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Autoimmune glial fibillary acidic protein astrocytopathy associated meningoencephalomyelitis and bilateral sensorineuronal deafness. | LitMetric

Autoimmune glial fibillary acidic protein astrocytopathy associated meningoencephalomyelitis and bilateral sensorineuronal deafness.

Mult Scler Relat Disord

Division of Neurology, Department of Medicine and Therapeutics, The Chinese University of Hong Kong, Prince of Wales Hospital, Shatin, Hong Kong SAR, China. Electronic address:

Published: May 2020

AI Article Synopsis

  • Autoimmune encephalitis can mimic infectious encephalitis and one severe variant is meningoencephalomyelitis, which can cause significant neurological symptoms.
  • A case study details an adult Chinese woman who experienced rapid confusion, high protein levels in her CSF, and deterioration leading to coma, diagnosed as anti-GFAP meningoencephalomyelitis.
  • Treatment with intravenous immunoglobulin and methylprednisolone showed partial improvement, but additional therapies like plasma exchange and rituximab led to significant recovery.

Article Abstract

Autoimmune encephalitis is an important group of disease that can mimic infectious encephalitis, with one of the most severe forms being meningoencephalomyelitis. One of the recently identified biomarkers, glial fibillary acidic protein (GFAP), targets the cytosolic intermediate filament protein of astrocytes and causes a variety of clinical symptoms. Here, we report an adult Chinese woman presented with acute onset of confusion, CSF lymphocytosis, markedly elevated total protein mimicking tuberculosis meningitis with rapid deterioration resulted in coma and respiratory failure. She was diagnosed with anti-GFAP meningoencephalomyelitis, which later developed tetraplegia, sensorineural hearing loss, brainstem, bulbar and respiratory dysfunction. Intravenous immunoglobulin and methylprednisolone resulted in partial improvement. Further immunotherapy with plasma exchange and rituximab resulted in marked recovery.

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Source
http://dx.doi.org/10.1016/j.msard.2019.101922DOI Listing

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