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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 electrophysiological characteristics of stiff-person syndrome. Methods The clinical data of 3 patients were analyzed, and the clinical manifestations, electrophysiological characteristics, treatment and prognosis of the disease were described in combination with the literature. Results Stiff human syndrome characterized by progressive muscle stiffness and muscle spasm, easily induced by internal and external stimuli, appear when awake, and reduce or disappear after falling asleep. EMG are characterized by sustained performance units issued. Stiff-limb syndrome is a variant of stiff-person syndrome, manifested as distal limb stiffness and painful spasms, trunk relatively less involved. Patients with stiff-person syndrome have glutamic acid decarboxylase antibodies in the blood and cerebrospinal fluid. Conclusion Stiff syndrome is an autoimmune disease. Diagnosis of stiff human syndrome need to be combined with clinical manifestations and characteristic EMG changes. With gamma globulin and stability drugs effective.