The Stiff People: Two Rare Cases of Stiff-person Syndrome
- PMID: 32175215
- PMCID: PMC7053798
- DOI: 10.7759/cureus.1602
The Stiff People: Two Rare Cases of Stiff-person Syndrome
Abstract
Stiff-person syndrome (SPS) is a rare disorder that affects the central nervous system and is characterized by progressive muscle stiffness, rigidity, and spasm of axial and limb muscles. The syndrome is caused by a lack of gamma aminobutyric acid (GABA), which occurs because of antibodies against glutamic acid decarboxylase (GAD), an essential enzyme for GABA synthesis. Hence, the patients present with increased muscular activity. In this article, we will discuss two case studies of stiff-person syndrome.
Keywords: gaba; gad; gamma butyric amino acid; glutamic acid decarboxylase; sps; stiff-man syndrome; stiff-person syndrome.
Copyright © 2017, Siddiqui et al.
Conflict of interest statement
The authors have declared that no competing interests exist.
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References
-
- Stiff-man syndrome: from the bedside to the bench. Helfgott SM. Arthritis Rheum. 1999;42:1312–1320. - PubMed
-
- Stiff-man syndrome and its variants. Shaw PJ. Lancet. 1999;353:86–87. - PubMed
-
- The "stiff-man" syndrome and hyperthyroidism. Werk EE Jr, Sholiton LJ, Marnell RT. Am J Med. 1961;31:647–653. - PubMed
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