Abstract Details

Title Clinical Characteristics of Autoimmune Glial Fibrillary Acidic Protein Astrocytopathy in Japan

Topic Autoimmune Neurology

Presentation(s) P2 - Poster Session 2 (8:00 AM-9:00 AM)

Poster/Presentation
Number 15-002

Objective To investigate the clinical features and outcomes of patients with autoimmune glial fibrillary acidic protein (GFAP) astrocytopathy (GFAP-A) in Japan.

Background GFAP-A is a spectrum of immunotherapy-responsive autoimmune inflammatory central nervous system (CNS) disorders that are distinct from infectious meningoencephalitis and idiopathic inflammatory CNS disorders.

Design/Methods Overall, 278 cerebrospinal fluid (CSF) samples were tested by both tissue-based and transfected cell-based immunofluorescence assays, and data from 23 patients, who tested positive for the GFAP autoantibody in their CSF were analyzed retrospectively.

Results Of the 23 patients (median age 56 [range: 31-66] years), nine were female (39%). During hospitalization, the neurological findings included consciousness disturbance (n=18, 75%), meningeal sign (n=15, 63%), tremor and/or myoclonus (n=15, 63%), autonomic dysfunction (n=15, 63%), hyperreflexia (n=14, 58%), limb and/or truncal ataxia (n=12, 50%), and cognitive dysfunction (n=11, 46%). Ovarian teratoma was detected in two patients; one of who had anti-N-methyl-D-aspartate receptor encephalitis While, the other had thyroid follicular adenoma. On admission, all patients showed lymphocyte-predominant pleocytosis (median: 102/μL, range: 34−177/μL) and elevated protein levels (median: 189 mg/dL, range: 135−230 mg/dL). Most patients showed transiently increased CSF adenosine deaminase (ADA) levels. On brain MRI, 10 of 18 patients (56%) showed linear perivascular radial enhancement patterns in the cerebral white matter. Seven of 16 patients (44%) showed longitudinally extensive T2-hyperintense lesions. All patients, except one (96%), were administered corticosteroid therapy, which decreased the modified Rankin Scale (mRS) scores (median mRS: on admission: 5, range 4−5; on discharge: 3, range 2−3; and at the last follow-up: 1, range 0−2). At the last follow-up visit, no patient was found with a relapse.

Conclusions Movement disorders including tremor and myoclonus are key symptoms of suspected GFAP-A. Brain linear perivascular radial gadolinium-enhancement patterns and transient elevation of ADA levels may be characteristic findings and hallmarks of GFAP-A in Japan.

Authors/Disclosures
Akio Kimura, MD (Gifu University Graduate School of Medicine) PRESENTER	Dr. Kimura has nothing to disclose.
Nobuaki Yoshikura, MD, PhD (Gifu univesity graduate school of medicine department of neurology)	Dr. Yoshikura has nothing to disclose.
Yuichi Hayashi (Department of Neurology, Gifu University Grad)	Yuichi Hayashi has nothing to disclose.
Takayoshi Shimohata, MD, FAAN (Department of Neurology, Gifu University)	Dr. Shimohata has nothing to disclose.

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