Nakamagoe K, Ohkoshi N, Hayashi A, Hisahara S, Shoji S
Department of Neurology, University of Tsukuba.
Rinsho Shinkeigaku. 1995 Aug;35(8):897-900.
We described a 56-year-old man with stiff-man syndrome, who was markedly improved after plasmapheresis therapy. He had a 12-year history of progressive painful stiffness of his back and limbs, muscle cramps and difficulty in walking. He had been taking oral diazepam and prednisolone. On examination the abdominal and paraspinal muscles and limbs were continuously contracting, confirmed by surface and needle electromyography. Antibodies against glutamic acid decarboxylase (GAD) and pancreatic islet cells in the serum were negative, but antinuclear antibody and anti-smooth-muscle antibody were present. The patient underwent a course of 4 double filtration plasma exchanges of 3,000 ml each in an 8-day period. Plasmapheresis resulted in marked clinical improvement. The disappearance of muscular cramps and a reduction of stiffness occurred within 24 hours after the first plasmapheresis, and he was able to walk unassisted. The patient's subjective improvement continued over 4 months after the plasma exchange. This case provides additional evidence of the autoimmune mechanism of stiff-man syndrome. Plasmapheresis is one choice in the management for stiff-man syndrome.
我们描述了一名患有僵人综合征的56岁男性，其在接受血浆置换治疗后有明显改善。他有12年的背部和四肢进行性疼痛僵硬、肌肉痉挛及行走困难病史。他一直在口服地西泮和泼尼松龙。检查时发现腹部、椎旁肌肉及四肢持续收缩，表面肌电图和针极肌电图证实了这一点。血清中谷氨酸脱羧酶（GAD）抗体和胰岛细胞抗体均为阴性，但存在抗核抗体和抗平滑肌抗体。该患者在8天内进行了4个疗程的双重过滤血浆置换，每次3000毫升。血浆置换使临床症状明显改善。首次血浆置换后24小时内肌肉痉挛消失，僵硬程度减轻，他能够独立行走。血浆置换后4个月患者的主观症状持续改善。该病例为僵人综合征的自身免疫机制提供了更多证据。血浆置换是僵人综合征治疗的一种选择。
