Tokimura Ryo, Matsuda Nozomu, Kobayashi Shunsuke, Kimura Akio, Kanai Kazuaki
Department of Neurology, Fukushima Medical University, 1 Hikarigaoka, Fukushima, Fukushima 960-1295, Japan.
Department of Neurology and Geriatrics, Gifu University Graduate School of Medicine, 1-1 Yanagido, Gifu 501-1194, Japan.
eNeurologicalSci. 2020 Feb 10;18:100229. doi: 10.1016/j.ensci.2020.100229. eCollection 2020 Mar.
Autoimmune GFAP astrocytopathy is a new clinical entity and only a limited number of cases have been reported. Here we report the results of multimodal central conduction studies performed in a case of this disorder. A 72-year-old woman developed gradual cognitive decline and gait disturbance. A neurological examination revealed moderate amnesia, papilloedema, and pyramidal tract impairment of the bilateral lower limbs. The diagnosis of autoimmune GFAP astrocytopathy was made based on the typical MRI findings of periventricular radial linear gadolinium enhancement in the brain and longitudinally extensive lesions in the spinal cord, and anti-GFAP antibody detected in the cerebrospinal fluid. Somatosensory evoked potentials and transcranial magnetic stimulation studies revealed prolongation of conduction times. Visual evoked potentials showed an unusual W-shaped pattern. To our knowledge, this is the first neurophysiological demonstration of prolonged central conduction times in the autoimmune GFAP astrocytopathy. Further investigations are needed to establish the clinical value the neurophysiological examinations in this disorder.
自身免疫性胶质纤维酸性蛋白星形细胞病是一种新的临床病症，目前仅有少数病例报告。在此，我们报告了对一名该病症患者进行多模式中枢传导研究的结果。一名72岁女性出现渐进性认知衰退和步态障碍。神经系统检查发现中度失忆、视乳头水肿以及双侧下肢锥体束受损。自身免疫性胶质纤维酸性蛋白星形细胞病的诊断基于脑部典型的MRI表现，即脑室周围放射性线性钆增强以及脊髓纵向广泛病变，以及脑脊液中检测到的抗胶质纤维酸性蛋白抗体。体感诱发电位和经颅磁刺激研究显示传导时间延长。视觉诱发电位呈现出异常的W形模式。据我们所知，这是自身免疫性胶质纤维酸性蛋白星形细胞病中枢传导时间延长的首次神经生理学证明。需要进一步研究以确定神经生理学检查在该病症中的临床价值。
