Stiff-person syndrome

Research output: Contribution to journalArticle

Abstract

Background: Stiff-person syndrome (SPS) is a rare neurologic disorder with autoimmune features. It is characterized by progressive, severe muscle rigidity or stiffness most prominently affecting the spine and lower extremities. Review Summary: Superimposed muscle spasms result in simultaneous contraction of agonist and antagonist muscles which are detectable by electromyography (EMG) and relieved by administration of benzodiazepines. The exacerbation of SPS by emotional stressors often results in the referral of these patients for psychiatric assessment although this was more common before the discovery of an association with antibodies to glutamic acid decarboxylase (GAD antibodies). Formerly known as stiff-man syndrome, the female to male ratio is 2:1 and the principle paraneoplastic variant is associated with breast cancer. Although rare, this is a disease of middle age that severely curtails the functional capacity of those it strikes. It is frequently associated with diabetes and other autoimmune diseases. IVIg is recently demonstrated to be effective in the treatment of SPS; diazepam remains useful in managing the symptoms. Conclusions: This article summarizes the history of SPS, describes important clinical features, discusses management, touches upon areas of uncertainty, and postulates some avenues for research.

Original languageEnglish (US)
Pages (from-to)131-137
Number of pages7
JournalNeurologist
Volume10
Issue number3
DOIs
StatePublished - May 2004

Fingerprint

Stiff-Person Syndrome
Muscle Rigidity
Glutamate Decarboxylase
Antibodies
Spasm
Electromyography
Diazepam
Nervous System Diseases
Benzodiazepines
Autoimmune Diseases
Uncertainty
Psychiatry
Lower Extremity
Spine
Referral and Consultation
History
Breast Neoplasms
Muscles
Research

Keywords

  • Amphiphysin
  • Gephyrin
  • Glutamic acid decarboxylase
  • Stiff-person syndrome

ASJC Scopus subject areas

  • Clinical Neurology

Cite this

Stiff-person syndrome. / Murinson, Beth B.

In: Neurologist, Vol. 10, No. 3, 05.2004, p. 131-137.

Research output: Contribution to journalArticle

Murinson, Beth B. / Stiff-person syndrome. In: Neurologist. 2004 ; Vol. 10, No. 3. pp. 131-137.
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