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

Publication date: Available online 31 December 2019Source: Multiple Sclerosis and Related DisordersAuthor(s): Bonaventure Ip, Crystal Lam, Vincent Ip, Anne Chan, Vincent Mok, Elaine Au, Eric Chan, Alexander LauAbstractAutoimmune 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.
Source: Multiple Sclerosis and Related Disorders - Category: Neurology Source Type: research