Stiff‐person syndrome: An autoimmune disease
Identifieur interne : 006122 ( Main/Exploration ); précédent : 006121; suivant : 006123Stiff‐person syndrome: An autoimmune disease
Auteurs : Philip Blum [États-Unis] ; Jankovic [États-Unis]Source :
- Movement Disorders [ 0885-3185 ] ; 1991.
Descripteurs français
- Pascal (Inist)
- Wicri :
English descriptors
- KwdEn :
- Antibody, Autoantibodies (analysis), Autoimmune Diseases (diagnosis), Autoimmune Diseases (drug therapy), Autoimmune Diseases (immunology), Autoimmune disease, Autoimmune diseases, Baclofen (administration & dosage), Case study, Cerebrospinal fluid, Chemotherapy, Corticosteroid, Corticosteroid drugs, Diazepam (administration & dosage), Drug Administration Schedule, Drug Therapy, Combination, Enzyme, Exploration, Female, Glutamate Decarboxylase (immunology), Glutamate decarboxylase, Human, Humans, Immunological investigation, Male, Middle Aged, Muscle Rigidity (diagnosis), Muscle Rigidity (drug therapy), Muscle Rigidity (immunology), Muscle Tonus (drug effects), Nervous system diseases, Neurologic Examination, Prednisone (administration & dosage), Serum, Stiff man syndrome, Stiff‐person syndrome, Striated muscle disease, Treatment.
- MESH :
- chemical , administration & dosage : Baclofen, Diazepam, Prednisone.
- chemical , analysis : Autoantibodies.
- diagnosis : Autoimmune Diseases, Muscle Rigidity.
- drug effects : Muscle Tonus.
- drug therapy : Autoimmune Diseases, Muscle Rigidity.
- immunology : Autoimmune Diseases, Glutamate Decarboxylase, Muscle Rigidity.
- Drug Administration Schedule, Drug Therapy, Combination, Female, Humans, Male, Middle Aged, Neurologic Examination.
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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<term>Autoimmune Diseases (immunology)</term>
<term>Autoimmune disease</term>
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<term>Muscle Rigidity (drug therapy)</term>
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<front><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>
</front>
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