting the complexity of the condition.

Contribution

This case report presents a rare instance of IIH with sustained normal opening pressure and discusses its differential diagnosis and management.

Findings

The patient showed significant vision improvement after treatment with plasma exchange and steroids.

Imaging confirmed demyelination, necessitating the exclusion of other neurological conditions like MOGAD and MS.

Normal opening pressure during lumbar puncture challenges typical diagnostic criteria for IIH.

Abstract

Idiopathic intracranial hypertension (IIH) is a clinical diagnosis made from the exclusion of other possible causes of increased intracranial pressure (ICP): cerebrospinal fluid (CSF) overflow obstruction, mass lesion, infection, or malignancies. IIH is a phenomenon with a rapid onset that most notably presents in obese women of childbearing age. We present the case of a 30-year-old African American woman with a past medical history of bipolar disorder who arrived at the emergency department (ED) with acute bilateral vision loss, two weeks of bilateral retro-orbital headache, painful eye movements, and progressive vision loss with a decrease in appetite. Management involved plasma exchange, high-dose steroids, consistent neurological and ophthalmic tests, and close observation and follow-up. One week after the most recent ED visit, the patient confirmed a significant improvement in vision. When diagnosing our patient with IIH, it was imperative to rule out myelinating oligodendrocyte glycoprotein antibody-associated disease (MOGAD), multiple sclerosis (MS), and other demyelinating diseases such as optic neuritis. Initial brain imaging using computed tomography (CT) without contrast showed no evidence of intracranial hemorrhage but demonstrated the presence of prominent sheath complexes, suggestive of optic neuritis, and a slightly small pituitary gland height for a patient who is 30 years of age. Further testing with a CT venogram (CTV) and a magnetic resonance imaging (MRI) of the head and neck confirmed demyelination. The patient's lumbar puncture continuously revealed a normal opening pressure during her hospital stay. This case presentation will highlight and compare the current literature regarding the clinical presentation and management of idiopathic intracranial hypertension.

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