Adult-onset generalized dystonia due to a mutation in the neuroferritinopathy gene

Mov Disord. 2005 Feb;20(2):243-5. doi: 10.1002/mds.20280.

Abstract

Neuroferritinopathy is a recently recognized autosomal dominant disorder that results in abnormal aggregates of iron and ferritin in the brain due to a mutation in the ferritin light chain gene on chromosome 19q13.3. We present the clinical details of a patient with adult-onset generalized dystonia associated with this mutation. Neuroferritinopathy appears to be a rare disorder; hence, there is a need to report new cases to further our understanding of the clinical phenotype, diagnostic challenges, the course of the condition and imaging characteristics.

MeSH terms

  • Adult
  • Basal Ganglia / pathology
  • Chromosome Disorders / genetics
  • DNA Mutational Analysis / methods
  • Dystonic Disorders / blood
  • Dystonic Disorders / genetics*
  • Dystonic Disorders / pathology
  • Ferritins / genetics*
  • Humans
  • Magnetic Resonance Imaging / methods
  • Male
  • Mutation*
  • Protein Subunits / genetics*

Substances

  • Protein Subunits
  • Ferritins