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

Alexander Allen; Shivam Gulhar; Ramin Haidari; Juan Pablo Pauta Martinez; Jonathan Bekenstein; Robert DeLorenzo; Yang Tang; Unsong Oh

doi:10.1016/j.msard.2019.101924

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

Mult Scler Relat Disord. 2020 Jan 3:39:101924. doi: 10.1016/j.msard.2019.101924. Online ahead of print.

Authors

Alexander Allen¹, Shivam Gulhar¹, Ramin Haidari¹, Juan Pablo Pauta Martinez¹, Jonathan Bekenstein¹, Robert DeLorenzo¹, Yang Tang¹, Unsong Oh²

Affiliations

¹ Department of Neurology, Virginia Commonwealth University School of Medicine, Richmond, VA, USA.
² Department of Neurology, Virginia Commonwealth University School of Medicine, Richmond, VA, USA. Electronic address: uoh@vcu.edu.

PMID: 31927153
DOI: 10.1016/j.msard.2019.101924

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.

Keywords: Autoimmune encephalomyelitis; Glial fibrillary acidic protein astrocytopathy; Neuropathy.