Related Experiment Video
Updated: Jun 7, 2025
Pre-Chiasmatic, Single Injection of Autologous Blood to Induce Experimental Subarachnoid Hemorrhage in a Rat Model
Published on: June 18, 2021
MOGAD presenting as fulminant intracranial hypertension
Sai Nagaratnam1, Amardeep Gill2, Niroshan Jeyakumar1
1Department of Neurology, Westmead Hospital, Sydney, NSW, Australia.
Objectives:
Myelin oligodendrocyte glycoprotein antibody disease (MOGAD) has an expanding phenotype. We describe two cases of MOGAD with associated severe intracranial hypertension. Case 1: A 21-year-old male presented with diffuse cortical encephalitis and intracranial hypertension with both serum and CSF MOG antibody positivity. Initial brain CT scan was normal but subsequent demyelination was evident on MRI. Case 2: A 44-year-old female presented with a progressive brainstem encephalitis and intracranial hypertension and normal MRI, with later development of subcortical demyelination which was confirmed on brain biopsy. CSF-restricted MOG antibody was detected following the biopsy results.
Results:
Both patients presented with clinical features of severe intracranial hypertension requiring surgical management followed by immunosuppressive therapy (methylprednisone and plasma exchange; and intravenous immunoglobulin and plasma exchange) leading to clinical improvement.
Discussion:
MOGAD should be in the differential diagnosis of acute severe intracranial hypertension even in the absence of demyelination on initial neuroimaging. Clinicians should be alert of this syndrome that requires combined management of intracranial pressure in addition to early and intensive immunotherapy.
Insights
Myelin oligodendrocyte glycoprotein antibody disease (MOGAD) can cause severe intracranial hypertension. Early immunotherapy and intracranial pressure management are crucial for MOGAD patients with this presentation.
Area of Science:
- Neuroimmunology
- Neurology
Background:
- Myelin oligodendrocyte glycoprotein antibody disease (MOGAD) is an autoimmune demyelinating disorder with a diverse clinical spectrum.
- Intracranial hypertension is not typically considered a primary manifestation of MOGAD.
Observation:
- Two cases of MOGAD presenting with severe intracranial hypertension are described.
- One patient had diffuse cortical encephalitis, while the other had brainstem encephalitis, with MOG antibodies detected in serum and CSF.
- Initial neuroimaging was normal in one case, with demyelination appearing later on MRI, and the other case showed normal MRI initially, with subcortical demyelination confirmed by biopsy.
Findings:
- Both patients required surgical management for intracranial hypertension followed by immunosuppressive therapies, including methylprednisone, plasma exchange, and intravenous immunoglobulin.
- Clinical improvement was observed in both cases after treatment.
Implications:
- MOGAD should be considered in the differential diagnosis of acute severe intracranial hypertension, even with normal initial neuroimaging.
- Prompt recognition and combined management of intracranial pressure and early, intensive immunotherapy are vital for favorable outcomes in MOGAD-associated intracranial hypertension.
Related Concept Videos
01:25Glaucoma: Overview
01:28Angle Closure Glaucoma: Treatment
01:25Increased Body Temperature