1991
DOI: 10.1002/mds.870060104
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Stiff‐person syndrome: An autoimmune disease

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 parti… Show more

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Cited by 123 publications
(54 citation statements)
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“…Interestingly, the amino acid sequence of the GAD67 ofCram most closely resembles rat brain GAD67, with differences at only three positions (6,10,41) The human GAD cDNAs we report here should allow the production of antigens for the routine detection of GAD autoantibodies. The presence of GAD autoantibodies is the earliest known predictor of IDDM and is also diagnostic of stiff-man syndrome (13,14,39,(44)(45)(46)(47). While bacterially produced GADs have so far proved immunologically identical to pancreatic and brain GADs, further work will be necessary to learn how islet cell GADs may be modified posttranslationally and how they become associated with the synapse-like vesicles within /8 cells (39,48).…”
Section: Discussionmentioning
confidence: 99%
“…Interestingly, the amino acid sequence of the GAD67 ofCram most closely resembles rat brain GAD67, with differences at only three positions (6,10,41) The human GAD cDNAs we report here should allow the production of antigens for the routine detection of GAD autoantibodies. The presence of GAD autoantibodies is the earliest known predictor of IDDM and is also diagnostic of stiff-man syndrome (13,14,39,(44)(45)(46)(47). While bacterially produced GADs have so far proved immunologically identical to pancreatic and brain GADs, further work will be necessary to learn how islet cell GADs may be modified posttranslationally and how they become associated with the synapse-like vesicles within /8 cells (39,48).…”
Section: Discussionmentioning
confidence: 99%
“…The disappearance of rigidity, spasms and continuous motor unit activity during sleep, and after peripheral nerve block, spinal or general anesthesia, favor a central origin of the disease. 2,5 The current hypothesis regarding the pathogenesis of SMS is that there is an imbalance between the catecholaminergic and GABA-ergic pathways controlling motor unit activity, probably due to impaired GABA-ergic pathways.…”
Section: Discussionmentioning
confidence: 99%
“…The etiology of the disease is unknown, although the association of SMS with diseases such as diabetes, vitiligo and hypothyroidism, and the detection of auto-antibodies against (γ-aminobutyric acid) GABA-ergic neurons in serum and cerebrospinal fluid (CSF) suggest that the disease has an autoimmune nature. [3][4][5] This is a report of a patient with stiff-man syndrome with unusual presentation, high titers of anti-GAD (glutamate acid decarboxylase) antibodies in serum and CSF, other organ-specific auto-antibodies and a favorable response to vigabatrin. …”
mentioning
confidence: 99%
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“…Consequently, the syndrome has been renamed SPS. [8,9] Several other variants have also been described, such as stiff baby syndrome and stiff limb syndrome. [10] Women appear to be affected more often than men, with a male:female ratio of 2:1.…”
mentioning
confidence: 99%