[Update on chorea]

Rev Neurol. 2009 Jan 23:48 Suppl 1:S11-6.
[Article in Spanish]

Abstract

Introduction: Chorea is a brief, random, involuntary movement that can affect any muscle group and flows in an unpredictable manner from one part of the body to another. Choreic movements are present at rest, increase with distracting manoeuvres, can be partially suppressed and often disappear during sleep. The differential diagnosis of choreic syndromes is very wide and includes both genetic and acquired causes (cerebrovascular disease and space-occupying lesions, immune-based choreas, choreas caused by infection, toxic and metabolic encephalopathies, and choreas induced by pharmaceuticals).

Development: We review the phenomenology and pathophysiology of choreic movements, the most frequent causes, the diagnostic process and the most important recent findings in its treatment.

Conclusions: Huntington's disease is the most frequent cause of hereditary chorea. In patients with a familial picture suggesting Huntington's disease, but with a negative genetic study, the chances of reaching a definitive diagnosis are, today, very low. Medication is the most frequent cause of acquired chorea. When dealing with a case of hemichorea, it is essential to rule out structural insult of the basal ganglia or their connections. Treatment of choreas must be aimed at correcting their cause if it is reversible, although the patient must always be given symptomatic treatment if the intensity and functional repercussions of the chorea require it. In cases of hereditary choreas, prevention could involve genetic counselling aimed at affected patients and their relatives.

Publication types

  • English Abstract
  • Review

MeSH terms

  • Basal Ganglia / anatomy & histology
  • Basal Ganglia / physiology
  • Chorea / etiology*
  • Chorea / genetics*
  • Chorea / physiopathology*
  • Chorea / therapy
  • Diagnosis, Differential
  • Dopamine Agents / therapeutic use
  • Genetic Counseling
  • Humans
  • Huntington Disease / genetics
  • Huntington Disease / physiopathology
  • Neurosurgical Procedures

Substances

  • Dopamine Agents