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  • Updated 08.16.2020
  • Released 08.05.1994
  • Expires For CME 08.16.2023

Chorea

Introduction

Overview

The author provides a practical approach to patients with chorea who present to neurologists. Emphasis is given to aspects of differential diagnosis, stressing the notion that among children, regardless of the geographic area of the world, Sydenham chorea remains the most common cause of this hyperkinetic movement disorder. On the other hand, drug-induced chorea and vascular chorea-hemiballism are the most frequent causes of chorea in adults. Huntington disease is the most important genetic disorder associated with chorea, although at least 3% of patients with this phenotype have a negative genetic test for this condition. Among Caucasians, C9orf72 is the most frequent cause of Huntington disease phenocopies, whereas HDL2 is the most common identifiable cause in countries with significant numbers of inhabitants of African origin. There is growing evidence to support the notion that benign hereditary chorea is a genetically and phenotypically heterogeneous syndrome. Treatment of chorea remains largely symptomatic, with antidopaminergic agents being the cornerstone of management.

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.

PRRT2 gene mutation is a major cause of hereditary paroxysmal chorea, seizures, and hemiplegic migraine.

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

Historical note and terminology

Chorea, derived from the Latin choreus meaning "dance," describes a syndrome characterized by brief, abrupt involuntary movements resulting from a continuous flow of random muscle contractions. The pattern of movement may sometimes appear playful, conveying a feeling of restlessness to the observer. When choreic movements are more severe, assuming a flinging, sometimes violent, character, they are called ballism (34). Data show that many subjects are capable of voluntarily suppressing the choreic movements (24). First noted in the Middle Ages, the most common illness was perhaps the postinfectious chorea now known as Sydenham chorea. There were also outbreaks of “dancing mania,” a psychiatric condition coinciding with epidemics of the plague in Central Europe (80). In the 19th century the concise report by George Huntington on affected families in the state of New York led to a clear recognition of hereditary chorea, which would be later named after him (90). In On Chorea and Choreiform Affectations published in 1894, William Osler defined distinct clinical differences between Sydenham chorea and Huntington disease. Based on clinical and pathological observations, Osler concluded that Sydenham chorea was a sporadic form of chorea associated with rheumatic fever, whereas Huntington chorea was a familial form of chorea associated with dementia (79). Sir William Gowers also recognized that chorea was a syndrome with a myriad of underlying causes, although Sydenham chorea accounted for the majority of his cases (210). The author and Vale published a comprehensive review of the history of chorea (209). From an epidemiological and neurobiological perspective, Huntington disease is the most important cause of chorea due to its selective neurodegeneration, well-characterized genetic basis, and important psychosocial implications. Many other causes of chorea are now recognized in clinical practice. Correct diagnosis is of course extremely important given the varied prognoses, treatments, and genetic factors.

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