Athetosis, an extrapyramidal sign, is characterized by slow, continuous, and twisting involuntary movements. Typically, these movements involve the face, neck, and distal extremities, such as the forearm, wrist, and hand. Facial grimaces, jaw and tongue movements, and occasional phonation are associated with neck movements. Athetosis worsens during stress and voluntary activity, may subside during relaxation, and disappears during sleep. Commonly a lifelong affliction, athetosis is sometimes difficult to distinguish from chorea (hence the term choreoathetosis). Typically, though, athetoid movements are slower than choreiform movements. (See Distinguishing athetosis from chorea.)
Athetosis usually begins during childhood, resulting from hypoxia at birth, kernicterus, or a genetic disorder. In adults, athetosis usually results from vascular or neoplastic lesions, degenerative disease, drug toxicity, or hypoxia.
Begin your neurologic evaluation by taking a comprehensive prenatal and postnatal history, covering maternal and child health, labor and delivery, and possible trauma. Obtain a family health history because many genetic disorders can cause athetosis. Also, ask about current drug therapy.
Ask about the decline in the patient’s functional abilities: When was he last able to roll over, sit up, or carry out daily activities? Find out what problem—uncontrollable movements, mental deterioration, or a speech impediment—prompted him to seek medical help. Ask about the effects of rest, stress, and routine activity on his symptoms.
Test the patient’s muscle strength and tone, range of motion, fine muscle movements, and ability to perform rapidly alternating movements. Observe the limb muscles during voluntary movements, noting the rhythm and duration of contraction and relaxation.
A brain tumor that affects the basal ganglia causes contralateral choreoathetosis and dystonia. Associated signs vary markedly with the type of tumor and its degree of invasion.
Calcification of the basal ganglia is a unilateral or bilateral disorder that’s characterized by choreoathetosis and rigidity. It usually arises in adolescence or early adulthood.
In cerebral infarction, contralateral athetosis is accompanied by altered level of consciousness. The patient may also display contralateral paralysis of the face or limbs.
Episodic or persistent choreoathetosis occurs in the chronic stage of hepatic encephalopathy and is accompanied by cerebellar ataxia, myoclonus of the face and limbs, asterixis, dysarthria, and dementia.
Huntington’s disease is a hereditary degenerative disease in which athetosis and chorea develop progressively in middle-aged adults. Accompanying signs and symptoms include dystonia, dysarthria, facial apraxia, rigidity, depression, and progressive mental deterioration leading to dementia.
Wilson’s disease is an inherited metabolic disorder in which choreoathetoid movements initially involve the fingers and hands and then spread to the arms, head, trunk, and legs. Associated signs and symptoms include Kayser-Fleischer rings (rusty brown rings around the corneas), arm and hand tremors, facial and muscular rigidity, dysarthria, dysphagia, drooling, and progressive dementia. Hepatomegaly, splenomegaly, jaundice, hematemesis, and spider angiomas may also occur.
Toxic levels of these drugs may cause athetoid or choreoathetoid movements.
The piperazine derivatives, such as meclizine and prochlorperazine, commonly cause athetosis. The aliphatic phenothiazines, such as chlorpromazine, occasionally cause it. A third type of derivative, the piperidine phenothiazines, such as thioridazine and perphenazine, rarely cause it. Other antipsychotics, such as haloperidol, thiothixene, and loxapine, commonly cause athetosis.
Prepare the patient for diagnostic tests, such as urine and blood studies, lumbar puncture, EEG, computed tomography scan, and magnetic resonance imaging.
Occasionally, athetosis can be prevented or treated (by decreasing body copper stores in Wilson’s disease or by adjusting drug dosages). Typically, though, it has a lifelong impact on the patient’s ability to carry out even routine activities. As a result, you’ll need to help him adapt to his condition—for example, by supplying him with assistive devices to help him carry out fine-motor tasks.
When appropriate, assist with rehabilitation; some patients can be taught to control erratic movements or convert them into purposeful ones. Also, encourage swimming, stretching, and balance and gait exercises to help maintain coordination, slow deterioration, and minimize antisocial behavior.
Encourage the patient and his family to discuss their feelings about athetosis and its cause. Refer the patient to a self-help group and appropriate support services such as physical therapy.
Childhood athetosis may be acquired or inherited. It can result from hypoxia at birth, which causes athetoid cerebral palsy; kernicterus; Sydenham’s chorea (in school-age children); and paroxysmal choreoathetosis. Inherited causes of athetosis include Lesch-Nyhan syndrome, Tay-Sachs disease, and phenylketonuria.
Help the child develop self-esteem and a positive self-image. Encourage the child and his family to set realistic goals, tailoring educational plans to the child’s level of intelligence.
Refer the child to special education services, rehabilitation centers, and support groups. Provide him with emotional support during the frequent medical evaluations required for athetosis.
Read excerpts from these other book chapters related to Chorea:
Copyright Details: Professional Guide to Signs & Symptoms (Fifth Edition), Copyright © 2008 Williams & Wilkins.
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More About This Book:
Title: Professional Guide to Signs & Symptoms (Fifth Edition) Authors: Springhouse Publisher: Lippincott Williams & Wilkins Copyright: 2006 ISBN: 1-58255-510-9 
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