| Literature DB >> 31927153 |
Alexander Allen1, Shivam Gulhar1, Ramin Haidari1, Juan Pablo Pauta Martinez1, Jonathan Bekenstein1, Robert DeLorenzo1, Yang Tang1, Unsong Oh2.
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.Entities:
Keywords: Autoimmune encephalomyelitis; Glial fibrillary acidic protein astrocytopathy; Neuropathy
Year: 2020 PMID: 31927153 DOI: 10.1016/j.msard.2019.101924
Source DB: PubMed Journal: Mult Scler Relat Disord ISSN: 2211-0348 Impact factor: 4.339