Namekawa Masaki, Oginezawa Shinya, Akio Kimura, Shimohata Takayoshi, Oyake Mutsuo, Fujita Nobuya
Department of Neurology, Nagaoka Red Cross Hospital.
Department of Neurology, Gifu University Graduate School of Medicine.
Rinsho Shinkeigaku. 2022 May 31;62(5):386-390. doi: 10.5692/clinicalneurol.cn-001713. Epub 2022 Apr 26.
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.
一名61岁男性，在两年内曾发生两次全身性惊厥发作，因过去五个月来进行性步态障碍入住我院。神经系统检查发现认知障碍、下肢痉挛、躯干共济失调以及自主神经功能障碍，包括体位性低血压、排尿困难和少汗。脑液体衰减反转恢复（FLAIR）磁共振成像（MRI）检测到脑室周围白质和半卵圆中心有高信号强度病变，有斑点状钆（Gd）强化。脊髓MRI检测到从C2至C6的颈髓长节段肿胀病变。尽管甲泼尼龙冲击治疗最初改善了症状和MRI异常表现，但两个月后临床症状和MRI异常复发，包括新出现的颈外侧柱病变，类似于自身免疫性胶质纤维酸性蛋白（GFAP）星形细胞病（GFAP-A）的病变。此时，脑脊液中检测到抗GFAPα抗体。尽管GFAP-A的临床病程已有充分报道，但本例呈现慢性难治性病程。
