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• Vol.62 No.5
• CLINICA NEUROL, 62: 386−390, 2022
• Vol.62 No.5 contents

Case Report

Recurrent autoimmune glial fibrillary acidic protein (GFAP) astrocytopathy with long cervical cord lesions: a case report

Masaki Namekawa, M.D.¹⁾, Shinya Oginezawa, M.D.¹⁾, Kimura Akio, M.D., Ph.D.²⁾, Takayoshi Shimohata, M.D., Ph.D.²⁾, Mutsuo Oyake, M.D., Ph.D.¹⁾ and Nobuya Fujita, M.D., Ph.D.¹⁾

¹⁾ Department of Neurology, Nagaoka Red Cross Hospital
²⁾ Department of Neurology, Gifu University Graduate School of Medicine

A 61-year-old man who had suffered two episodes of generalized convulsion in a two-year period was admitted to our hospital because of progressive gait disturbance during the previous five months. Neurological examination revealed cognitive impairment, spasticity of the lower limbs, truncal ataxia, and dysautonomia including orthostatic hypotension, dysuria and hypohydrosis. Brain fluid-attenuated inversion recovery (FLAIR) MRI detected high-signal-intensity lesions in the periventricular white matter and centrum semiovale, with punctate gadolinium (Gd) enhancement. Spinal MRI detected swollen cervical long cord lesions extending from C2 to C6. Although methylprednisolone pulse treatment initially ameliorated the symptoms and MRI abnormal findings, clinical symptoms and MRI abnormalities including new cervical lateral column lesions reminiscent of those in autoimmune glial fibrillary acidic protein (GFAP) astrocytopathy (GFAP-A) relapsed two months later. At this point, anti-GFAPα antibody was detected in the cerebrospinal fluid. Although the clinical course of GFAP-A has been well reported, the present case showed a chronic refractory course.
Full Text of this Article in Japanese PDF (2034K)

(CLINICA NEUROL, 62: 386−390, 2022)
key words: autoimmune glial fibrillary acidic protein (GFAP) astrocytopathy, chronic meningoencephalomyelitis, long cervical cord lesion, spinal lateral columns lesion

(Received: 14-Oct-21)