Stiff-person syndrome following West Nile fever

Arch Neurol. 2004 Jun;61(6):938-41. doi: 10.1001/archneur.61.6.938.

Abstract

Background: Stiff-person syndrome is a rare autoimmune disorder associated with antibodies against glutamic acid decarboxylase (GAD), the key enzyme in gamma-aminobutyric acid synthesis. In most cases, a trigger cannot be identified.

Objective: To describe a 41-year-old man who developed stiff-person syndrome and antibodies to GAD following acute West Nile virus infection.

Design: A case report and a search in GenBank for common epitopes.

Result: The search revealed a stretch of 12 amino acids in the NS1 protein of West Nile virus with a high degree of homology to the GAD65 region (an isoform of GAD) containing the PEVKEK motif.

Conclusion: Cross-reactivity between antibodies directed against West Nile virus and GAD may have contributed to the development of stiff-person syndrome in this patient.

Publication types

  • Case Reports

MeSH terms

  • Adult
  • Amino Acid Sequence
  • Autoantibodies / blood
  • Glutamate Decarboxylase / chemistry
  • Glutamate Decarboxylase / immunology
  • Humans
  • Isoenzymes / chemistry
  • Isoenzymes / immunology
  • Male
  • Molecular Sequence Data
  • Sequence Homology, Amino Acid
  • Stiff-Person Syndrome / etiology*
  • Stiff-Person Syndrome / genetics
  • Stiff-Person Syndrome / immunology
  • Viral Nonstructural Proteins / chemistry
  • West Nile Fever / complications*
  • West Nile virus

Substances

  • Autoantibodies
  • Isoenzymes
  • NS1 protein, Flavivirus
  • Viral Nonstructural Proteins
  • Glutamate Decarboxylase
  • glutamate decarboxylase 2