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Abstract Details

Clinical and Immunological Investigation of Glial Fibrillar Acidic Protein Astrocytopathy in China—A Single Center Study of 11 Cases
Autoimmune Neurology
P4 - Poster Session 4 (5:30 PM-6:30 PM)
15-001

To report the clinical and immunological characteristics of 11 Chinese patients with GFAP autoimmunity from Beijing Tongren Hospital.

Recently, a novel meningoencephalomyelitis associated with IgG against GFAP isoforms has been described.

From June 2016 to June 2019, 208 patients with suspected neurological autoimmune disease after serum and cerebrospinal fluid tested for conventional neural antibodies were scanned for additional autoantibodies with prefixed rat brain section. Samples showed a characteristic immunoreactive pattern reminiscent of GFAP of astrocytes were selected and detected with in-house cell based assay using cells expressing GFAP-alpha and GFAP-delta protein, respectively. Finally, the dual positive results were confirmed with pre-fixed cell based assay commercial kits expressing GFAP-alpha protein only.
Serum and/or cerebrospinal fluid of 12/248 (5.7%) patients were dual positive by both tissue based assay and in-house cell based assay. IgG of 12/12 patients bound to in-house GFAP-alpha, 5/12 bound to in-house GFAP-delta. IgG of 11/12 bound to commercial GFAP-alpha. The median age of 11 subjects was 59 years (range: 39–78 years). Three patients were females (27%). The clinical manifestation including:encephalitis in 3, ophthalmoplegia in 1, optic neuritis in 1, tremor and urinary retention in 1, parkinsonism with /without cerebellar ataxia in 3, meningoencephalomyelitis in 2. Three patients present with prodromal fever of unknown origin. All three patients with encephalitis had cerebrospinal fluid NMDAR-IgG. One meningoencephalomyelitis patient had coexisting serum Yo-IgG. None of the subjects had neoplasms discovered. Eight patients were treated with immunotherapy (intravenous methylprednisolone, 6; intravenous immunoglobulin, 2). Three patients also treated with acyclovir. One patient treated with acyclovir only. Eight patients (73%) improved.

The clinical presentation of GFAP astrocytopathy is heterogeneous. Coexisting other neurological autoimmunity is common. Although intracellular antigen location, GFAP-IgG could indeed be markers of a T-cell mediated autoimmune response. And immunotherapy is beneficial in most cases.
Authors/Disclosures
Lei Liu, MD, PhD (Beijing Tongren Hospital,Capital Medical University)
PRESENTER 		Dr. Liu has nothing to disclose.
No disclosure on file