Autoimmune glial fibrillary acidic protein astrocytopathy manifesting as subacute meningoencephalitis with descending my
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CASE REPORT
Open Access
Autoimmune glial fibrillary acidic protein astrocytopathy manifesting as subacute meningoencephalitis with descending myelitis: a case report Han Wang1*† , Jerome H. Chin2†, Bo-yan Fang3, Xi Chen1,4, Ai-lin Zhao5, Hai-tao Ren1 and Hong-zhi Guan1
Abstract Background: Glial fibrillary acidic protein (GFAP) autoimmune astrocytopathy is characterized by GFAP autoantibody positive encephalitis, meningoencephalitis or meningoencephalomyelitis. The initial clinical presentation may be similar to central nervous system infections making early diagnosis challenging. Case presentation: A Chinese female patient presented with subacute meningitis with symptoms of headache, vomiting, and fever. Cerebrospinal fluid (CSF) analysis showed monocytic pleocytosis, elevated protein level, low glucose level, and negative basic microbiological studies including Xpert MTB/RIF. Brain magnetic resonance imaging (MRI) showed bilateral cerebral cortical and white matter hyperintensities on FLAIR sequences. The patient was diagnosed with possible tuberculous meningitis and started on anti-tuberculosis therapy (ATT). Three months later, the patient developed cervical myelopathy and encephalopathy with persistent CSF pleocytosis. Five months later, tissue-based and cell-based assays demonstrated GFAP antibodies in blood and CSF. Her symptoms improved with repeated administration of intravenous immunoglobulin (IVIG) and corticosteroids. One-and-a-half -year followup showed neither clinical progression nor relapses. Conclusions: Anti-GFAP astrocytopathy should be included in the differential diagnosis of patients who present with subacute meningitis with negative microbiological studies and a progressive clinical course including encephalitis and/or myelitis. Keywords: GFAP, Encephalitis, Meningitis, Myelitis, Autoimmune
Background Autoimmune astrocytopathy with antibodies to glial fibrillary acidic protein (GFAP) was first described in 2016 [1]. The predominant clinical syndrome is meningoencephalo-myelitis or a limited form of the same, i.e. meningitis, encephalitis, and/or myelitis [1–7]. * Correspondence: [email protected] † Han Wang and Jerome H. Chin are authors contributed equally to the manuscript 1 Department of Neurology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences, Beijing 100730, China Full list of author information is available at the end of the article
The biomarker of this disorder is auto-antibody in CSF against GFAPα, the predominant intermediate filament protein in adult astrocytes, detected by cell-based testing. Since GFAP is an intracellular antigen, it is unknown if another target is responsible for the immunopathogenesis of the clinical manifestations of this autoimmune neurological disorder. Case reports and case series have described associations of anti-GFAP astrocyopathy with neoplasms and prodromal infectious symptoms [6]. Here we report a Chinese patient who developed a subacute febrile meningitis and was treated for
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