Case report: Autoimmune glial fibrillary acidic protein astrocytopathy misdiagnosed as tuberculous meningitis

Abstract

IntroductionAutoimmune glial fibrillary acidic protein (GFAP) astrocytopathy is a new form of autoimmunity-mediated central nervous system disease. It is especially easy to misdiagnose when clinical symptoms and cerebrospinal fluid (CSF) indicators are similar to those observed in patients with tuberculous meningitis (TBM).MethodsWe retrospectively analyzed five cases of autoimmune GFAP astrocytopathy that were initially misdiagnosed as TBM.ResultsIn the five reported cases, all but one patient had meningoencephalitis in the clinic, and all patients exhibited increased pressure, lymphocytosis, increased protein levels, and decreased glucose levels in their CSF results and did not have typical imaging findings of autoimmune GFAP astrocytopathy. TBM was the initial diagnosis in all five patients. However, we found no direct evidence of tuberculosis infection, and anti-tuberculosis treatment had inconclusive effects. Following a GFAP antibody test, the diagnosis of autoimmune GFAP astrocytopathy was made.ConclusionWhen there is a suspected diagnosis of TBM but TB-related tests are negative, the possibility of autoimmune GFAP astrocytopathy should be considered.

Clinical and neuroimaging phenotypes of autoimmune glial fibrillary acidic protein astrocytopathy: A systematic review and meta‐analysis

“…Corpus callosum involvement was rather rare, reported in only 5% of the patients, and often associated with reversible splenial lesion syndrome [21][22][23][24]. Findings of restricted diffusion were found in only 3% of the patients, commonly in the corpus callosum.…”

“…However, GFAP-A generally exhibits a positive response to immunotherapies, particularly high-dose corticosteroids administered intravenously, even though a propensity for relapses and, in some cases, fatal outcomes have been reported [2][3][4][5].…”

“…The clinical picture may be further complicated by coexisting neuronal autoantibodies and concurrent malignancies. However, GFAP‐A generally exhibits a positive response to immunotherapies, particularly high‐dose corticosteroids administered intravenously, even though a propensity for relapses and, in some cases, fatal outcomes have been reported [2–5].…”