Apraxia in anti-glutamic acid decarboxylase-associated stiff person syndrome: link to corticobasal degeneration?

Ann Neurol. 2015 Jan;77(1):173-6. doi: 10.1002/ana.24245. Epub 2014 Dec 8.

Abstract

Corticobasal syndrome (CBS) is associated with asymmetrical rigidity as well as asymmetrical limb-kinetic and ideomotor apraxia. Stiff person syndrome (SPS) is characterized by muscle stiffness and gait difficulties. Whereas patients with CBS have several forms of pathology, many patients with SPS have glutamic acid decarboxylase antibodies (GAD-ab), but these 2 disorders have not been reported to coexist. We report 2 patients with GAD-ab-positive SPS who also had signs suggestive of CBS, including asymmetrical limb rigidity associated with both asymmetrical limb-kinetic and ideomotor apraxia. Future studies should evaluate patients with CBS for GAD-ab and people with SPS for signs of CBS.

Publication types

  • Case Reports

MeSH terms

  • Aged
  • Apraxias / etiology*
  • Autoantibodies / blood*
  • Female
  • Glutamate Decarboxylase / immunology*
  • Humans
  • Middle Aged
  • Neurodegenerative Diseases / complications
  • Stiff-Person Syndrome / complications*
  • Stiff-Person Syndrome / etiology
  • Stiff-Person Syndrome / immunology*

Substances

  • Autoantibodies
  • Glutamate Decarboxylase