Immunobiology of stiff-person syndrome

Raghavan Pillai Raju; Christiane S. Hampe

doi:10.1080/08830180701883240

Immunobiology of stiff-person syndrome

Raghavan Pillai Raju, Christiane S. Hampe

Research output: Contribution to journal › Review article

22 Scopus citations

Abstract

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 language	English (US)
Pages (from-to)	79-92
Number of pages	14
Journal	International Reviews of Immunology
Volume	27
Issue number	1-2
DOIs	https://doi.org/10.1080/08830180701883240
State	Published - Jan 1 2008
Externally published	Yes

Keywords

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

ASJC Scopus subject areas

Immunology

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Raju, R. P., & Hampe, C. S. (2008). Immunobiology of stiff-person syndrome. International Reviews of Immunology, 27(1-2), 79-92. https://doi.org/10.1080/08830180701883240

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abstract = "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.",

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