Stiff person syndrome with cerebellar disease and high-titer anti-GAD antibodies

Neurology. 2006 Sep 26;67(6):1068-70. doi: 10.1212/01.wnl.0000237558.83349.d0.

Abstract

We report five of 38 patients with stiff person syndrome (SPS), who also had cerebellar disease, gait ataxia, dysarthria, and oculomotor dysfunction (SPS-Cer). Cerebellar manifestations either preceded SPS or occurred concurrently. Brain MRI was normal. The intrathecal production of glutamic acid decarboxylase antibodies was elevated. Gamma-aminobutyric acid-enhancing drugs and immunotherapies improved only the stiffness. SPS-Cer is a distinct subset of SPS causing a more severe and complex clinical phenotype.

Publication types

  • Case Reports
  • Comparative Study
  • Research Support, N.I.H., Intramural

MeSH terms

  • Adult
  • Aged
  • Anticonvulsants / therapeutic use
  • Autoantibodies / metabolism*
  • Cerebellar Diseases / complications*
  • Cerebellar Diseases / drug therapy
  • Cerebellar Diseases / metabolism
  • Diazepam / therapeutic use
  • Female
  • Glutamate Decarboxylase / immunology*
  • Humans
  • Magnetic Resonance Imaging / methods
  • Male
  • Middle Aged
  • Stiff-Person Syndrome / complications*
  • Stiff-Person Syndrome / drug therapy
  • Stiff-Person Syndrome / metabolism

Substances

  • Anticonvulsants
  • Autoantibodies
  • Glutamate Decarboxylase
  • Diazepam