Objective:

We highlight a rare case with severe myelin oligodendrocyte glycoprotein (MOG)-
IgG-associated cerebral cortical encephalitis (CCE) presenting as bilateral cytotoxic edema.

Background:

MOGAD is a recognized cause of neuroinflammation, and typically presents as acute disseminated encephalomyelitis (ADEM) in children. Recently, cerebral cortical encephalitis (CCE) involving cortical gray matter inflammation has been identified as a manifestation of the disease. Cases typically present with unilateral lesions and seizures which are steroid responsive. We report the case of MOG-IgG-associated CCE in a patient who presented with bilateral cortical cytotoxic edema, refractory seizures, and critical illness. This fulminant phenotype challenges the mild clinical course and expands the spectrum of MOGAD-related cerebral cortical encephalitis.

Design/Methods:

NA

Results:

A 13 year-old male with prior history of ADEM, six years ago, presents with fever,
headache, and encephalopathy after recent otitis media. Initial workup showed leukocytosis, CSF pleocytosis, negative infectious panel, and normal CT brain. He was treated empirically for meningitis vs. recurrent encephalomyelitis with antibiotics, IVIG and steroids. During his clinical course, he developed seizures and became obtunded. Repeat CT brain revealed diffuse cerebral edema and MRI brain revealed severe bilateral cortical edema with diffusion restriction, concerning for cytotoxic edema. He was intubated and treated with hyper-osmolor therapy. CSF encephalitis panel returned negative. Serum MOG antibody returned positive (1:320). He received 5 sessions of PLEX, cyclophosphamide, and tocilizumab and was eventually extubated; however he showed limited neurological improvement

Conclusions:

This case highlights a rare, fulminant presentation of MOG antibody–associated disease characterized by severe encephalopathy and bilateral cortical cytotoxic edema. Recognition of this imaging pattern is crucial, as it indicates a more aggressive disease course requiring early, intensive immunotherapy. Despite prompt initiation of multiple immunomodulatory treatments, the neurological outcome in such cases can remain poor, underscoring the need for further research to understand the pathophysiology and optimize management of severe MOG-CCE variants.