Chorea

Pichet Termsarasab, MD p. 1001-1035 August 2019, Vol.25, No.4 doi: 10.1212/CON.0000000000000763
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PURPOSE OF REVIEW: This article provides an overview of the approach to chorea in clinical practice, beginning with a discussion of the phenomenologic features of chorea and how to differentiate it from other movement disorders. The diagnostic approach, clinical features of important acquired and genetic choreas, and therapeutic principles are also discussed. Practical clinical points and caveats are included.

RECENT FINDINGS: C9orf72 disease is the most common Huntington disease phenocopy, according to studies in the European population. Anti-IgLON5 disease can present with chorea. The role of immunotherapies in Sydenham chorea has increased, and further clinical studies may be useful. Benign hereditary chorea is a syndrome or phenotype due to mutations in several genes, including NKX2-1, ADCY5, GNAO1, and PDE10A. New-generation presynaptic dopamine-depleting agents provide more options for symptomatic treatment of chorea with fewer adverse effects. Deep brain stimulation has been performed in several choreic disorders, but features other than chorea and the neurodegenerative nature should be taken into consideration. Studies on genetic interventions for Huntington disease are ongoing.

SUMMARY: Clinical features remain crucial in guiding the differential diagnosis and appropriate investigations in chorea. Given the complexity of most choreic disorders, treating only the chorea is not sufficient. A comprehensive and multidisciplinary approach is required.

Address correspondence to Dr Pichet Termsarasab, Ramathibodi Hospital, Mahidol University, Bangkok, Thailand, pichet.ter@mahidol.ac.th.

RELATIONSHIP DISCLOSURE: Dr Termsarasab serves as associate editor of the Journal of Clinical Movement Disorders and on the editorial board of Brain Science Journal. Dr Termsarasab has received personal compensation for speaking engagements for the American Academy of Neurology and Novartis AG and receives publishing royalties from MedLink Neurology.

UNLABELED USE OF PRODUCTS/INVESTIGATIONAL USE DISCLOSURE: Dr Termsarasab discusses the unlabeled/investigational use of the current recommended treatments of chorea, none of which are approved by the US Food and Drug Administration except the use of deutetrabenazine for the treatment of chorea associated with Huntington disease and tardive dyskinesia, tetrabenazine for the treatment of chorea associated with Huntington disease, and valbenazine for the treatment of tardive dyskinesia.

© 2019 American Academy of Neurology.