The differential diagnosis of chorea

Pract Neurol. 2007 Nov;7(6):360-73. doi: 10.1136/pn.2007.134585.


Chorea is a hyperkinetic movement disorder characterised by excessive spontaneous movements that are irregularly timed, randomly distributed and abrupt. In this article, the authors discuss the causes of chorea, particularly Huntington's disease and the genetic syndromes that may resemble it, including HDL1-3, inherited prion disease, spinocerebellar ataxias 1, 3 and 17, neuroacanthocytosis, dentatorubro-pallidoluysian atrophy (DRPLA), brain iron accumulation disorders, Wilson's disease, benign hereditary chorea, Friedreich's ataxia and mitochondrial disease. Acquired causes of chorea include vascular disease, post-infective autoimmune central nervous system disorders (PANDAS), drugs, systemic lupus erythematosus, antiphospholipid syndrome, thyrotoxicosis, AIDS, chorea gravidarum, and polycythaemia rubra vera. The authors suggest an approach to the clinical assessment of chorea, the value of investigations, including genetic tests (for which they offer a structured framework highlighting the importance of prior counselling), and finally briefly discuss symptomatic drug treatment of chorea.

Publication types

  • Review

MeSH terms

  • Anti-Dyskinesia Agents / therapeutic use
  • Autoimmune Diseases of the Nervous System / complications
  • Autoimmune Diseases of the Nervous System / microbiology
  • Autoimmune Diseases of the Nervous System / physiopathology
  • Basal Ganglia Diseases / diagnosis*
  • Basal Ganglia Diseases / etiology
  • Basal Ganglia Diseases / physiopathology*
  • Brain Diseases, Metabolic / complications
  • Brain Diseases, Metabolic / genetics
  • Brain Diseases, Metabolic / physiopathology
  • Chorea / diagnosis*
  • Chorea / etiology
  • Chorea / physiopathology*
  • Diagnosis, Differential
  • Genetic Counseling / standards
  • Genetic Predisposition to Disease / genetics*
  • Humans
  • Huntington Disease / diagnosis
  • Huntington Disease / genetics
  • Huntington Disease / physiopathology
  • Neurologic Examination / standards


  • Anti-Dyskinesia Agents