Choreoathetosis

Choreoathetosis is a movement disorder that combines involuntary, dance-like jerking movements with slow, writhing motions, creating a pattern of uncontrollable body movements that can affect daily activities and quality of life.

Table of contents

• What is choreoathetosis?
• Medical identification codes
• Symptoms and characteristics
• Causes and related conditions
• How is it diagnosed?
• Treatment options
• Outlook and living with the condition

What is choreoathetosis?

Choreoathetosis is the occurrence of involuntary movements in a combination of chorea (irregular migrating contractions) and athetosis (twisting and writhing)^[1]. Rather than being a disease itself, choreoathetosis is a symptom that can result from several very different disorders^[3].

The term choreoathetosis combines two types of movement problems. Chorea is characterized by random, flowing involuntary movements that cannot be suppressed. They start in one part of the body and move abruptly, unpredictably, and often continuously to another part. Chorea typically involves the face, mouth, trunk, and limbs^[3]. The word “chorea” comes from the Greek word that means “to dance,” as the movements can resemble dancing^[5].

Athetosis is a continuous stream of slow, flowing, writhing involuntary movements. It usually affects the hands and feet^[3]. These movements are smooth and don’t appear to be composed of movement fragments. Unlike chorea, athetosis may involve the same regions of the body^[15].

When chorea and athetosis occur together as choreoathetosis, they usually cause writhing, dance-like movements^[3]. The combined movements are not as slow as athetosis alone but aren’t as fast as chorea alone^[11].

Medical identification codes

G25.5

Symptoms and characteristics

The symptoms of choreoathetosis are easily recognized and include involuntary movements throughout the body. These movements can range from mild restlessness to severe, disabling motions^[11].

Common symptoms include:

• Muscle tightness
• Involuntary twitching
• Fixed hand position
• Uncontrollable muscle jerks
• Abnormal movements of the body or specific body parts
• Consistent writhing movements^[11]

People with choreoathetosis may exhibit what doctors call “milkmaid’s grip.” This means they cannot maintain a coordinated hand grip and will squeeze and release their hand repeatedly, similar to the motion used when milking a cow^[1]. Another common symptom is involuntarily sticking out the tongue^[1].

Choreoathetosis episodes can occur randomly or be triggered by certain factors such as caffeine, alcohol, or stress. Prior to an episode, a person may feel their muscles begin to tighten or experience other physical symptoms. Attacks can last anywhere from 10 seconds to over an hour^[11].

The condition can affect posture, walking ability, and everyday movement. It may also interfere with speech, swallowing, and the ability to perform daily tasks^[11]. More severe cases can cause permanent disability^[11].

Causes and related conditions

Choreoathetosis results from overactivity in the basal ganglia, the part of the brain that helps initiate and smooth out voluntary movements. In most forms of chorea, an excess of dopamine (a chemical messenger in the brain) prevents the basal ganglia from functioning normally^[3].

It is caused by many different diseases and agents^[1]. Choreoathetosis is often associated as a symptom from other triggering conditions or disorders^[11].

The condition is a symptom of several diseases, including:

• GLUT1 deficiency syndrome
• Lesch-Nyhan syndrome
• Phenylketonuria
• Huntington disease
• Kernicterus (rapidly increasing unconjugated bilirubin that crosses the blood-brain barrier in infants)^[1]

Additional possible causes include:

• Medication or trauma or injury
• Cerebral palsy
• Brain tumors
• Tourette syndrome
• Wilson’s disease^[11]

Choreoathetosis is also a common presentation of dyskinesia (abnormal involuntary movements) as a side effect of levodopa-carbidopa in the treatment of Parkinson disease^[1]. The use of crack cocaine or amphetamines can result in conditions described as choreoathetoid^[1].

The condition can affect people of any age or gender. People 15 to 35 years old are most likely to have this disorder. While some cases of choreoathetosis are short-lived, more severe episodes can remain for years. The condition can occur suddenly or can develop over time^[11].

How is it diagnosed?

Diagnosing choreoathetosis requires careful observation of the entire body throughout the clinical encounter^[6]. A healthcare provider will need to differentiate choreoathetosis from other movement disorders, which can be challenging.

A complete medication and family history are required to investigate for acquired or inherited causes of the condition^[6]. After a thorough review of medical history, doctors may recommend various tests to identify the underlying cause^[11].

The time course and onset of symptoms can offer valuable clues for diagnosis. In general, genetic causes are associated with chronic symptoms, whereas acquired causes commonly result in acute or sudden symptoms^[6].

Treatment options

There is no cure for choreoathetosis. Treatment options focus on managing the symptoms of this condition. Treatment also depends on the underlying cause^[11].

If an underlying condition causes choreoathetosis, doctors will prescribe medication for that condition. If choreoathetosis is caused by a medication someone is taking, the doctor will either switch the dose or change the medication^[20].

After reviewing medical history, doctors may recommend medication to reduce or eliminate choreoathetosis episodes. These drugs are meant to relax muscles and ease pain. Common medication options include:

• Carbamazepine, an anticonvulsant to treat nerve pain and prevent seizures
• Phenytoin, an anticonvulsant to treat and prevent seizures
• Muscle relaxants^[11]

The drug carbamazepine is most often used to treat paroxysmal choreoathetosis, a related condition involving episodes of uncontrollable movements^[2].

In some cases, if choreoathetosis is not severe enough to inhibit daily life, treatment might not be necessary^[20]. Another treatment that doctors could recommend for severe cases that have not responded to other treatments is deep brain stimulation. This is a newer treatment and is considered experimental^[20].

Often, the medications used to treat choreoathetosis are very specialized and can affect brain chemistry. People should let their doctor know if they have a history of anxiety, depression, or other mental health issues^[20].

Outlook and living with the condition

The outlook for choreoathetosis depends on its underlying cause. Some cases resolve with treatment of the primary condition, while others may persist or worsen over time.

For people living with choreoathetosis, surgical procedures may sometimes be needed. Physical and occupational therapy can help manage symptoms and improve daily functioning^[11].

Many of the causes of choreoathetosis, such as inherited conditions or age-related changes, cannot be prevented. The best approach is to maintain general health and get regular medical treatment, especially if someone has a condition that might affect the brain and body enough to cause choreoathetosis^[20].

People experiencing consistent, uncontrollable, uncomfortable, and debilitating movements should seek medical attention. Early diagnosis and appropriate treatment can help manage symptoms and improve quality of life.