Choreo-Athetosis is a term that describes jerky movements of the arms, legs, face and other parts of the body. These can be mixed with slower twisting movements of athetosis.
Often nonrhythmical rapid involuntary movements are called choreic, but they can also be tics or myoclonus. Occasionally antipsychotic medications can cause tardive dyskinesia.
Causes
Choreo-athetosis consists of flowing, involuntary, dancelike movements that can be difficult to suppress. They are most often caused by a neurological disorder, but can also occur with certain autoimmune diseases, some endocrine conditions and some medications.
Overactivity of a hormone called dopamine in parts of the brain that control movement (basal ganglia) causes chorea. It usually occurs along with another condition, such as Huntington’s disease or rheumatic fever. Symptoms of this condition may appear in infancy or early childhood. In other cases, symptoms develop during or after adulthood as a result of a stroke or lesion in the basal ganglia.
Sometimes, chorea and athetosis are part of a larger group of neurological disorders called dyskinesias. The other members of this group include tremor, tics and jerky movements. These are all caused by abnormalities in the brain’s structure or function.
Chorea can be mild or severe, depending on the underlying cause. If the underlying disorder is treatable, such as with a movement disorder like Parkinson’s disease or tardive dyskinesia, the chorea will usually go away.
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However, if the cause is untreated, the condition can lead to other problems. For example, people with untreated Huntington’s disease can eventually lose their ability to walk and speak. They may also experience depression or memory loss, which can have a negative impact on their quality of life.
Other causes of chorea include a brain tumor near the basal ganglia, an autoimmune disease like multiple sclerosis or lupus, an infection or complication from an infection such as rheumatic fever and a metabolic or endocrine disorder like hyperthyroidism or kernicterus. Some medications can also cause this condition, including levodopa, tricyclic antidepressants and some antiseizure medicines.
In some people, chorea and athetosis may be a normal symptom of aging. It can also occur for no obvious reason in young women who are pregnant. It can also be a side effect of some medications, including barbiturates. If it occurs as a side effect of a medication, the doctor can prescribe a different medicine. Usually, the treatment that relieves the chorea will also help to relieve athetosis.
Symptoms
The involuntary jerks of choreo-athetosis are slow, writhing movements that involve your hands, feet or face. They look dancelike and are a continuous stream of movement, rather than sporadic bursts of activity. They may also feel semipurposeful or even intentional, making it hard to distinguish them from purposeful movements of a person who is mentally healthy. These movements may be accompanied by a sense of restlessness, and attempts to mask or camouflage the movement can make them more noticeable.
Overactivity of a hormone called dopamine in the part of your brain that controls movement (basal ganglia) causes chorea. Sometimes, it’s a sign of an underlying genetic disorder like Huntington’s disease. It’s also a common side effect of certain drugs, including neuroleptic and dopaminergic medications. Symptoms of chorea include:
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Athetosis is an involuntary jerk that disrupts stable posture. It’s slower than chorea and doesn’t have a dancelike quality. Athetosis is seen in a number of conditions, including Parkinson’s disease, lupus erythematosus, rheumatic fever and perinatal asphyxia. It’s also associated with a stroke or other vascular injury. Athetosis can occur alone or with chorea to form the comorbid condition Choreo-Athetosis.
The combination of athetosis and chorea is referred to as a dyskinesia. The combination is a group of disorders that belong to the category of hyperkinetic movement disorders. They’re caused by overactivity of dopamine in areas of your brain that control movement. In some cases, the combination is a sign of a neurological problem, such as Huntington’s disease or a stroke. In others, it’s due to metabolic or endocrine disorders and a variety of drug side effects. In some people, the combination is a sign of rheumatic valvular heart disease. In other people, it’s a sign of a psychiatric illness. The underlying cause of the disorder determines how severe it is. In people with severe dyskinesias, a psychiatric evaluation and treatment are important. In addition to medication, treatment options for this condition include rehabilitative therapies and support services. It’s important to see a healthcare provider who specializes in movement disorders, such as a neurologist.
Diagnosis
Choreoathetosis may be due to a large number of neurological disorders, and often coexists with dystonia. Its phenomenology does not provide clues to the underlying diagnosis. Rather, features of the family and clinical history, physical and neurological examination and work-up help to determine the cause. This includes structural, metabolic, pharmacological, infectious and inherited neurodegenerative causes. It is also important to distinguish chorea from athetosis, which is characterized by slower movements that have a writhing or twisting quality and is not affected by changes in the position of the body.
Dystonia is often caused by lesions of the basal ganglia or by thalamic disease. The movement disturbances can be jerky or they can have a slow, writhing quality (chaote). Athetosis has the characteristic zigzagging movement of the handwriting “C” and it is often accompanied by sustained contractions that produce abnormal postures. Both athetosis and chorea can be associated with parakinesia, which is an intentional movement designed to mask or camouflage the involuntary movements.
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Chorea is usually asymmetric, with the limbs on one side of the body being affected more than the other. It is also often accompanied by hemiballismus, which involves a single limb (usually the arm more than the leg) that flings wildly. It may also be accompanied by a rapid, jerky gait with loss of balance and falls and abnormal postures.
Inherited forms of the disorder are difficult to distinguish, although an unremarkable family history does not exclude hereditary chorea. Other diagnostic clues include a history of an acute CNS infection or postinfection syndrome, diffuse/scattered fairly discrete signal changes on the MRI of the brain and spinal cord and normal cerebrospinal fluid (CSF) examination with mild pleocytosis.
Choreoathetosis often occurs as a feature of cerebral palsy and other encephalitis. It can occur in association with a sudden episode of epilepsy or after a stroke. It is a cardinal feature of familial paroxysmal chorea and it may also be present in other benign familial forms of the disorder. It is a feature of the disorder that occurs as a sequela to neonatal or early postnatal cerebral damage and it can be seen in the Parkinson-like syndrome that follows encephalitis.
Treatment
In most cases, there is no cure for chorea and athetosis, but treatment can help control symptoms and prevent complications. The specific treatments vary depending on the underlying condition.
Chorea is a disorder that occurs when fast, uncontrollable, and random muscle movements occur. The movements may be either slow or fast, and they may move from part of the body to another, such as a limb to the trunk of the body. These movements can cause abnormal postures, or they can lead to jerking or writhing motions. They may also affect the face or trunk of the body, and they may be accompanied by a loss of balance.
The movement problems in BHC usually start in early childhood and become more pronounced over time. Affected children have hypotonia, delayed motor milestones, and a variety of psychiatric symptoms. They may have difficulty swallowing, and they can develop speech difficulties such as stuttering or dysarthria. Some affected children have cognitive skills that are close to those of other children of the same age, while others have lower cognition.
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Athetosis is a slow, writhing movement of the body that blends with chorea and can hide it. It can be found in conditions that affect the basal ganglia, such as Sydenham chorea and lupus erythematosus. It can be caused by a lesion in the striatum or the subthalamic nucleus. It is also associated with certain medications, such as antipsychotics and antiseizure medications.
Symptoms can be managed with physical and occupational therapy, a healthy diet, and other therapies. Some patients benefit from taking a medication called tetrabenazine (Xenazine(r), Austedo(r)), which is approved for treating chorea and athetosis. If you have a movement disorder, your healthcare provider will determine whether tetrabenazine is right for you. Your healthcare provider will monitor your progress while you are taking this medicine. They will check if the medicine is working and can recommend other ways to improve your symptoms. Your healthcare provider will also watch for side effects such as a feeling of drowsiness, dry mouth, and constipation. They will give you tips for preventing these side effects.
