RT Journal Article
SR Electronic
T1 Autopsy Case of Meningoencephalomyelitis Associated With Glial Fibrillary Acidic Protein Antibody
JF Neurology - Neuroimmunology Neuroinflammation
JO Neurol Neuroimmunol Neuroinflamm
FD Lippincott Williams & Wilkins
SP e1081
DO 10.1212/NXI.0000000000001081
VO 8
IS 6
A1 Yamakawa, Mai
A1 Hogan, Keenan O.
A1 Leever, John
A1 Jassam, Yasir N.
YR 2021
UL http://nn.neurology.org/content/8/6/e1081.abstract
AB Background and Objectives To describe the autopsy findings and neuropathologic evaluation of autoimmune meningoencephalomyelitis associated with glial fibrillary acidic protein (GFAP) antibody.Methods We reviewed the clinical course, imaging, laboratory, and autopsy findings of a patient with autoimmune meningoencephalomyelitis associated with GFAP antibody who had a refractory course to multiple immunosuppressive therapies.Results The patient was a 70-year-old man who was diagnosed as GFAP antibody-associated autoimmune meningoencephalomyelitis. MRI of the head showed linear perivascular enhancement in the midbrain and the basal ganglia. Despite treatment with high-dose corticosteroids, plasma exchange, IV immunoglobulins, and cyclophosphamide, he died with devastating neurologic complications. Autopsy revealed a coexistent neuroendocrine tumor in the small intestine and diffuse inflammation in the brain parenchyma, perivascular spaces, and leptomeninges, with predominant T-cells, macrophages, and activated microglia. B-cells and plasma cells were absent. There was no astrocyte involvement with change in GFAP immunostaining.Discussion This case illustrates autoimmune meningoencephalomyelitis associated with GFAP antibody in the CSF and coexistent neuroendocrine tumor. The autopsy findings were nonspecific and did not demonstrate astrocyte involvement. Further accumulation of cases is warranted to delineate the utility and pathogenic significance of the GFAP autoantibody.AQP-4=aquaporin 4; CLIPPERS=chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids; GFAP=glial fibrillary acidic protein; WDNET=well-differentiated neuroendocrine tumor