STIFF-PERSON SYNDROME - AN AUTOIMMUNE-DISEASE

被引：80

作者：

BLUM, P ^{[1
]}

JANKOVIC, J ^{[1
]}

机构：

[1] BAYLOR UNIV,DEPT NEUROL,6550 FANNIN 1801,HOUSTON,TX 77030

来源：

MOVEMENT DISORDERS | 1991年 / 6卷 / 01期

关键词：

STIFF-PERSON SYNDROME; AUTOIMMUNE DISEASES; CORTICOSTEROID DRUGS;

D O I：

10.1002/mds.870060104

中图分类号：

R74 [神经病学与精神病学];

学科分类号：

摘要：

Stiff-person syndrome (SPS) is characterized by progressive, usually symmetric rigidity of the axial muscles with superimposed painful spasms precipitated by tactile stimuli, passive stretch, volitional movement of affected or unaffected muscles, startling noises, and emotional stimuli. electromyography demonstrates continuous normal motor unit potentials in the affected muscles. Both the rigidity and the spasms are relieved by sleep, general anesthesia, myoneural blockade, peripheral nerve blockade, and partially by diazepam. Evidence for an autoimmune etiology of SPS includes its association with other autoimmune diseases and autoantibodies and the presence of antibodies against glutamic acid decarboxylase (GAD) in the cerebrospinal fluid (CSF) of many affected patients. We describe two patients with this syndrome who had GAD antibodies in both CSF and serum. Partial relief of the symptoms in these patients by corticosteroid therapy provides additional evidence of an autoimmune etiology of SPS and of the role of immunotherapy in its treatment.

引用

页码：12 / 20

页数：9

共 40 条

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