Nakamura Kumiko, Murao Kohei, Kimoto-Shirakawa Michiyo, Takahira Kazuyo, Toorabally Farah, Shingu Koh
Masui. 2016 Feb;65(2):164-7.
The stiff-person syndrome (SPS) is a rare autoimmune neurologic disorder that affects the gamma-aminobutyric acid (GABA) mediated inhibitory network in the central nervous system with anti-glutamic acid decarboxylase (GAD) antibodies. SPS is characterized by muscle rigidity and painful episodic spasms in axial and lower limb muscles. This case report describes successful peri-operative management of a 61-year-old female (height, 158 cm; weight, 60 kg, ASA-PS 2) with her right upper arm fracture who was scheduled for open reduction and internal fixation. This patient had bulbar paralysis, dysphagia and muscle rigidity associated with a high titer of anti-GAD auto antibodies (2,800 U x ml(-1)). She was diagnosed as SPS and has been treated with predonisolone (30 mg x day(-1)) and diazepam (20 mg x day(-1)) for 1 year. Predonisolone (15 mg) and diazepam (30 mg) was given orally before induction of general anesthesia with propofol, remifentanil and rocuronium bromide. Posture change from supine to beach-chair position led to sudden drop in blood pressure to 38/25 mmHg, which recovered promptly by injecting intravenous ephedrine hydrochloride (28 mg) and hydrocortisone (100 mg). Postanesthetic course was uneventful without postoperative neurologic abnormalities.
僵人综合征（SPS）是一种罕见的自身免疫性神经系统疾病，它通过抗谷氨酸脱羧酶（GAD）抗体影响中枢神经系统中γ-氨基丁酸（GABA）介导的抑制网络。SPS的特征是轴向和下肢肌肉出现肌肉僵硬和疼痛性发作性痉挛。本病例报告描述了一名61岁女性（身高158厘米；体重60千克，美国麻醉医师协会身体状况分级2级），因右肱骨骨折计划行切开复位内固定术，其围手术期的成功管理。该患者患有球麻痹、吞咽困难和肌肉僵硬，伴有高滴度的抗GAD自身抗体（2800 U/ml）。她被诊断为SPS，并已接受泼尼松龙（30毫克/天）和地西泮（20毫克/天）治疗1年。在使用丙泊酚、瑞芬太尼和罗库溴铵进行全身麻醉诱导前，口服泼尼松龙（15毫克）和地西泮（30毫克）。从仰卧位变为沙滩椅位导致血压突然降至38/25 mmHg，通过静脉注射盐酸麻黄碱（28毫克）和氢化可的松（100毫克）后迅速恢复。麻醉后过程顺利，无术后神经功能异常。
