A Fulminant Case of Demyelinating Encephalitis With Extensive Cortical Involvement Associated With Anti-MOG Antibodies

Frontiers in Neurology

Thomas Gattringer¹  Michaela Tanja Haindl¹  Sonja Hochmeister¹  Martin Asslaber²  Verena Stangl²  Romana Höftberger³  Christian Enzinger⁴ 

[1] Department of Neurology, Medical University Graz, Graz, Austria;Department of Pathology, Medical University Graz, Graz, Austria;Division of Neuropathology and Neurochemistry, Department of Neurology, Medical University of Vienna, Vienna, Austria;Division of Neuroradiology, Vascular and Interventional Radiology, Department of Radiology, Medical University Graz, Graz, Austria;

关键词: MOG;    autoimmunity;    antibodies;    encephalitis;    demyelination;   

DOI  :  10.3389/fneur.2020.00031

来源: DOAJ

【 摘 要 】

Anti-myelin oligodendrocyte glycoprotein (MOG) antibodies (MOG-Abs) are commonly associated with clinical presentations as acute disseminated encephalomyelitis (ADEM) in both adults and children and anti-aquaporin 4 antibody-seronegative neuromyelitis optica spectrum disorder (NMOSD) and related syndromes such as optic neuritis, myelitis, and brainstem encephalitis. Most often, the presence of MOG-Abs is associated with a more benign clinical course and a good response to steroids. Here, we present a case report of a previously healthy 52-year-old female patient with fulminant demyelinating encephalitis, leading to death within a week after the first presenting symptoms from a massive brain edema irresponsive to high-dose intravenous steroids as well as osmotic therapy. The final diagnosis was only made postmortem after serum anti-MOG-Abs results were available. Histopathological analysis of the brain revealed extensive, predominantly cortical demyelinating lesions in the frontal, temporal, and parietal lobes with intracortical, leukocortical, and subpial plaques, associated with pronounced perivenous deposition of activated complement complex as well as features of acute MS characterized by destructive lesions.

【 授权许可】

Unknown