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Type: Journal article
Title: Stiff man syndrome and related conditions
Author: Meinck, H.
Thompson, P.
Citation: Movement Disorders, 2002; 17(5):853-866
Publisher: John Wiley & Sons, Inc., Journals
Issue Date: 2002
ISSN: 0885-3185
Statement of
Hans-Michael Meinck, Philip D. Thompson
Abstract: The stiff man syndrome (SMS) and its variants, focal SMS, stiff limb (or leg) syndrome (SLS), jerking SMS, and progressive encephalomyelitis with rigidity and myoclonus (PERM), appear to occur more frequently than hitherto thought. A characteristic ensemble of symptoms and signs allows a tentative clinical diagnosis. Supportive ancillary findings include (1) the demonstration of continuous muscle activity in trunk and proximal limb muscles despite attempted relaxation, (2) enhanced exteroceptive reflexes, and (3) antibodies to glutamic acid decarboxylase (GAD) in both serum and spinal fluid. Antibodies to GAD are not diagnostic or specific for SMS and the role of these autoantibodies in the pathogenesis of SMS/SLS/PERM is the subject of debate and difficult to reconcile on the basis of our present knowledge. Nevertheless, evidence is emerging to suggest that SMS/SLS/PERM are manifestations of an immune-mediated chronic encephalomyelitis and immunomodulation is an effective therapeutic approach.
Keywords: Upper Extremity
Muscle, Skeletal
Muscle Rigidity
Stiff-Person Syndrome
Glutamate Decarboxylase
Diagnosis, Differential
Middle Aged
Description: Copyright © 2002 Movement Disorders Society Published in Movement Disorders, 2002; 17 (5):853-866 at
DOI: 10.1002/mds.10279
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