1. Autoimmune glial fibrillary acidic protein astrocytopathy resulting in treatment-refractory flaccid paralysis
- Author
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Shivam Gulhar, Ramin Haidari, Alexander Allen, Jonathan Bekenstein, Unsong Oh, Juan Pablo Pauta Martinez, Robert J. DeLorenzo, and Yang Tang
- Subjects
Pathology ,medicine.medical_specialty ,Flaccid paralysis ,Nerve root ,Encephalomyelitis ,macromolecular substances ,Neurological disorder ,03 medical and health sciences ,0302 clinical medicine ,Medicine ,030212 general & internal medicine ,Denervation ,Glial fibrillary acidic protein ,biology ,business.industry ,Multiple sclerosis ,Meningoencephalitis ,General Medicine ,medicine.disease ,nervous system ,Neurology ,biology.protein ,Neurology (clinical) ,medicine.symptom ,business ,030217 neurology & neurosurgery - 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.
- Published
- 2019