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Stiff person syndrome
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Stiff person syndrome

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Contributors: Andrea Wasilewski MD, Jamie Adams MD, Richard L. Barbano MD, PhD
Other Resources UpToDate PubMed

Synopsis

Stiff person syndrome is a rare autoimmune or paraneoplastic disorder characterized by progressive muscle stiffness and spasms. It can be associated with significantly elevated levels of anti-glutamic acid decarboxylase (anti-GAD) antibodies. Average age of onset is between 20 and 50 years old. Onset in childhood is rare. Women are affected 2-3 times more often than men.

Patients present with nonspecific back pain or axial stiffness with or without episodes of severe spasms that resolve over hours to days. Some patients may only present with stiffness in the extremities or facial muscles. Spasms can be precipitated by stress, anxiety, startle responses, stimulation, or emotion. There can be abdominal contractions as well as significant lumbar lordosis. The disorder is associated with diabetes mellitus type 1 and thyroid disease; it rarely occurs as a paraneoplastic syndrome. Prognosis is variable.

For more information, see OMIM.

Codes

ICD10CM:
G25.82 – Stiff-man syndrome

SNOMEDCT:
5217008 – Stiff-man syndrome

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Last Reviewed: 02/25/2019
Last Updated: 02/25/2019
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Stiff person syndrome
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Stiff person syndrome : Gait disturbance, Lumbar lordosis, Muscle spasm, Limb pain, Myalgia, Axial rigidity
Copyright © 2019 VisualDx®. All rights reserved.