Background: Chorea consists of involuntary movements affecting the limbs, trunk, neck or face, that can move from one body part to another. Chorea is conceptualized as being "primary" when it is attributed to Huntington's disease (HD) or other genetic etiologies, or "secondary" when it is related to infectious, pharmacologic, metabolic, autoimmune disorders, or paraneoplastic syndromes. The mainstay of the secondary chorea management is treating the underlying causative disorder; here we review the literature regarding secondary chorea. We also discuss the management of several non-HD genetic diseases in which chorea can be a feature, where metabolic targets may be amenable to intervention and chorea reduction.
Methods: A PubMed literature search was performed for articles relating to chorea and its medical and surgical management. We reviewed the articles and cross-references of pertinent articles to assess the current clinical practice, expert opinion, and evidence-based medicine to synthesize recommendations for the management of secondary chorea.
Results: There are very few double-blind randomized controlled trials assessing chorea treatments regardless of etiology. Most recommendations are based on small open-label studies, case reports, and expert opinion.
Discussion: Treatment of secondary chorea is currently based on expert opinion, clinical experience, and small case studies, with limited evidence-based medical data. When chorea is secondary to an underlying infection, medication, metabolic abnormality, autoimmune process, or paraneoplastic illness, the movements typically resolve following treatment of the underlying disease. Tardive dyskinesia is most rigorously studied secondary chorea with the best evidence-based medicine treatment guidelines recommending the use of pre-synaptic dopamine-depleting agents. Even though there is an insufficient pool of EBM, small clinical trials, case reports, and expert opinion are valuable for guiding treatment and improving the quality of life for patients with chorea.
Highlights: There is a dearth of well-controlled studies regarding the treatment of chorea. Expert opinion and clinical experiences are fundamental in guiding chorea management and determining successful treatment. In general, secondary chorea improves with treating the underlying medical abnormality; treatments include antibiotics, antivirals, immunosuppression, dopamine depleting agents, chelation, and supportive care.
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http://dx.doi.org/10.5334/tohm.351 | DOI Listing |
JCEM Case Rep
November 2024
Chicago Medical School, Rosalind Franklin University of Medicine and Science, North Chicago, IL, USA.
BMJ Case Rep
October 2024
Neurology, Northampton General Hospital NHS Trust, Northampton, Northamptonshire, UK.
Chorea is a hyperkinetic movement disorder characterised by involuntary, brief, random and irregular contractions. Acquired chorea can present acutely or subacutely and may be asymmetrical or unilateral. A detailed history and examination are crucial to identify triggering factors and underlying cause.
View Article and Find Full Text PDFJCEM Case Rep
November 2024
Department of Endocrinology, University Hospitals Leuven, 3000 Leuven, Belgium.
Neurobiol Dis
November 2024
Cente de Recherche du CHU de Québec, Axe Neurosciences, T2-07, 2705, Boulevard Laurier, Québec, QC G1V 4G2, Canada; Département de Médecine Moléculaire, Université Laval, Québec, QC, Canada; Département de Psychiatrie et Neurosciences, Université Laval, Québec, QC, Canada. Electronic address:
Pharmacol Res
November 2024
Department of Pharmacology, Dalhousie University, Halifax, Nova Scotia B3H 4R2, Canada. Electronic address:
Microglia, the resident immune cells of the brain, regulate the balance of inflammation in the central nervous system under healthy and pathogenic conditions. Huntington's disease (HD) is a chronic neurodegenerative disease characterized by activated microglia and elevated concentrations of pro-inflammatory cytokines within the brain. Chronic hyperactivation of microglia is associated with brain pathology and eventual neuron death.
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