Chorea: What Is It and Why Does It Occur?

Chorea refers to involuntary and sudden movements that can't be controlled. We'll explain which diseases can cause it and what can be done about it here.

Reviewed and approved by the doctor Diego Pereira.

Written by Carmen Martín

Last update: 08 May, 2023

The word ‘chorea’ is used to describe a neurological symptom that accompanies certain diseases. This term comes from the Greek choreia which translates as ‘dance’. It’s defined as a sudden, involuntary, and short-lived movement in some part of the body.

It usually affects the hands or face; for example, it occurs when a person moves his hand unconsciously, as if he or she were going to hit something. As this is something that cannot be controlled, this tends to affect the quality of life of those who suffer from it.

In itself, this is a condition associated with diseases such as rheumatic fever or Huntington’s disease, although they are not its only causes. In this article, we’ll explain everything you need to know about chorea and why it occurs.

What is chorea?

Chorea is the medical term used to refer to rapid, involuntary, and jerky movements that usually occur in the distal portion of the limbs or in the face. However, the trunk can also be affected.

As explained in a publication in the National Institute of Neurological Disorders and Stroke the movements in this condition are unpredictable. They change from one area of the body to another, without following any sequence.

It usually appears as a consequence of lesions in certain brain areas, such as the caudate nucleus or the putamen. It isn’t a disease, but a clinical manifestation or symptom of other health disorders. Therefore, it’s not considered life-threatening in itself.

However, as it usually occurs in the context of other pathologies, it may constitute a risk. Thus, depending on its cause, chorea can be temporary or not. Sometimes, it’s maintained and worsens progressively.

For example, this is what happens in Huntington’s disease. It may also be accompanied by two other characteristic movements.

• Firstly, it may be related to ballismus’. This term refers to more intense movements, such as throwing an arm sharply and concisely.
• It can also be related to athetosis. In this case, what appears are slow movements, also uncontrollable. These resemble sustained wringing of the hands or limbs.

The symptoms of chorea

Chorea usually produces involuntary movements of the limbs. Spasms of the limbs are also common. As there is no pattern and it’s unpredictable, these occur at different places in the body.

According to a publication of the Clínica Universidad Navarra, at the beginning of the symptoms, people only experience nervous tics or excessive agitation. However, as the picture progresses, the movements become more intense and marked.

Therefore, jerks of the head, extremities, or neck appear. These movements can interfere with the person’s daily life. This is because they tend to cause difficulties in speaking, interacting, walking, or eating.

Moreover, the mood also deteriorates. Chorea often produces abrupt mood changes. It’s common for a person with this condition to be apathetic, irritable, depressed, or aggressive. Cognitive functions are progressively impaired. Hence, memory or thinking may be affected.

Related diseases

Chorea may occur in the context of numerous pathologies. They are usually classified according to whether they are acquired or hereditary. Within the hereditary ones, the main cause is Huntington’s disease.

This represents a genetic disorder that is inherited in an autosomal dominant manner. That is, only one copy of the defective gene is needed for the disease to appear. In this disease, chorea is often accompanied by personality disorders and speech problems.

Symptoms worsen progressively and, on average, patients have a life expectancy of 10 to 30 years when the manifestations appear. Unfortunately, there is no cure for Huntington’s disease.

Chorea can also occur as a complication of rheumatic fever. This, in turn, is a complication of a bacterial infection (group A streptococcus). It’s common in children who have had streptococcal pharyngitis.

Other infections may be associated, such as syphilis, Lyme disease or toxoplasmosis. Autoimmune diseases, such as systemic lupus erythematosus, can also cause it. The same applies to multiple sclerosis or sarcoidosis.

Finally, endocrine disorders should be highlighted. The most common ones associated with chorea are hypoglycemia, hyperglycemia, thyroid problems, and hypocalcemia.

Causes and risk factors for chorea

Chorea is often a symptom of another disorder. As a Cleveland Clinic article explains, an estimated 30,000 people in the United States alone suffer from Huntington’s disease. Moreover, there are approximately 4000 cases of Sydenham’s chorea per year.

The incidence of Sydenham’s chorea itself is unknown. However, cerebral vascular lesions are one of its most important causes. This explains why approximately 56 patients out of every 1,500 who have suffered a stroke eventually develop it.

To distinguish the cause of this disorder, it’s useful to look at whether it presents acutely or progressively. Acute onset cases are usually caused by a toxic substance or a drug. For example, levodopa to treat Parkinson’s disease or neuroleptics.

Meanwhile, as mentioned above, the most typical progressive forms are caused by Huntington’s disease and rheumatic fever.

Risk factors

There are certain factors that increase the risk of chorea. This is a condition that can occur at any age. However, it’s more typical in elderly patients and children.

In the elderly, it can occur without any apparent cause. Children, on the other hand, are more likely to have rheumatic fever, so they are the ones who tend to suffer from Sydenham’s chorea.

As one gets older, there is also an increased risk of cardiovascular events. Strokes are related to chorea. The same is true for tumors located near the basal ganglia of the brain.

One of the biggest risk factors is family history. In particular, having a family member with Huntington’s disease. Pregnancy is another risk situation, although it’s much less common and is usually self-limiting.

Being under certain medical treatments can increase the likelihood of having this disorder. We noted earlier that it’s associated with levodopa and neuroleptics. Other drugs may include anticonvulsants, antihistamines, or certain antidepressants.

How is it diagnosed?

Diagnosis of chorea can be complex, especially finding the underlying cause of the condition. Suspicion is usually made on clinical grounds, when uncontrollable and unexplained nervous tics are observed.

It’s essential for the physician to know the patient’s medical history, past history, whether the patient has had any infection, whether there are other symptoms, etc. In addition, certain complementary tests are usually performed to guide the diagnosis.

• First of all, a complete blood test is recommended. Through this test, it’s possible to detect infections and hormonal or metabolic alterations. It’s also used to look for antibodies in the case of suspected autoimmune diseases.
• Imaging tests can also be helpful. MRI and CT scans are used when a brain injury is suspected. For example, a stroke.
• Finally, genetic testing can be performed to determine if it has its origin in Huntington’s disease.

Treatments available for chorea

Chorea is treated depending on what is causing it. According to an article by Mapfre Channels Health, Huntington’s disease has no treatment. There are only measures that can help alleviate some of the symptoms, but there is no cure.

For example, dopamine antagonists are used to try to reduce the movements. If psychiatric disturbances occur, neuroleptics, antidepressants, or anxiolytics may be recommended.

In general, most cases of chorea are treated with these drugs. Benzodiazepines are usually the most commonly used anxiolytics. Tetrabenazine and reserpine, which help reduce catecholamines, are also used.

If the cause is a metabolic disorder, it’s essential to correct it. Sydenham’s chorea is usually self-limiting. The movements are also treated with dopamine antagonists. For the condition itself, antibiotics such as penicillin are prescribed, which can eliminate the infection.

What to remember about Chorea disease

Chorea itself is not a disease, but can occur in the context of many disorders. Many people tend to use this term as a synonym for Huntington’s disease. However, as we have seen, it can be a symptom of infections, metabolic disorders, autoimmune diseases, etc.

This symptom can seriously interfere with the sufferer’s life. Therefore, it’s essential to try to find the cause quickly and establish the best treatment in each case.