僵人综合征的临床及电生理特点

Clinical and eletrophysiological characteristics of stiff-person syndrome

目的探讨僵人综合征的临床及电生理特点。方法分析3例患者的临床资料,并结合文献阐述该病的临床表现、电生理特点、治疗及预后。结果僵人综合征以进行性波动的肌肉僵直和肌痉挛为特点,易由内外刺激诱发,醒时出现,入睡后减轻或消失。肌电图均特征性地表现为持续性运动单位发放。僵肢综合征为僵人综合征的变异型,多表现为肢体远端强直及痛性痉挛,躯干相对较少累及。僵人综合征患者血和脑脊液中存在谷氨酸脱羧酶抗体。结论僵人综合征是一种自身免疫疾病。诊断僵人综合征需结合临床表现及特征性的肌电图改变。用丙种球蛋白及安定类药物治疗有效。

Objective To investigate the clinical and electrophysiologica l characteristics of stiff- person syndrome(SPS). Methods The clinical man ifestations,electrophysiologic features, treatment and prognosis of 3 cases of S PS were analyzed and the related literatures were also reviewed. Results SP S was characterized by progressive muscular rigidity and muscular spasm which co uld easily be evoked by intrinsic or peripheral stimuli. Both the rigidity and t he spasms were relieved or disappeared by sleep and emerged by wakefulness. EMG presented continuous motor unit activity. Anti-glutamic acid decarboxylase (anti -GAD) antibodies in blood and cerebral spinal fluid could be founded. Stiff limb syndrome was one of variations of SPS, whose clinical picture was dominated by rigidity and painful spasms of the distal limbs associated with relatively less involved in the trunk. We now think SPS as an autoimmune disorder. Conclusion The SPS can be diagnosed by clinical manifestations and electromyography. Th e immunology features of patients were abnormal and the treatment with beriglobi n and diazepam was effective.