The differential diagnosis of chorea.

Pract Neurol

Department of Neurodegenerative Disease, UCL Institute of Neurology, London/National Hospital for Neurology and Neurosurgery, Queen Square, London, UK.

Published: November 2007

AI Article Synopsis

  • Chorea is a movement disorder with irregular and excessive involuntary movements, often associated with conditions like Huntington's disease and various genetic syndromes.
  • The article explores both genetic and acquired causes of chorea, which include diseases like Wilson's, neuroacanthocytosis, and conditions like lupus and drug side effects.
  • It also recommends a clinical assessment approach, emphasizes the importance of genetic testing with prior counseling, and briefly covers treatment options for managing symptoms.

Article Abstract

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.

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http://dx.doi.org/10.1136/pn.2007.134585DOI Listing

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