Athetosis is the name given to a movement disorder. It is one of the hyperkinesias.
What is athetosis?
Medical professionals understand athetosis to be a form of movement disorder. It belongs to the group of extrapyramidal hyperkinesias. In this case, affected individuals suffer from slow and uncontrolled movements on their limbs that are screw-like. The hands are particularly affected. Most patients also develop chorea. This is a restlessness of movement that is accompanied by involuntary, rapid muscle contractions. In medicine, this process is called chorea athetosis. Athetosis is most frequently seen in children. A typical feature of the movement disorder is bizarre malpositions that alternate. In addition, unnatural overextension of the joints occurs. Athetosis can occur on only one side of the body, which is called hemiathetosis, or on both halves of the body (athetosis double). Athetosis is considered by some professionals to be simply distal dystonia or slow chorea. In most cases, the movement disorder is accompanied by other neurologic symptoms.
Causes
Athetosis, including chorea, is caused by damage to the basal ganglia pallidum and striatum. Basal ganglia are nuclei from the gray matter of the brain. They are located under the cerebral cortex. Their main function is to regulate motor function. The striatum (striate body) is composed of the caudate nucleus as well as the outer lenticular nucleus (putamen). Brain damage resulting in athetosis occurs in early childhood. A common cause is bilirubin encephalopathy (kernicterus). In this case, considerable damage occurs in the central nervous system of babies due to an above-average increase in bilirubin, which is a degradation substance of the red blood pigment. Another early childhood trigger is Little’s disease. This severe damage to the brain occurs before or during birth. Possible causes include complications during pregnancy, infections involving the brain, vascular occlusions in the brain region, or a lack of oxygen. However, athetosis can also occur in adults. In most cases, this is hemiathetosis. It is caused by a lenticular infarction, a special form of stroke. This occurs due to spontaneous reduced blood flow to the pallidum and putamen.
Symptoms, complaints, and signs
The main feature of athetosis forms a disturbance in tonic coordination. This leads to hyperkinetic movements. The patient performs uncontrolled, slow, and screwing movements of his or her hands, arms, legs, and trunk. The movements of the body occur involuntarily. In addition, muscle twitching occurs. Attention and mental arousal further intensify the restlessness of movement. In contrast, restless movements are usually absent during sleep. The movements often have a screw shape and occur randomly. Hands, neck and face are strongly affected. Typical symptoms are spreading of the fingers, grimacing and distortion of the mouth as well as overextension of the hands, which appear bizarre. Other conspicuous features include a cramped posture of the neck, twisting movements with the head, and increased salivation. The patient walks in a stumbling and overshooting manner. Not infrequently, there is a combination of athetosis and spasticity. In some joints, there is a risk of dislocation (luxation) due to the hypermobility.
Diagnosis and course
If athetosis is suspected in a child because of motor abnormalities or bizarre hand malpositions, a physician should be consulted. The same applies to adults who notice unusual movement disorders in themselves. The first step in diagnosis is to review the patient’s medical history. This involves asking the child’s parents about the symptoms and possible previous illnesses. The physician then performs a physical examination. He also uses imaging methods to look for possible damage to the brain that could be responsible for the movement disorder. Depending on the cause of the athetosis, further examinations are conceivable.Athetosis often leads to communicative misunderstandings in children in the further course, which are caused by speech disorders as well as involuntary gestures and facial expressions. As a result, emotional disturbances of the child are conceivable. In addition, sensorimotor development is delayed, causing affected individuals to walk late or only with assistive devices.
Complications
Athetosis has a very negative effect on the patient’s daily life and can restrict his or her life relatively severely. Here, very rapid and uncontrolled movements occur, which the patient cannot control himself. This can lead to severe problems, especially in children, who are bullied or teased because of the athetosis. In this case, depression and other psychological limitations occur. Due to the athetosis, the daily life of the affected person is restricted, many activities cannot be performed. Often, in addition to movements, there is uncontrolled salivation and bizarre movements or hyperextension of the limbs. Athetosis may cause the patient to involuntarily dislocate his or her joints or limbs. Direct treatment is not possible in athetosis, so there are no further complications in this case. However, it is possible to limit the bizarre movements and also to control and calm the breathing of the affected person. Through certain therapies, social problems can also be treated, so that there is no discomfort or problems here at an older age. However, athetosis does not have a negative effect on life expectancy. However, especially children with athetosis need special treatment to avoid being socially excluded.
When should you see a doctor?
