Immunobiology of stiff-person syndrome

Raghavan Raju, Christiane S. Hampe

Research output: Contribution to journalReview articlepeer-review

26 Scopus citations


The two possibilities to explain the pathogenic basis of stiff-person syndrome (SPS) are intrathecal sensitization of GAD65-reactive CD4+T cells and synthesis of GAD65-specific autoantibodies within the CNS [Rakocevic et al., Arch. Neurol. 61: 902-904, 2004]; and peripheral antigen sensitization followed by CNS antigen recognition by autoantibodies that cross the blood-brain barrier. Antigen-specific CD4+ T cells are essential for the generation of high-affinity autoantibodies [Lanzavecchia, Nature 314: 537-539, 1985], but there is no evidence of cellular infiltration in the CNS of SPS patients [Warich-Kirches et al., Clin. Neuropathol. 16: 214-219, 1997; Ishizawa et al., Acta Neuropathol.(Berl) 97: 63-70, 1999]. This review discusses the possible role of autoantibodies and autoreactive T cells specific to neuronal antigens in SPS pathogenesis.

Original languageEnglish (US)
Pages (from-to)79-92
Number of pages14
JournalInternational Reviews of Immunology
Issue number1-2
StatePublished - Jan 2008
Externally publishedYes


  • Autoimmunity
  • Gamma aminobutyric acid (GABA)
  • Glutamic acid decarboxylase autoantibodies
  • Stiff-person syndrome
  • T cells

ASJC Scopus subject areas

  • Immunology and Allergy
  • Immunology


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