Movement Disorders (revue)

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Stiff‐person syndrome: An autoimmune disease

Identifieur interne : 006122 ( Main/Exploration ); précédent : 006121; suivant : 006123

Stiff‐person syndrome: An autoimmune disease

Auteurs : Philip Blum [États-Unis] ; Jankovic [États-Unis]

Source :

RBID : ISTEX:46A51ABDA48DA09DE29E44CF9E7A34B0BD04394C

Descripteurs français

English descriptors

Abstract

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.

Url:
DOI: 10.1002/mds.870060104


Affiliations:


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Le document en format XML

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<div type="abstract" xml:lang="en">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.</div>
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