# Myelin-oligodendrocyte glycoprotein antibody-positive encephalitis in a patient with Sturge–Weber syndrome

## TL;DR

A 63-year-old woman with Sturge-Weber syndrome was diagnosed with MOG antibody-positive encephalitis after showing stroke-like symptoms and MRI findings.

## Contribution

This case highlights the rare co-occurrence of SWS and MOG antibody-positive encephalitis and suggests MOG testing in similar cases.

## Key findings

- The patient showed MRI features of leptomeningeal enhancement and cerebellar lesions.
- CSF analysis revealed pleocytosis and positive MOG antibodies in both serum and CSF.
- The patient responded rapidly to intravenous methylprednisolone treatment.

## Abstract

Sturge-Weber syndrome (SWS) is a rare congenital disorder associated with systemic vascular malformations characterized by port-wine stains, epilepsy, and glaucoma. Patients with SWS can develop stroke-like symptoms such as hemiparesis. We report a case of a 63-year old woman with SWS who developed left-sided hemiparesis and was finally diagnosed with myelin-oligodendrocyte glycoprotein (MOG) antibody-positive encephalitis. Brain magnetic resonance imaging (MRI) revealed right-dominant bilateral leptomeningeal enhancement, thickened dura mater, and a cerebellar lesion. Cerebrospinal fluid (CSF) examination showed pleocytosis. Both serum and CSF proved positive for MOG antibodies. The patient recovered immediately after intravenous methylprednisolone administration. SWS and MOG antibody-positive encephalitis share similar clinical findings of stroke-like symptoms and leptomeningeal enhancement on MRI. However, MOG antibody-positive encephalitis is highly steroid-responsive in most cases. If a patient with SWS develops stroke-like symptoms accompanied by abnormal CSF findings or subtentorial lesions, testing for MOG antibodies should be considered.