Chorea [Choreiform movements]

Chorea—brief, unpredictable bursts of rapid, jerky motion that interrupt normal coordinated movement—indicates dysfunction of the extrapyramidal system.
Unlike tics, choreiform movements are seldom repetitive but tend to appear purposeful despite their involuntary nature. Although any muscle can be affected, chorea usually involves the face, head, lower arms, and hands. It can affect both sides of the body or only one side; however, when it affects the face, both sides are usually involved. Chorea may be aggravated by excitement or fatigue and may disappear during sleep. In some patients, it may be difficult to distinguish chorea from athetosis (snakelike, writhing movements), although choreiform movements are generally more rapid than athetoid ones. (See Distinguishing athetosis from chorea, page 82.)  

History and physical examination

Ask the patient and his family when they first noticed the choreiform movements. Do the movements disappear when the patient is asleep? Find out if anyone in the patient’s family exhibits the same type of movements, and ask about a family history of such diseases as Huntington’s disease. Also ask which medications the patient is taking. Obtain an occupational history, noting especially prolonged exposure to manganese or other metals. As you obtain the history, observe the patient for excessive restlessness and periodic facial grimaces that may interrupt his speech.

Perform a physical examination to evaluate the severity of the patient’s chorea. Ask him to stick out his tongue and keep it out. Typically, he’ll be unable to do this; instead, his tongue will dart in and out of his mouth. Observe the patient’s arms and legs separately for involuntary jerky movements. Ask him to extend and flex his hand as if halting traffic; the choreiform movements will be extremely evident in this position. Also, check for such related signs as athetosis, rigidity, or tremor.

To assess the patient for choreoathetotic gait, ask him to walk. He may change the position of his trunk and upper body parts with each step and jerk and tilt his head to one side. Because of superimposed involuntary movements and postures, the patient’s legs may move slowly and awkwardly. (An involuntary movement suspending his leg momentarily with each step may give a dancing quality to his gait.)

Medical causes

Cerebral infarction

An infarction that involves the thalamic area produces unilateral or bilateral chorea. The patient may also experience dysarthria, tremors, rigidity, weakness, and sensory disturbances such as paresthesia.

Encephalitis

Chorea may occur in the recovery phase of encephalitis. Low-grade fever and athetosis may also be present, in addition to such focal neurologic signs as hemiparesis, hemiplegia, and facial droop.

Huntington’s disease

In this inherited disease, chorea may be the first sign or it may accompany the intellectual decline that leads to emotional disturbances and dementia. The patient’s movements tend to be choreoathetotic and may be accompanied by dysarthria, dystonia, prancing gait, dysphagia, and facial grimacing.

Wilson’s disease

Chorea and dystonia affecting the arms and legs are early indicators of Wilson’s disease. The patient typically experiences dysarthria, tremors, hoarseness, dysphagia, and slowed body movements; he may also exhibit emotional and behavioral disturbances, drooling, rigidity, and mental deterioration. The pathognomonic Kayser-Fleischer ring in the cornea appears as the disease progresses.

Other causes

Carbon monoxide poisoning

A patient who survives severe carbon monoxide poisoning may have neurologic signs and symptoms, such as chorea, rigidity, dementia, impaired sensory function, masklike facies, generalized seizures, and myoclonus.

Drugs

Phenothiazines (especially the piperazine derivatives), haloperidol, thiothixene, and loxapine commonly produce chorea. Metoclopramide, metyrosine, hormonal contraceptives, levodopa, and phenytoin may also cause this sign.

Lead poisoning

In the later stages, lead poisoning produces chorea in addition to seizures, headache, memory lapses, and severe mental impairment. The patient may also develop masklike facies, footdrop, wristdrop, dizziness, ataxia, weakness, lethargy, abdominal pain, anorexia, nausea, vomiting, constipation, lead line on the gums, and a metallic taste in his mouth.

Manganese poisoning

In miners who have been exposed to manganese dioxide for prolonged periods, chorea characteristically occurs with a propulsive gait, dystonia, and rigidity. Initially, the patient may have masklike facies, a resting tremor, and personality changes; later, extreme muscle weakness and lethargy occur.

Special considerations

Because the patient’s movements are involuntary and increase his risk of severe injury, pad the side rails of his bed and keep sharp objects out of his environment. Help him minimize physical activity and emotional upset to avoid aggravating the chorea and ensure adequate periods of rest and sleep.

Pediatric pointers

Sydenham’s chorea occurs in childhood as a delayed manifestation of rheumatic fever. In Hallervorden-Spatz disease, a rare and inherited degenerative disorder, choreoathetotic movements occur in late childhood or early adolescence. Chorea can also occur in children with athetoid cerebral palsy.

Book Source Details

• Book Title: Professional Guide to Signs & Symptoms (Fifth Edition)
• Author(s): Springhouse
• Year of Publication: 2006
• Copyright Details: Professional Guide to Signs & Symptoms (Fifth Edition), Copyright © 2006 Lippincott Williams & Wilkins.

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