stiff-person syndrome
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Etiology
- autoimmune disease (suspected)
- associated neoplasms
Epidemiology
rare
Clinical manifestations
- recurrent & uncontrollable contractions of proximal limb muscles following slight provocation
- the muscle spasms are extremely painful
- limbs are rigid & immobile during a spasm
Laboratory
- antibodies against pancreatic islet cells found in high titer
- antibodies against AMPH
- CRMP-5 antibody
- glutamic acid decarboxylase Ab[2]
Management
- diazepam
- baclofen
- phenytoin
- clonidine
- tizanidine
- intravenous immune globulin
- mivocabtagene autoleucel (investigational 2016)[3]
- physical therapy
More general terms
Additional terms
References
- ↑ Mayo Internal Medicine Board Review, 1998-99, Prakash UBS (ed) Lippincott-Raven, Philadelphia, 1998, pg 651
- ↑ 2.0 2.1 Medical Knowledge Self Assessment Program (MKSAP) 11, 16. American College of Physicians, Philadelphia 1998, 2012
Medical Knowledge Self Assessment Program (MKSAP) 20 American College of Physicians, Philadelphia 2025 - ↑ 3.0 3.1 George J Stiff Person Syndrome Patients Regain Mobility After Single-Dose Cell Therapy. CAR-T therapy shows promise in clinical trial. MedPage Today. April 23, 2026 https://www.medpagetoday.com/meetingcoverage/aan/120926
- ↑ NINDS Stiff-Person Syndrome Information Page https://www.ninds.nih.gov/Disorders/All-Disorders/Stiff-Person-Syndrome-Information-Page