Chorea in children: etiology, diagnostic approach and management

J Neural Transm (Vienna). 2020 Oct;127(10):1323-1342. doi: 10.1007/s00702-020-02238-3. Epub 2020 Aug 9.


Chorea is defined by the presence of abnormal, involuntary, continuous, random movements that results from a number of autoimmune, hereditary, vascular, metabolic, drug-induced and functional (psychogenic) causes. Chorea may present at all stages of life, from newborns to elderly individuals. While Huntington disease is the main suspicion in adults presenting with chorea, once a drug-induced or parkinsonian dyskinesia have been ruled out; Huntington disease exceptionally presents with chorea in children. Sydenham chorea is considered the most common cause of acute childhood-onset chorea, but its prevalence has decreased in Western countries. However, in younger children other etiologies such as dyskinetic cerebral palsy, anti-NMDAR receptor encephalitis, other autoimmune conditions, or mutations in NKX2-1, ADCY-5, FOXG1, GNAO1, GPR88, SLC2A1, SQSTM1, ATP8A2, or SYT-1 should be considered. In this manuscript, we review the main causes, diagnosis and management of chorea in children.

Keywords: Autoimmune; Children; Chorea; Infancy; Sydenham.

Publication types

  • Review

MeSH terms

  • Autoimmune Diseases*
  • Causality
  • Child
  • Chorea* / diagnosis
  • Chorea* / etiology
  • Chorea* / therapy
  • GTP-Binding Protein alpha Subunits, Gi-Go
  • Humans
  • Huntington Disease*
  • Mutation


  • GNAO1 protein, human
  • GTP-Binding Protein alpha Subunits, Gi-Go