Choreoathetosis: Definition, Causes, Symptoms, Diagnosis, Treatment and Prognosis

The term is a combination of the word “chorea” and the word “athetosis.”

Chorea and athetosis are two types of irregular movements that often occur in combination as the same disorder.
Chorea is characterized by rapid, short, rigorous, unpredictable and irregular muscle jerks, such as restlessness or movements of the arms and legs.
Chorea mainly affects the face, extremities, or trunk of the body.
Athetosis is a movement disorder in which the patient has slow twisting movements with forced uncoordination involving the extremities.
These movements can also affect the facial muscles.
Choreoathetosis is a movement disorder that causes involuntary twitching or twisting, which commonly develops as a result of sudden infections, muscle movement disorders, or injuries.
Choreoathetosis can occur chronically or acutely. It can be transitory or a permanent phenomenon in the life of a patient.
Choreoathetosis can also affect the patient’s limb movements, the ability to speak, change position, and walk.
Normally the movements are involuntary and are always occurring in the human body.
However, sometimes these movements become chronic and disabling disorders, representing a serious condition.
While some cases of choreoathetosis are short-lived, the more severe episodes can persist for years. The condition can occur suddenly or it can develop over time.
Choreoathetosis can affect people of any age or gender. People 15 to 35 years of age are more likely to have this disorder.

Causes of choreoathetosis

Choreoathetosis is often associated as a symptom of other triggering conditions or disorders. Possible causes include:
  • Side effects of certain medications.
  • Hyperthyroidism .
  • Systemic lupus
  • Acantocitosis.
  • Wilson’s disease.
  • Lesch-Nyhan syndrome.
  • Trauma or injuries.
  • Infections
  • Cerebral palsy.
  • Tumors
  • Huntington’s disease.
  • Tourette syndrome.
  • Wilson’s disease.
  • Kernicterus, a type of brain damage in newborns with jaundice.
  • Chorea gravidarum.
  • Corea de Sydenham.
  • Ataxia telangiectasia.
There are also congenital forms of choreoathetosis.
Severe choreoathetosis is a complication in up to 10% of children with congenital heart disease after cardiopulmonary bypass surgery and profound hypothermia.
Profound hypothermia and circulatory arrest are not essential factors in pathophysiology, but they are often associated.

Symptoms of choreoathetosis

There are easily identifiable symptoms of choreoathetosis, it can affect posture, ability to walk and daily movement. The most severe cases can cause permanent disability.
Choreoathetosis patients have difficulty controlling their muscles and cannot control how their body moves.
The symptoms of choreoathetosis are easily recognizable and include:
  • Muscle tightness
  • Involuntary spasms
  • Fixed hand position.
  • Uncontrollable muscle jerks.
  • Abnormal movements of the body or specific parts of the body.
  • Constant twisting movements.
Choreoathetosis episodes can occur randomly. Certain factors can also trigger an episode, such as caffeine, alcohol, or stress.
Before an episode, you may feel your muscles begin to tense or other physical symptoms. Attacks can last from 10 seconds to over an hour.

Diagnosis

Clinical features are the only basis for diagnosis. MRI results are normal during acute illness.

Treatment of choreoathetosis

Treatment for choreoathetosis will depend on the physical condition of the patient.
There is no cure for choreoathetosis. Treatment options focus on managing the symptoms of this condition. Treatment also depends on the underlying cause.
After a thorough review of the patient’s medical history, the doctor may recommend medications to reduce or eliminate episodes of choreoathetosis. These medications are intended to relax muscles and relieve pain.
Common drug options for choreoathetosis include:
  • Carbamazepine, an anticonvulsant to treat nerve pain and prevent seizures.
  • Phenytoin, an anticonvulsant to treat and prevent seizures.
  • Muscle relaxants.
Smaller doses of medications, such as phenytoin and carbamazepine, can be very effective in treating choreoathetosis, however, these medications should be administered according to the physical condition of the patient.
Sedation prevents exhaustion in severely affected children. Choreoathetosis is often refractory to drug therapy. Clonazepam and levetiracetam may be helpful in reducing movement.
Surgery, although invasive, can also help reduce episodes of choreoathetosis. Doctors may recommend deep brain stimulation, which places the electrodes in the part of the brain that controls muscle movements.
The electrodes are connected to a device that delivers electrical pulses and blocks tremors. While this procedure has been successful, it carries the risk of infection and requires replacement of the surgical battery over time.
Research continues to develop therapies aimed at curing choreoathetosis rather than just managing the symptoms of this disease.

Prognosis of choreoathetosis

Choreoathetosis is considered a chronic and more dangerous movement disorder, as this condition directly affects the muscles, joints, bones, and tissues.
Patients suffering from choreoathetosis have to struggle to move properly and naturally.
Choreoathetosis can be very serious and can cause permanent disability in the patient.
While there is no cure for choreoathetosis, different treatment options can address the symptoms.
The prescription medication instructions should be followed so that symptoms do not worsen.
Making changes at home can also improve your quality of life. If choreoathetosis is affecting daily movement, the home should be adapted to prevent injury or further trauma from slips and falls.