Treatment options for chorea

ABSTRACT

Introduction: Chorea is defined as jerk-like movements that move randomly from one body part to another. It is due to a variety of disorders and although current symptomatic therapy is quite effective there are few etiology- or pathogenesis-targeted therapies. The aim of this review is to summarize our own experience and published evidence in the treatment of chorea.

Areas covered: After evaluating current guidelines and clinical practices for chorea of all etiologies, PubMed was searched for the most recent clinical trials and reviews using the term ‘chorea’ cross referenced with specific drug names.

Expert commentary: Inhibitors of presynaptic vesicular monoamine transporter type 2 (VMAT2) that cause striatal dopamine depletion, such as tetrabenazine, deutetrabenazine, and valbenazine, are considered the treatment of choice in patients with chorea. Some clinicians also use dopamine receptor blockers (e.g. antipsychotics) and other drugs, including anti-epileptics and anti-glutamatargics. ‘Dopamine stabilizers’ such as pridopidine and other experimental drugs are currently being investigated in the treatment of chorea. Deep brain stimulation is usually reserved for patients with disabling chorea despite optimal medical therapy.

KEYWORDS:

• Chorea
• Huntington’s disease
• dyskinesia
• tetrabenazine
• deutetrabenazine
• antipsychotics
• neuroleptics
• DBS

Declaration of interest

J Jankovic has received research or training grants from: Adamas Pharmaceuticals Inc, Allergan, Biotie Therapies, CHDI Foundation, Civitas/Acorda Therapeutics, Dystonia Coalition, Dystonia Medical Research Foundation, Hoffman-LaRoche, Huntington Study Group, Kyowa Haako Kirin Pharma, Medtronic Neuromodulation, Merz Pharmaceuticals, Michael J Fox Foundation for Parkinson’s Research, National Institutes of Health, Neurocrine, Biosciences, NeuroDerm Ltd, Parkinson’s Foundation, Parkinson Study Group, Pfizer, Prothena Biosciences, Psyadon Pharmaceuticals, Revance Therapeutics, Sangamo Biosciences, St Jude Medical, and Teva Pharmaceuticals. He has served as a consultant or on the advisory board for: Adamas Pharmaceuticals, Allergan, Pfizer, Prothena Biosciences, Revance Therapeutics, Teva Pharmaceuticals. He has received royalties or other payments from: Cambridge, Elsevier, Future Science Group, Hodder Arnold, Medlink, Neurology, Lippincott Williams and Wilkins, and Wiley-Blackwell. The authors have no other relevant affiliations or financial involvement with any organization or entity with a financial interest in or financial conflict with the subject matter or materials discussed in the manuscript apart from those disclosed.

A peer reviewer on this manuscript has declared receipt of honoraria from Temmler, AOP Orphan, Destin, and Teva but have no other relevant financial relationships to disclose.

Additional information

Funding

This paper was not funded.