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  • Updated 12.30.2021
  • Released 08.05.1994
  • Expires For CME 12.30.2024

Chorea

Introduction

Overview

Chorea, derived from the Latin choreus meaning "dance," describes a syndrome characterized by irregular, hyperkinetic, involuntary movements resulting from a continuous flow of random muscle contractions. Athetosis refers to the writhing, slow, and irregular movements involving the hand and fingers. Choreoathetosis is the term to describe chorea with a writhing quality. When chorea is mild, it is difficult to differentiate from restlessness. Proximal flinging movements is referred as ballism. Although the mechanism is not completely understood, chorea is thought to be resulted from the imbalance in the direct and indirect pathways in the basal ganglia circuitry (12). Enhanced activity of the direct pathway, or disruption of the inhibitory function of the indirect pathway, leads to the development of chorea. Primary chorea is either idiopathic or hereditary. Secondary chorea is related to autoimmune, vascular, infectious, pharmacologic, and metabolic. The author aims to provide an up-to-date discussion of the clinical presentations, etiology, pathogenesis, differential, and management of this order.

Key points

• Vascular disease of the brain is the most common cause of nongenetic chorea in adults.

• Cognitive and behavioral symptoms are the first manifestations of Huntington disease, the most common cause of genetic chorea.

• Antidopaminergic drugs are the mainstay to achieve symptomatic control of chorea, regardless of the underlying etiology.

• There is a growing list of genetic causes of chorea as well as seizure disorders in children.

ADCY5 gene mutation is the second most common cause of benign hereditary chorea.

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