The stiff-person syndrome: A brief review and report of two cases

Zsuzsanna Kemény, Patrícia Pálfi, Judit Demeter, Gyula Poór, Emese Kiss, Péter Bálint

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Abstract

The stiff-person syndrome is a rare and progressive neuromuscular disease which appears to have an immunpathological basis. It is characterised by painful muscle spasms and stiffness in the proximal muscles, especially those attached to the axial skeleton. The precise pathophysiology is still unknown, but several antibodies have been shown to be present in patients and these antibodies are directed against proteins which play a role in the inhibitor synapse linked to gamma-amino-butyrate. In the first part of the article the authors present two cases. In the second part they present a comprehensive review of our current knowledge about this rare disease. Orv.Hetil., 154(50), 1984-1990.

Original languageEnglish
Pages (from-to)1984-1990
Number of pages7
JournalOrvosi hetilap
Volume154
Issue number50
DOIs
Publication statusPublished - Dec 1 2013

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Keywords

  • Amphyphysin
  • GABARAP
  • GAD
  • Gephyrin
  • Stiff-person syndrome

ASJC Scopus subject areas

  • Medicine(all)

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