In any case of athetosis, a doctor must be consulted. There is no self-healing in this disease and the affected persons are dependent on medical treatment in this case in any case. Without treatment, hyperextension or dislocation of the joints may also occur. Usually, involuntary movements and twitching of the muscles or joints represent complaints of athetosis and should be investigated. Cramps and pain may also occur in the affected regions. It is not uncommon for those affected by athetosis to also suffer from disorders of attention and concentration and, for example, be unable to follow lessons. Likewise, a doctor should be consulted in the event of hyperextension of the hands or legs. Increased salivation is also a symptom of athetosis and must be examined by a medical professional. It is not uncommon for those affected to also suffer from various spasticities and thus significant limitations in their lives. In the first instance, the general practitioner can be consulted for diagnosis. Further treatment of the individual complaints is then carried out by an appropriate specialist.
Treatment and therapy
There are limited options available for the treatment of athetosis. The primary goal of therapy is to alter postural control by controlling involuntary movements in a voluntary manner. At the same time, it is important to improve the patient’s breathing and support communication. It is also important to promote locomotion, whereby aids can also be used, and to facilitate food intake. Furthermore, sensorimotor and emotional-social sequelae should be prevented. Physiotherapeutic exercises are considered the best method for combating the movement disorder. This primarily includes the so-called Bobath method. The Bobath concept was developed in 1943 by the German physiotherapist Berta Bobath (1907-1991) and her husband, a neurologist, specifically for children and adults suffering from neurological disorders. The method involves special exercises designed to teach natural postures and movements. Some patients also receive drug therapy. Preparations such as clonazepam, haloperidol and tiapride are administered. In this way, a positive influence on athetosis should be achieved.
Outlook and prognosis
The prognosis for athetosis is not very optimistic. Recovery from symptoms does not occur with the disease. The brain damage cannot be cured according to current scientific knowledge. However, alleviation of symptoms is possible in some areas and is successfully practiced. The extent of the improvements is individual and depends on the severity of the disease.The treatment plan usually includes physiotherapy, nutritional counseling and psychotherapy. Strengthening the musculature and training the movement sequences helps to experience an improved sense of well-being in everyday life. An increase in the quality of life can also be observed in the patient with a healthy and optimally balanced diet. It is tailored to the needs of the body and thus to those of the musculature as well as the immune system. Emotional and mental issues are addressed by a therapist. Since the life span with athetosis is shortened, social exclusion is imminent and depression often develops, therapeutic support helps to cope with and process the circumstances in everyday life. In addition, a restructuring of the domestic situation is helpful to strengthen the well-being. Targeted exercises and aids that promote the learning of natural postures and movement sequences help the patient to cope with everyday life. In addition, drug treatment provides relief from some symptoms.
Prevention
Measures that serve to prevent athetosis are not known. This makes it all the more important to start therapy quickly as soon as the first signs of the movement disorder appear.
Follow-up
Athetosis requires prolonged therapy, which is usually followed by several years of aftercare. Causative treatment is not possible in many cases because the damage to the brain stems is permanent. Follow-up care focuses on regular progress checks, where the neurologist or orthopedist in charge will perform various ultrasound examinations and brain scans. This allows the health status to be accurately determined and treatment to be optimized. Follow-up care also includes comprehensive physiotherapy. This treatment often needs to be continued for years, depending on whether the athetosis continues to progress and which areas of the brain are affected as the disease progresses. Follow-up care also includes patient consultations. Depending on the severity of the disease, these can take place every four to eight weeks or every six months. If the course of the disease is severe, the physician will use the patient discussions to find possible causes for the progressively worsening symptoms. Since athetosis can have very different effects, follow-up always depends on the patient’s individual health status. Basically, brain scans, patient interviews, bone and joint examinations, and physiotherapeutic measures are performed. In addition, medication must be reviewed and adjusted if necessary to ensure optimal healing of athetosis.
What you can do yourself
Since athetosis cannot be cured, those affected are forced to come to terms with the condition. The earlier this happens, the better it can be dealt with. Self-help groups have proven to be helpful here, in which patients exchange their experiences and everyday tips. Furthermore, special physiotherapeutic exercises provide relief. These keep ligaments, muscles and joints supple. They also improve posture and breathing. Some of the supported movement patterns can also be performed at home. The so-called Bobath method, which focuses on special movement exercises in which posture and movement patterns are naturally relearned, is particularly suitable. A balanced and, if possible, relaxed lifestyle – in terms of diet and daily routine – is also indicated. Since movements are difficult to perform, the living situation should be adapted accordingly (keyword stairs). Any aids can also be applied for at the health insurance company. It is worth asking there in any case. Contact with other affected persons, but also with encouraging people from the normal environment, helps against emerging depressions. A big mistake would be the withdrawal from life and the self-chosen isolation. This would only cause those affected to focus exclusively on their illness and have little joy in life.
