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Stiff-Person Syndrome Spectrum Disorders

  • José Fidel Baizabal-Carvallo
  • Marlene Alonso-Juarez
Chapter
Part of the Contemporary Clinical Neuroscience book series (CCNE)

Abstract

Stiff-person syndrome was first described in 1956; its further characterization as an autoimmune neurological disorder occurred more than 30 years later with the discovery of glutamic acid decarboxylase (GAD) antibodies (Abs), frequently coexisting in these patients. In the following years, clinical variants of SPS have been characterized, and a paraneoplastic presentation was also recognized, the latter mainly associated with amphiphysin antibodies. Although the presence of GAD-Abs has led to theorize that these antibodies cause disinhibition of the central nervous system through decreased production of the inhibitory neurotransmitter (GABA), the pathogenic role of GAD-Abs has not been demonstrated, although the evidence suggests that antibodies directed against amphiphysin and glycine receptor α1 are likely pathogenic. The treatment aims to attenuate the immunological response through immunotherapy, control the symptoms, mainly with GABAergic drugs, and remove an underlying tumor, if present. The course is usually chronic and the prognosis is frequently poor.

Keywords

Stiff-man syndrome Stiff-person syndrome γ-aminobutyric acid Glutamic acid decarboxylase Progressive encephalomyelitis with rigidity and myoclonus PERM Paraneoplastic stiff-person syndrome Amphiphysin Myoclonus Glycine receptor GAD antibodies 

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Copyright information

© Springer Nature Switzerland AG 2019

Authors and Affiliations

  • José Fidel Baizabal-Carvallo
    • 1
  • Marlene Alonso-Juarez
    • 2
  1. 1.Department of Internal MedicineUniversity of GuanajuatoGuanajuatoMexico
  2. 2.National Polytechnic InstituteMexico CityMexico

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