Chorea
by Drs. Like Wu, Xiaojuan Wang, Bo Cheng, Susan Chu, Xinrui Xi and Fang Peng
Wu Medical Center, Bejing, China
Chorea is the name for a group of disorders that causes involuntary movements or contractions in varied parts of the body. The type present may indicate where the body movements occur, though not always. With Huntington's disease, for instance, jerky body movements may happen with great frequency in the face and in other areas, and these symptoms tend to worsen as the disease progresses. In contrast, Chorea gravidarum, which occurs during pregnancy, is usually noticed the most in the limbs and face, and with Sydenham's chorea, mostly the face is affected by a series of uncontrolled facial contractions and grimaces.
The term hemichorea refers to chorea of one side of the body, such as chorea of one arm but not both (comparable to hemiballismus).
Presentation
Chorea is characterized by brief, semi-directed, irregular movements that are not repetitive or rhythmic, but appear to flow from one muscle to the next. These 'dance-like' movements of chorea often occur with athetosis, which adds twisting and writhing movements. Walking may become difficult, and include odd postures and leg movements. Unlike ataxia, which affects the quality of voluntary movements, or Parkinsonism, which is a hindrance of voluntary movements, the movements of chorea and ballism occur on their own, without conscious effort. Thus, chorea is said to be a hyperkinetic movement disorder. When chorea is serious, slight movements will become thrashing motions; this form of severe chorea is referred to as ballism or ballismus.
Causes
Chorea can occur in a variety of conditions and disorders. Chorea is a primary feature of Huntington's disease, a progressive neurological disorder.
Twenty percent (20%) of children and adolescents with rheumatic fever develop Sydenham's chorea as a complication.
Chorea gravidarum is a rare type of chorea which occurs as a complication during pregnancy. Chorea may also be caused by drugs (levodopa, anti-convulsants, anti-psychotics), metabolic disorders, endocrine disorders, and stroke.
Wilson's disease is a genetic disorder that leads to toxic levels of copper in the body.
Transmissible spongiform encephalopathies: A group of neurodegenerative diseases such as Creutzfeldt-Jakob disease and Kuru, caused by prions.
Neuroacanthocytosis
A genetic disorder that may affect the blood, brain, peripheral nerves, muscles and heart. Common symptoms include peripheral neuropathy, cardiomyopathy and hemolytic anemia. Other symptoms include limb chorea, facial tics, other oral movements (lip and tongue biting), seizures, a late-onset dementia and behavioral changes.
Treatment
Treatment can be different depending on the cause. For instance, some treatments for pregnant women have included using anti-psychotic medications like haloperidol (Haldol®), and some benzodiazepine tranquilizers. These may not fully control movements but might eliminate them to some degree. There is great concern about using strong medications during pregnancy because of the possible negative effects on the fetus.
Although drug therapy can relieve some patients' symptoms, for most patients medication cannot drastically improve their quality of life, and these kinds of drugs need to be monitored closely as they often have clear adverse reactions. With long-term research, the Wu Stem Cell Medical Center (WSCMC) found that the neural stem cell implantation is good for patients who suffer from chorea, especially for those patients whose brain, particularly in the thalamus - striatum system, have a lot of nerve cell degeneration and necrosis. Thus, the supplement of nerve cells can fundamentally solve the imbalance of neurotransmitter in the brain. The effects of this treatment are better than those of the drug treatment.
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