Recent advances in the management of choreas

Abstract

The management of patients with chorea, in particular Huntington’s disease, is a complex task requiring skills in a number of areas. This paper reviews new knowledge on this topic and places it in the context of established procedures. It is focused on Huntington’s disease, since this is the disorder, for which most publications on management have been published in the past few years. Management starts with appropriate diagnosis and differential diagnosis, with the aim of finding disorders with chorea amenable to causative treatment. The place of genetic testing and the importance of genetic counselling is stressed, as well as the importance of precise observation in the course of the disorder to tailor appropriate therapies. Pharmacological treatment is based on poor evidence but to a large extent on expertise from centres devoted to the care of patients with Huntington’s disease. It is focused mainly on motor and psychiatric aspects of the phenotype. Nonpharmacological treatment is important and is best offered in a multidisciplinary care setting.