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Autoimmune glial fibrillary acidic protein astrocytopathy resulting in treatment-refractory flaccid paralysis

Authors :
Shivam Gulhar
Ramin Haidari
Alexander Allen
Jonathan Bekenstein
Unsong Oh
Juan Pablo Pauta Martinez
Robert J. DeLorenzo
Yang Tang
Source :
Multiple sclerosis and related disorders. 39
Publication Year :
2019

Abstract

Autoimmune glial fibrillary acidic protein (GFAP) astrocytopathy is an autoimmune neurological disorder associated with the presence of anti-GFAP IgG. Meningoencephalitis is the predominant clinical presentation of autoimmune GFAP astrocytopathy in published case series. We report a case of autoimmune GFAP astrocytopathy with the unusual feature of radiculoneuritis in addition to encephalomyelitis, resulting in flaccid paralysis unresponsive to immunotherapy. Imaging data confirmed involvement of brain, spinal cord and nerve roots. Electrodiagnostic testing showed changes consistent with a severe sensorimotor neuropathy with active denervation. The results of this case suggest the need for future studies to assess the impact of peripheral nerve involvement on the outcome of autoimmune GFAP astrocytopathy.

Details

ISSN :
22110356
Volume :
39
Database :
OpenAIRE
Journal :
Multiple sclerosis and related disorders
Accession number :
edsair.doi.dedup.....0d064b18e84957841418ed0084400a05