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[A case of autoimmune glial fibrillary acidic protein (GFAP) astrocytopathy]
Hiroshi Adachi1, Yuma Shiomi1, Akio Kimura2
1Department of Neurology, Hyogo Prefectural Amagasaki General Medical Center.
Abstract:
A 46-year-old man developed acute meningo-encephalitis with confusion, headache, fever, intractable hiccups, dysuria, myoclonus/tremor, and ataxia. Analysis of cerebrospinal fluid (CSF) showed elevated levels of cell counts and protein. Brain MRI demonstrated multiple linear increased FLAIR signals in bilateral basal ganglia and corona radiata. Repeated MRI showed T2 hyperintensity areas in the lower brainstem, sparing the area postrema. Immunotherapy with intravenous high-dose steroid and subsequent oral steroid was successful, and the symptoms improved completely. Later MRI study showed a striking resolution. Glial fibrillary acidic protein (GFAP) α antibody was positive in the CSF, while anti-aquaporin-4 antibody, anti-myelin oligodendrocyte glycoprotein antibody, and N-methyl-D-aspartate receptor antibody were all negative. There were no relapses at final follow-up of 6 months after onset. Autoimmune GFAP astrocytopathy is not an uncommon immune-mediated inflammatory disorder in the central nervous system.
Insights
Autoimmune GFAP astrocytopathy is an immune-mediated central nervous system disorder. This case study highlights successful immunotherapy treatment for acute meningo-encephalitis caused by GFAP alpha antibody.
Area of Science:
- Neuroimmunology
- Central Nervous System Inflammatory Disorders
- Astrocytic Disorders
Background:
- Autoimmune GFAP astrocytopathy is an emerging immune-mediated inflammatory disorder affecting the central nervous system.
- Distinguishing GFAP astrocytopathy from other autoimmune encephalitides is crucial for appropriate treatment and prognosis.
Observation:
- A 46-year-old male presented with acute meningo-encephalitis, exhibiting confusion, headache, fever, intractable hiccups, dysuria, myoclonus/tremor, and ataxia.
- Cerebrospinal fluid analysis revealed elevated cell counts and protein.
- Brain MRI showed characteristic linear FLAIR signals in the basal ganglia and corona radiata, with T2 hyperintensities in the lower brainstem.
Findings:
- Cerebrospinal fluid testing confirmed positivity for the Glial fibrillary acidic protein (GFAP) alpha antibody.
- Other tested antibodies, including anti-aquaporin-4, anti-myelin oligodendrocyte glycoprotein, and N-methyl-D-aspartate receptor antibodies, were negative.
- High-dose steroid immunotherapy led to complete symptom resolution and significant MRI improvement.
Implications:
- This case underscores the importance of considering GFAP astrocytopathy in the differential diagnosis of autoimmune encephalitis.
- Prompt diagnosis and immunotherapy, particularly with high-dose steroids, can lead to favorable outcomes and prevent relapses.
- Further research into GFAP astrocytopathy is warranted to understand its pathogenesis and optimize management strategies.
