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The differential diagnosis of chorea

E J Wild1, S J Tabrizi

  • 1Department of Neurodegenerative Disease, UCL Institute of Neurology, London/National Hospital for Neurology and Neurosurgery, Queen Square, London, UK.

Practical Neurology
|November 21, 2007
PubMed

Insights

This review covers diverse causes of chorea, a hyperkinetic movement disorder. It outlines clinical assessment strategies and diagnostic investigations, including genetic testing, for managing involuntary movements.

Area of Science:

  • Neurology
  • Genetics
  • Movement Disorders

Background:

  • Chorea is a hyperkinetic movement disorder defined by excessive, irregular, and abrupt spontaneous movements.
  • It can stem from various genetic and acquired conditions, necessitating a comprehensive diagnostic approach.

Purpose of the Study:

  • To review the diverse etiologies of chorea, encompassing genetic syndromes and acquired causes.
  • To propose a structured framework for clinical assessment and investigations, including genetic testing and counseling.
  • To briefly discuss symptomatic management of chorea.

Main Methods:

  • Literature review of genetic syndromes and acquired conditions causing chorea.
  • Discussion of clinical assessment strategies and diagnostic investigations.
  • Outline of a framework for genetic testing and counseling.
  • Brief overview of symptomatic drug treatments.

Main Results:

  • Identified numerous genetic causes (e.g., Huntington's disease, spinocerebellar ataxias) and acquired causes (e.g., vascular disease, autoimmune disorders, drugs).
  • Proposed a systematic approach to clinical evaluation and investigations.
  • Emphasized the importance of genetic counseling prior to genetic testing.

Conclusions:

  • Chorea presents with a wide spectrum of underlying causes requiring careful diagnostic evaluation.
  • A structured approach to assessment, investigation, and genetic counseling is crucial for effective management.
  • Symptomatic treatment options are available for managing chorea.

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