Autoimmune Glial Fibrillary Acidic Protein Astrocytopathy Presenting with Slowly Progressive Myelitis and Longitudinally Extensive Spinal Cord Lesions

Takuya Kudo; Akio Kimura; Kazuhiro Higashida; Megumi Yamada; Yuichi Hayashi; Takayoshi Shimohata

doi:10.2169/internalmedicine.5074-20

This article has now been updated. Please use the final version.

Autoimmune Glial Fibrillary Acidic Protein Astrocytopathy Presenting with Slowly Progressive Myelitis and Longitudinally Extensive Spinal Cord Lesions

Takuya Kudo, Akio Kimura, Kazuhiro Higashida, Megumi Yamada, Yuichi Hayashi, Takayoshi Shimohata

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Keywords: astrocytopathy, autoantibody, glial fibrillary acidic protein (GFAP), longitudinally extensive spinal cord lesion (LESCL), myelitis, neuromyelitis optica spectrum disorders (NMOSDs)

JOURNAL OPEN ACCESS Advance online publication

Article ID: 5074-20

DOI https://doi.org/10.2169/internalmedicine.5074-20

The final version of this article is now available: Vol. 59 (2020), No. 21 pp. 2777-2781

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Abstract

We report a 65-year-old man with autoimmune glial fibrillary acidic protein astrocytopathy (GFAP-A) who presented with gait disturbance that he had experienced for approximately half a year. On neurological examination, he displayed spastic paraplegia and autonomic dysfunctions including dysuria and constipation. Spinal cord magnetic resonance imaging showed longitudinally extensive spinal cord lesions (LESCLs) extending from the cervical to the thoracic cords. The patient was negative for anti-myelin oligodendrocyte glycoprotein and anti-aquaporin 4 antibodies. Treatment with corticosteroids and intravenous immunoglobulin resulted in a clinical improvement. It is important to distinguish GFAP-A from slowly progressive myelitis with LESCLs.

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