Rinsho Shinkeigaku (Clinical Neurology)

Case Report

A case of autoimmune glial fibrillary acidic protein astrocytopathy with various symptoms such as optic disc edema and cerebellar ataxia

Marimo Yonesu, M.D.1), Akiko Hosokawa, M.D., Ph.D.1), Kenji Yutani, M.D., Ph.D.2), Akio Kimura, M.D., Ph.D.3), Takayoshi Shimohata, M.D., Ph.D.3) and Misa Nakano, M.D., Ph.D.1)

1)Department of Neurology, Suita Municipal Hospital
2)Department of Radiology, Suita Municipal Hospital
3)Department of Neurology, Gifu University

A 59-year-old man had developed visual abnormality, nausea, headache, and weight loss since three months before. The ophthalmologist found severe optic disc edema in both eyes, and referred him to our hospital. The patient had mild cerebellar ataxia. Increased cerebrospinal fluid pressure, increased protein and cell counts, positive oligoclonal band, and contrast-enhanced head MRI showed multiple linear perivascular radial gadolinium enhancement around bilateral lateral ventricles. His subjective and objective findings significantly improved with steroid treatment. The cerebrospinal fluid was found to be positive for glial fibrillary acidic protein (GFAP) antibodies, and a diagnosis of GFAP astrocytopathy was obtained. When optic edema or radial contrast effects was observed on contrast-enhanced MRI, GFAP astrocytopathy should be considerd. Prompt immunotherapy is required to circumvent the development of permanent visual impairment.
Full Text of this Article in Japanese PDF (1215K)

(CLINICA NEUROL, 64: 408−412, 2024)
key words: meningoencephalitis, autoimmune GFAP astrocytopathy, increased intracranial pressure, optic disc edema, cerebellar ataxia

(Received: 15-Nov-23)