Erin Feinstein1, Ruth Walker2. 1. Department of Neurology, Rutgers New Jersey Medical School, 90 Bergen St, suite 5200, Newark, NJ, 07103, USA. Erin.Feinstein@njms.rutgers.edu. 2. Veterans Affairs Medical Center, Department of Neurology, Bronx Mount Sinai School of Medicine, New York, NY, 10029, USA.
Abstract
PURPOSE OF REVIEW: There are many causes for chorea, including genetic, autoimmune, pharmacological, and structural lesions. Where appropriate, treatment is based on reversing the underlying cause of chorea; many cases are self-limited, resolving when the primary disorder is treated. This review focuses on the management of chorea due to untreatable causes. RECENT FINDINGS: There are a limited number of double-blind randomized control trials assessing the efficacy of specific chorea treatments. Most therapeutic recommendations are based on small open-label studies, case reports, and expert opinion. This is in part due to the heterogeneity of chorea and chorea-associated syndromes and the variety of neurodegenerative phenotypes with variable progression rates. Chorea can be treated with a variety of medications ranging from antiepileptics to antipsychotics. The recent development of selective vesicular monoamine transporter blocking agents has allowed for targeted chorea management with minimal side effects. Neurosurgical interventions such as deep brain surgery (DBS) and pallidotomy are reserved for medication-refractory chorea. As a symptom of neurodegenerative disease, chorea is only one aspect of the basal ganglia syndromes, and often, a multidisciplinary approach tailored to individual patient needs provides the best management.
PURPOSE OF REVIEW: There are many causes for chorea, including genetic, autoimmune, pharmacological, and structural lesions. Where appropriate, treatment is based on reversing the underlying cause of chorea; many cases are self-limited, resolving when the primary disorder is treated. This review focuses on the management of chorea due to untreatable causes. RECENT FINDINGS: There are a limited number of double-blind randomized control trials assessing the efficacy of specific chorea treatments. Most therapeutic recommendations are based on small open-label studies, case reports, and expert opinion. This is in part due to the heterogeneity of chorea and chorea-associated syndromes and the variety of neurodegenerative phenotypes with variable progression rates. Chorea can be treated with a variety of medications ranging from antiepileptics to antipsychotics. The recent development of selective vesicular monoamine transporter blocking agents has allowed for targeted chorea management with minimal side effects. Neurosurgical interventions such as deep brain surgery (DBS) and pallidotomy are reserved for medication-refractory chorea. As a symptom of neurodegenerative disease, chorea is only one aspect of the basal ganglia syndromes, and often, a multidisciplinary approach tailored to individual patient needs provides the best management.
Entities:
Keywords:
Chorea; Deep brain stimulation; Deutetrabenazine; Valbenazine
Authors: Michael S Okun; Michele Tagliati; Michael Pourfar; Hubert H Fernandez; Ramon L Rodriguez; Ron L Alterman; Kelly D Foote Journal: Arch Neurol Date: 2005-06-13
Authors: Frances M Velez-Lago; Amanda Thompson; Genko Oyama; Angela Hardwick; Justin M Sporrer; Pamela Zeilman; Kelly D Foote; Dawn Bowers; Herbert E Ward; Juan Sanchez-Ramos; Michael S Okun Journal: Stereotact Funct Neurosurg Date: 2013-01-22 Impact factor: 1.875
Authors: G Bernhard Landwehrmeyer; Bruno Dubois; Justo Garcia de Yébenes; Berry Kremer; Wilhelm Gaus; Peter H Kraus; Horst Przuntek; Michel Dib; Adam Doble; Wilhelm Fischer; Albert C Ludolph Journal: Ann Neurol Date: 2007-09 Impact factor: 10.422
Authors: S Zittel; V Tadic; C K E Moll; T Bäumer; A Fellbrich; A Gulberti; D Rasche; N Brüggemann; V Tronnier; A Münchau Journal: Parkinsonism Relat Disord Date: 2018-02-20 Impact factor: 4.891
Authors: Alessandro Orsini; Thomas Foiadelli; Attilio Sica; Andrea Santangelo; Niccolò Carli; Alice Bonuccelli; Rita Consolini; Sofia D'Elios; Nicolò Loddo; Alberto Verrotti; Giuseppe Di Cara; Chiara Marra; Maria Califano; Anna Fetta; Marianna Fabi; Stefania Bergamoni; Aglaia Vignoli; Roberta Battini; Marta Mosca; Chiara Baldini; Nadia Assanta; Pietro Marchese; Gabriele Simonini; Edoardo Marrani; Francesca Felicia Operto; Grazia Maria Giovanna Pastorino; Salvatore Savasta; Giuseppe Santangelo; Virginia Pedrinelli; Gabriele Massimetti; Liliana Dell'Osso; Diego Peroni; Duccio Maria Cordelli; Martina Corsi; Claudia Carmassi Journal: Int J Environ Res Public Health Date: 2022-08-25 Impact factor: 4.614