Dystonia
Dystonia: Excerpt from Professional Guide to Signs & Symptoms (Fifth Edition)
Dystonia is marked by slow, involuntary movements of large-muscle groups in the limbs, trunk, and neck. This extrapyramidal sign may involve flexion of the foot, hyperextension of the legs, extension and pronation of the arms, arching of the back, and extension and rotation of the neck (spasmodic torticollis). It’s typically aggravated by walking and emotional stress and relieved by sleep. Dystonia may be intermittent—lasting just a few minutes—or continuous and painful. Occasionally, it causes permanent contractures, resulting in a grotesque posture. Although dystonia may be hereditary or idiopathic, it usually results from extrapyramidal disorders or the use of certain drugs.
History and physical examination
If possible, include the patient’s family in history taking; they may be more aware of behavior changes than the patient is. Begin by asking them when dystonia occurs. Is it aggravated by emotional upset? Does it disappear during sleep? Is there a family history of dystonia? Obtain a drug history, noting especially the use of a phenothiazine or an antipsychotic. Dystonia is a common adverse effect of these drugs, and the dosage may need to be adjusted to minimize this effect.
Next, examine the patient’s coordination and voluntary muscle movement. Observe his gait as he walks across the room; then have him squeeze your fingers to assess muscle strength. (See Recognizing dystonia.) Check coordination by having him touch your fingertip and then his nose repeatedly. Follow this by testing gross motor movement of the leg: Have him place his heel on one knee, slide it down his shin to the top of his great toe, and then return it to his knee. Finally, assess fine-motor movement by asking him to touch each finger to his thumb in succession.
Medical causes
Alzheimer’s disease
Dystonia is a late sign of this disorder, which is marked by slowly progressive dementia. The patient typically displays decreased attention span, amnesia, agitation, an inability to carry out activities of daily living, dysarthria, and emotional lability.
Dystonia musculorum deformans
Prolonged, generalized dystonia is the hallmark of this disorder, which usually develops in childhood and worsens with age. Initially, it causes foot inversion, which is followed by growth retardation and scoliosis. Late signs include twisted, bizarre postures, limb contractures, and dysarthria.
Hallervorden-Spatz disease
This degenerative disease causes dystonic trunk movements accompanied by choreoathetosis, ataxia, myoclonus, and generalized rigidity. The patient also exhibits a progressive intellectual decline and dysarthria.
Huntington’s disease
Dystonic movements mark the preterminal stage of Huntington’s disease. Characterized by progressive intellectual decline, this disorder leads to dementia and emotional lability. The patient displays choreoathetosis accompanied by dysarthria, dysphagia, facial grimacing, and a wide-based, prancing gait.
Olivopontocerebellar atrophy
Ataxia, an early sign in this rare disorder, slowly progresses to dystonia. Other findings include dysarthria, action tremor, bradykinesia, and visual deterioration.
Parkinson’s disease
Dystonic spasms are common in this disease. Other classic features include uniform or jerky rigidity, pill-rolling tremor, bradykinesia, dysarthria, dysphagia, drooling, masklike facies, monotone voice, stooped posture, and a propulsive gait.
Pick’s disease
Dystonia appears as a late sign in this rare disorder, which resembles Alzheimer’s disease.
Supranuclear ophthalmoplegia
Also known as Steele-Richardson-Olszewski syndrome, this rare disorder affects mainly middle-aged people, causing intermittent dystonia with extreme neck flexion or extension. Other signs and symptoms include impaired extraocular movement, diminished voice volume, dysarthria, truncal rigidity, dementia, ataxia, masklike facies, and dysphagia.
Wilson’s disease
Progressive dystonia and chorea of the arms and legs mark this disorder. Other common signs and symptoms include hoarseness, bradykinesia, behavior changes, dysphagia, drooling, dysarthria, tremors, and Kayser-Fleischer rings (rusty-brown rings at the periphery of the cornea).
Other causes
Drugs
. Phenothiazines can cause dystonia. Aliphatics such as chlorpromazine cause it occasionally, and piperidines rarely cause it.
Haloperidol, loxapine, and other antipsychotics usually produce acute facial dystonia, as do risperidone, metyrosine, antiemetic doses of metoclopramide, and excessive doses of levodopa.
Special considerations
Encourage the patient to obtain adequate sleep and avoid emotional upset. Avoid range-of-motion exercises, which can aggravate dystonia. If dystonia is severe, protect the patient from injury by raising and padding his bed rails. Provide an uncluttered environment if he’s ambulatory.
Pediatric pointers
Children don’t exhibit dystonia until after they can walk. Even so, it rarely occurs until after age 10. Common causes include Fahr’s syndrome, dystonia musculorum deformans, athetoid cerebral palsy, and the residual effects of anoxia at birth.
Pictures
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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- Dystonia
- "Professional Guide to Signs & Symptoms (Fifth Edition)" (2006)
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Copyright notice for book excerpts: Copyright © 2008 Lippincott Williams & Wilkins. All rights reserved.
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