title: [Genetics in movement disorders--dystonia, tremor and chorea].
abstract: BACKGROUND
  Several dystonias, Huntington's disease, essential tremor and various rare conditions are among the hereditary movement disorders, disorders with considerable genetic and clinical heterogeneity. It may be hoped that better understanding of the genetics and pathogenetic mechanisms involved will improve management.
  
  
  METHODS
  This review is based on personal experience and recent literature.
  
  
  RESULTS
  At least 13 types of dystonias are genetically defined. DYT 1 seems to be the most important, and is easily tested. The Huntington gene is readily available for symptomatic as well as presymptomatic testing. Presymptomatic testing gives rise to ethical concerns and require thorough genetic counselling. Also essential tremor seems to show an autosomal dominant inheritance, but the responsible gene(s) has not been identified.
  
  
  CONCLUSIONS
  As for other movement disorders, involvement of different genes may give similar phenotypes and defects in a single gene give rise to various different phenotypes.
authors.affiliation: Nevrologisk avdeling, Rikshospitalet, 0027 Oslo. espen.dietrichs@klinmed.uio.no
authors.forename: Espen
authors.initials: E
authors.lastname: Dietrichs
authors.forename: Chantal M E
authors.initials: CM
authors.lastname: Tallaksen
mesh_terms: D020821:Dystonic Disorders / Q000175:diagnosis / Q000235:genetics* / Q000628:therapy; D020329:Essential Tremor / Q000175:diagnosis / Q000235:genetics* / Q000628:therapy; D005817:Genetic Counseling; D006801:Humans; D006816:Huntington Disease / Q000175:diagnosis / Q000188:drug therapy / Q000235:genetics*; D010641:Phenotype