Highlights
- •A woman developed meningo-encephalomyelitis associated GFAP astrocytopathy.
- •Patient had acute hearing loss, tetraplegia, bulbar palsy and respiratory failure.
- •Partial recovery was noted with immunosuppressants, plasma exchange and rituximab.
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.
Keywords
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References
- Glial fibrillary acidic protein immunoglobulin G as biomarker of autoimmune astrocytopathy: analysis of 102 patients.Ann Neurol. 2017; 81: 298-309
- Clinical and immunological characteristics of the spectrum of GFAP autoimmunity: a case series of 22 patients.J. Neurol. Neurosurg. Psychiatry. 2018; 89: 138-146
- Autoimmune glial fibrillary acidic protein astrocytopathy: a novel meningoencephalomyelitis.JAMA Neurol. 2016; 73: 1297-1307
- Overlapping autoimmune syndromes in patients with glial fibrillary acidic protein antibodies.Front. Neurol. 2018; 9: 251
- Autoimmune glial fibrillary acidic protein astrocytopathy: a review of the literature.Front. Immunol. 2018; 9: 2802
Article info
Publication history
Published online: December 31, 2019
Accepted:
December 30,
2019
Received in revised form:
December 24,
2019
Received:
September 23,
2019
Identification
Copyright
© 2019 Elsevier B.V. All rights reserved.
