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stiff-man syndrome
Etiology:
- autoimmune disease (suspected)
- associated neoplasms
- breast cancer
- lung cancer
- thymoma
Epidemiology: rare
Clinical manifestations:
1) recurrent & uncontrollable contractions of proximal limb muscles following slight provocation
2) the muscle spasms are extremely painful
3) limbs are rigid & immobile during a spasm
Laboratory:
1) antibodies against pancreatic islet cells found in high titer
2) antibodies against AMPH
3) CRMP-5 antibody
Management:
1) diazepam
2) baclofen
3) phenytoin
4) clonidine
5) tizanidine
6) intravenous immune globulin
7) physical therapy
Related
anti-islet cell antibody (ICA)
General
paraneoplastic syndrome
References
- Mayo Internal Medicine Board Review, 1998-99, Prakash UBS (ed)
Lippincott-Raven, Philadelphia, 1998, pg 651
- Medical Knowledge Self Assessment Program (MKSAP) 11, 16.
American College of Physicians, Philadelphia 1998, 2012
- NINDS Stiff-Person Syndrome Information Page
https://www.ninds.nih.gov/Disorders/All-Disorders/Stiff-Person-Syndrome-Information-Page