Pediatric chorea

What is pediatric chorea?

Chorea causes random, involuntary muscle activity. The movements are often described as dance-like because they seem to flow across the body, from one muscle to the next. These movements can be small or large. They may affect the same body area, but each time they look a bit different.

Chorea can affect any muscle in the body and usually affects many muscles. It can involve a child’s limbs, their facial movements, posture, speech, and ability to swallow and walk.

What are the signs and symptoms of pediatric chorea?

Symptoms of chorea are random, uncontrollable movements that can affect any part of the body. For most children with chorea, the extra movements happen in all parts of their body.

For others, certain body areas are affected more than others. Chorea may affect one side of the body more than the other side, or the hands may be more affected than the feet. The location of chorea in the body does not tell us much about the severity or the cause of chorea.

Other symptoms may include:

• Difficulty with coordination
• Restlessness
• Low muscle tone (hypotonia)
• Uncontrolled, continuous movement
• Difficulty with fine, precise movements

How is pediatric chorea diagnosed?

Your child’s first visit with a neurologist will be a detailed one-hour appointment.

Here is what you can expect:

• Your neurologist will ask you about your child’s medical history, your family’s medical history, and what you have noticed about your child’s movement.
• Your neurologist will do a neurological exam to test your child's reflexes, strength and muscle tone to see how their nerves are functioning. The neurologist will also check your child’s balance, coordination and motor and sensory skills.
• Your neurologist will observe your child at rest, how your child moves when following directions, and how they move without direction. Observation gives the neurologist important clues to what is happening to your child.
• Your neurologist may take a video of your child’s movement. Having a video is important because a child may have more than one movement disorder, and the movements may change over time. Videos can help the neurologist pinpoint which disorder your child has and track any evolution in the movements.

We may recommend one or more of the following tests:

• Electroencephalogram (EEG) can test for seizures, which can occur with chorea. During this painless test, a member of the care team places electrodes on your child’s scalp to record the brain’s electrical activity.
• Magnetic resonance imaging (MRI) takes detailed pictures of the brain that can show whether an injury is causing your child’s symptoms.
• Genetic testing can identify changes (also called mutations) in your child's chromosomes, genes or proteins that may have caused chorea.

What causes pediatric chorea?

Chorea can occur at any age and with many conditions.

Possible causes may be:

• Birth injuries
• Cerebral palsy (CP)
• Exposure to toxins such as carbon monoxide, manganese or pesticides
• Head/brain trauma
• Hemorrhages or strokes
• Brain tumors
• Hormone imbalance such as hyperthyroidism or hypothyroidism
• Infections such as chicken pox, Lyme disease, HIV-related immune deficiencies and bacterial endocarditis (a heart infection)
• Kidney failure
• Metabolic disorders, including low levels of blood sugar, calcium, magnesium, sodium and vitamin B12
• Reaction to medicines, including those for seizures, allergies, attention deficit/hyperactivity disorder (ADHD), and psychiatric/mood disorders
• Rheumatic fever which can result from strep throat or scarlet fever
• Wilson’s disease which causes too much copper to build up in the body

How is pediatric chorea treated?

Treatments for chorea depend on the cause. Sometimes, treating the underlying condition treats the movement disorder. When that’s not possible, we prescribe medications to relieve symptoms.

In rare cases, doctors may recommend deep brain stimulation (DBS), which is like a heart pacemaker but for the brain. Your child’s surgeon will implant a tiny device (called a pulse generator) under the skin of your child’s chest, below the collarbone or in the belly. The surgeon will also implant tiny electrodes in the deepest part of the brain that controls movement. The pulse generator sends electrical signals to the electrodes to help control your child's involuntary movements.