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Parainfectious Anti-Glial Fibrillary Acidic Protein-Associated Meningoencephalitis
Jae Young Joo1, Dallah Yoo2, Tae-Beom Ahn1,2
1Department of Medicine, Graduate School, Kyung Hee University, Seoul, Korea.
Abstract:
Movement disorders associated with glial fibrillary acidic protein (GFAP) autoantibodies have rarely been reported as ataxia or tremors. A 32-year-old man with headache and fever, initially diagnosed with viral meningoencephalitis, showed gradual improvement with empirical treatment. Two weeks after the illness, he suddenly developed orofacial, tongue, and neck dyskinesia accompanied by oculomotor abnormalities, which developed into severe generalized choreoballism. Brain magnetic resonance imaging (fluid-attenuated inversion recovery) showed signal hyperintensities in the bilateral globus pallidus interna. The clinical picture suggested an acute inflammatory trigger of secondary autoimmune encephalitis. The autoimmune antibody test was positive for GFAP, with the strongest reactivity in the cerebrospinal fluid (CSF) before treatment and decreased reactivity in serial CSF examinations during immunotherapy. Dyskinesia gradually improved to the extent that it could be controlled with only oral medications. This patient presented with parainfectious GFAP meningoencephalitis with distinctive clinical features and imaging findings.
Insights
Glial fibrillary acidic protein (GFAP) meningoencephalitis can cause severe movement disorders like choreoballism. This case highlights a parainfectious presentation with unique clinical and imaging findings, improving with immunotherapy.
Area of Science:
- Neuroimmunology
- Neurology
Background:
- Glial fibrillary acidic protein (GFAP) autoantibodies are rarely linked to movement disorders, typically presenting as ataxia or tremors.
- Autoimmune encephalitis can be triggered by infections, leading to diverse neurological symptoms.
Observation:
- A 32-year-old man developed severe orofacial, neck, and generalized choreoballism with oculomotor abnormalities after an initial diagnosis of viral meningoencephalitis.
- Brain MRI revealed signal hyperintensities in the bilateral globus pallidus interna, suggesting an acute inflammatory process.
Findings:
- The patient tested positive for GFAP autoantibodies, with highest levels in cerebrospinal fluid (CSF) before treatment.
- Immunotherapy led to decreased GFAP antibody reactivity in serial CSF examinations and gradual improvement of dyskinesia.
Implications:
- This case demonstrates a rare parainfectious GFAP meningoencephalitis with distinctive choreoballism and specific MRI findings.
- Early diagnosis and immunotherapy are crucial for managing GFAP-antibody-associated autoimmune encephalitis and its associated movement disorders.
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