Abstract Details

Title An Autopsy Case of Autoimmune Glial Fibrillary Acidic Protein Astrocytopathy

Topic Autoimmune Neurology

Presentation(s) Autoimmune Neurology Posters (7:00 AM-5:00 PM)

Poster/Presentation
Number 024

Objective Autoimmune glial fibrillary acidic protein (GFAP) astrocytopathy is an emerging disease entity of autoimmune encephalitis, myelitis, and meningoencephalitis defined by the presence of anti-GFAP IgG in cerebrospinal fluid. Here, we report the first case of autoimmune GFAP astrocytopathy with autopsy and complete neuropathological evaluation, who presented with subacute meningoencehpalomyelitis and deceased after four different immunosuppressive therapies. An autopsy revealed co-existent neuroendocrine tumor in the small intestine, diffuse inflammation in the brain parenchyma, perivascular spaces, and the leptomeninges, with predominant T-cells, macrophages, and activated microglia. The B-cells and plasma cells were absent. Further studies are warranted to delineate the pathogenesis of this new autoimmune neurological disorder.

Background NA

Design/Methods NA

Results NA

Conclusions NA

Authors/Disclosures
Mai Yamakawa, MD (University of California Los Angeles) PRESENTER	Dr. Yamakawa has nothing to disclose.
	No disclosure on file
Yasir N. Jassam, MD, FRCP, FAAN (Hoag Specialty clinic)	Dr. Jassam has received personal compensation in the range of $5,000-$9,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Biogen. Dr. Jassam has received research support from Roche, Chugai Pharmaceutical MedImmune LLC.Novartis, MedDay. Actelion, Alexion Pharmaceuticals,TG Therapeutics Alexion Pharmaceuticals, Teva Pharmaceuticals, Genentech phamarcetucal companies..

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