Chorea

Erin Furr Stimming, MD, FAAN; Danny Bega, MD Movement Disorders p. 1379-1408 October 2022, Vol.28, No.5 doi: 10.1212/CON.0000000000001169
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PURPOSE OF REVIEW This article provides an overview of the diagnostic and therapeutic approach to a patient with chorea. The phenomenology of chorea is described in addition to other common hyperkinetic movements that may be mistaken for or coexist with chorea. Chorea can be acquired or hereditary. Key historical and clinical features that can aid in determining the etiology are reviewed, and pharmacologic and nonpharmacologic treatment strategies are discussed.

RECENT FINDINGS Clinical investigations are under way to target transcription and translation of the mutant huntingtin protein as a potential disease-modifying strategy in Huntington disease (HD). Additional heritable factors have been revealed through genome-wide association studies. Symptom-focused treatments for HD are are being studied, including a third vesicular monoamine transporter-2 (VMAT2) inhibitor for chorea attenuation and drugs to target irritability and cognitive impairment. Increased availability of genetic testing has led to increased awareness of HD mimics (eg, C9orf72 and IgLON5).

SUMMARY Chorea is a relatively common hyperkinetic disorder with a broad differential. The first step in the approach to a patient with chorea is accurately defining the phenomenology. Once it has been determined that the patient has chorea, the investigation into determining an etiology can begin. Factors such as age of onset, time course, family history, unique clinical features, and imaging and laboratory findings can guide the diagnosis. Treatments for most causes of chorea are purely symptomatic, although it is important to recognize causes that are reversible or have disease-modifying interventions.

Address correspondence to Dr Erin Furr Stimming, 6431 Fannin St, MSB 7.004, Houston, TX 77030, [email protected].

RELATIONSHIP DISCLOSURE: Dr Furr Stimming has received personal compensation in the range of $500 to $4999 for serving as a consultant for Teva Pharmaceutical Industries Ltd and Wave Life Sciences, on a scientific advisory board for Amneal Pharmaceuticals LLC, and on speakers bureaus for Sunovion Pharmaceuticals Inc and Teva Pharmaceutical Industries Ltd. Dr Furr Stimming has served as an editor and author for McGraw Hill and Oxford University Press. The institution of Dr Furr Stimming has received research support from the CHDI Foundation; Cures Within Reach; Huntington Study Group/Neurocrine Biosciences, Inc; the Huntington's Disease Society of America; F. Hoffman-La Roche/Genetech, Inc; the National Institutes of Health/University of Iowa; uniQure NV; and Vaccinex Inc. Dr Bega has received personal compensation in the range of $500 to $4999 for serving on speakers bureaus for Acorda Therapeutics; Adamas Pharmaceuticals, Inc; Kyowa Kirin Co, Ltd; Neurocrine Biosciences, Inc; and Teva Pharmaceutical Industries Ltd and as an editor, associate editor, or editorial advisory board member for the Annals of Clinical and Translational Neurology/American Neurological Association. The institution of Dr Bega has received research support from the Huntington’s Disease Society of America.

UNLABELED USE OF PRODUCTS/INVESTIGATIONAL USE DISCLOSURE: Drs Furr Stimming and Bega discuss the unlabeled/investigational use of amantadine, benzodiazepines, botulinum toxin injections, cannabinoids, carbamazepine, clozapine, deep brain stimulation, olanzapine, quietipine, risperidone, and valbenazine for the treatment of Huntington disease and other causes of chorea.

© 2022 American Academy of Neurology.