A case of chronic progressive autoimmune GFAP astrocytopathy with extensive meningoencephalomyelitis and contrast enhancement on MRI

Hironori Oka; Takumi Nakamura; Takashi Sugawara; Kunihiko Ishizawa; Masakuni Amari; Takeshi Kawarabayashi; Koichi Okamoto; Masamitsu Takatama; Satoshi Nakata; Yuhei Yoshimoto; Ayako Yamazaki; Hideaki Yokoo; Akio Kimura; Takayoshi Shimohata; Yoshio Ikeda; Mikio Shoji

doi:10.1016/j.ensci.2024.100507

A case of chronic progressive autoimmune GFAP astrocytopathy with extensive meningoencephalomyelitis and contrast enhancement on MRI

eNeurologicalSci. 2024 Jun 1:35:100507. doi: 10.1016/j.ensci.2024.100507. eCollection 2024 Jun.

Authors

Hironori Oka^{1

2}, Takumi Nakamura¹, Takashi Sugawara², Kunihiko Ishizawa², Masakuni Amari², Takeshi Kawarabayashi², Koichi Okamoto², Masamitsu Takatama², Satoshi Nakata³, Yuhei Yoshimoto³, Ayako Yamazaki⁴, Hideaki Yokoo⁴, Akio Kimura⁵, Takayoshi Shimohata⁵, Yoshio Ikeda¹, Mikio Shoji²

Affiliations

¹ Department of Neurology, Gunma University Graduate School of Medicine, 3-39-22 Showa-machi, Maebashi 371-0034, Japan.
² Geriatrics Research Institute and Hospital, 3-26-8 Otomo-machi, Maebashi 371-0847, Japan.
³ Department of Neurosurgery, Gunma University Graduate School of Medicine, 3-39-22 Showa-machi, Maebashi 371-8511, Japan.
⁴ Department of Human Pathology, Gunma University Graduate School of Medicine, 3-39-22 Showa-machi, Maebashi 371-8511, Japan.
⁵ Department of Neurology, Gifu University Graduate School of Medicine, 1-1 Yanagito-machi, Gifu 501-1112, Japan.

Abstract

•We herein present a case of chronic progressive autoimmune GFAP astrocytopathy.•Symmetrical high-intensity signals on FLAIR were observed in the white matter of the temporal and occipital lobes, lateral cerebral ventricle walls, hippocampus, amygdala, and occipital cortex, with extensive Gd enhancement in radial perivascular lesions and the ependyma in the choroid plexus.•Improvements were achieved by 4 courses of IVMP and one of IVIg.

Keywords: Autoimmune GFAP astrocytopathy; Chronic progressive meningoencephalomyelitis; Extensive perivascular gadolinium enhancement; Intravenous immunoglobulin; Intravenous methylprednisolone.