Chorea

Francisco Cardoso MD PhD (Dr. Cardoso of the Federal University of Minas Gerais received consultation fees from Roche, Teva and Zambon and honorariums from Boehringer-Ingelheim, Roche, Teva, and UCB for speaking engagements.)
Joseph Jankovic MD, editor. (Dr. Jankovic, Director of the Parkinson's Disease Center and Movement Disorders Clinic at Baylor College of Medicine, received research funding from Allergan, Allon, Ceregene, Chelsea, EMD Serono, Impax, Ipsen, Lundbeck, Medtronic, Merz, and Teva, and compensation for his services as a consultant or an advisory committee member by Allergan, Auspex, EMD Serono, Lundbeck, Merz, Neurocrine Biosciences, and Teva.)
Originally released August 5, 1994; last updated April 6, 2016; expires April 6, 2019

This article includes discussion of chorea and paraneoplastic chorea. The foregoing terms may include synonyms, similar disorders, variations in usage, and abbreviations.

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 populations where there is a large presence of Africans. There is growing evidence to support the notion that benign hereditary chorea is a syndrome with several possible underlying genetic causes. 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.

 

• Although a rare condition, deficiency of glucose transporter 1 is increasingly recognized as a cause of chorea and different types of seizure disorders in childhood.

 

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 (Cardoso et al 2006). 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 (Goetz et al 2001). 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 (Huntington 1872). 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 (Goetz 2000). 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 (Vale et al 2013). The author and Vale published a comprehensive review of the history of chorea (Vale and Cardoso 2015). From a 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.

The content you are trying to view is available only to logged in, current MedLink Neurology subscribers.

If you are a subscriber, please log in.

If you are a former subscriber or have registered before, please log in first and then click select a Service Plan or contact Subscriber Services. Site license users, click the Site License Acces link on the Homepage at an authorized computer.

If you have never registered before, click Learn More about MedLink Neurology  or view available Service Plans.