What is dystonia? Dystonia is an extrapyramidal disease associated with abnormal prolonged twisting movement. It is characterized by involuntary slow movements of the large muscle groups such as the neck, trunks and limbs. Dystonia may involve spasmodic torticollis or extension and rotation of the neck, hyperextension of the legs, arching of the back, pronation and extension of the arms and flexion of the foot. Dystonia may occur continuously and a patient may also complain of pain or dystonia that occur intermittently (that last for a few minutes). Dystonia is associated with drugs, extrapyramidal disorder, hereditary or idiopathic. Dystonia may be precipitated by stressful condition or walking. However, dystonia is relieved by sleeping. On of the complication of dystonia include a grotesque posture as a result of permanent contracture.
A patient presenting with dystonia may require a series of assessment which include history and examination. Personal history from the patient is taken which include the drug history/intake such as the intake of anti psychotic or phenothiazine as these drugs may lead to dystonia. In this situation, the dose of the drugs should be adjusted to minimize the adverse effect. Questions such as any episodes of emotional upset (triggering factor) is also relevant. Besides that, the patient is also asked about the dystonia symptoms which are relieved by sleeping. Physician also should focus on the present of dystonia that run in the family. Family history is also important. Relatives or family members are asked about recent changes of the patient’s behavior.
The aim of the examination is to assess the voluntary muscle movement of the patient as well as the coordination of the patient. The patient is asked to walk across the room. The patient’s gait is observed. The strength of the muscle is assessed by asking the patient to squeeze the physician‘s hand. The patient is ordered to touch the physician‘s fingertips and then touch his /her nose repeatedly to assess the coordination of the patient. The examination also should focus on the gross motor examination and fine motor examination. The gross motor examination is performed by asking the patient to place his/her heel on the patient’s knee and slide it down the skin before return back to the knee. This is performed on both legs. The patient is asked to touch each finger and thumb in succession to assess the fine motor movement.
Dystonia, athetosis and chorea may occur at the same time. However, athetosis may involve athetoid movement which is writhing, sinuous and slow movement which is continuous and affects the extremities and hand. Jerky movement which is highly rapid is known as choreiform movements. Dystonic movement may occur continuously or intermittently. Dystonic movement is a twisting and slow movement which involves large muscle groups in the limbs, trunk, neck and the head.
Common causes of dystonia may include Wilson’s disease, Alzheimer’s disease, Parkinson’s disease, Dystonia Musculorum Deformans, Huntington’s disease and Hallervorden -Spatz disease.
Wilson’s disease sufferer may develop progressive chorea and dystonia which affect the arms and legs. Wilson’s disease may also associate with changes in behaviour, bradykinesia, hoarseness, tremor, dysarthria, drooling and dysphagia. The present of the rusty brown rings at the periphery of the cornea /Kayser Fleischer ring may indicate the presence of one of the complication of the Wilson’s disease.
Dystonia appear to be a late sign of a patient who suffers from Alzheimer’s disease. Alzheimer’s disease is usually presented with slowly progressive dementia. Symptoms and signs such as emotional lability, dysarthria, amnesia, decreased attention span and inability to perform daily work and agitation are also associated with Alzheimer’s disease.
Dystonic spasm is commonly present in Parkinson’s disease. Besides that other classic signs of Parkinson’s disease may include mask-like facies, dysarthria, drooling, bradykinesia, dysphagia, jerky/uniform rigidity, propulsive gait, stooped posture and monotone voice.
Dystonia musculorum deformans is a disorder commonly develops during childhood and getting worse as the children grow older. Generally dystonia musculorum deformans is characterized by generalized prolonged dystonia. Initially the patient’s foot is inverted and as the disease progress the patient may suffer from scoliosis and growth retardation. The late signs may include dysarthria, contracture of the limb and bizarre as well as twisted posture.
Huntington’s disease is characterized by progressive decline in mental function/intellectual level. This will later lead to emotional lability and dementia. The pre terminal stages of the Huntington’s disease are characterized by dystonic movement. The patient may also present with facial grimacing, dysphagia, dysarthria and prancing, wide based gait.
Hallervorden - Spatz disease is also presented with progressive decline in intellectual level. Hallervorden -Spatz disease is a degenerative disease. It is characterized by dystonic movement of the trunk, ataxia, generalized rigidity, myoclonus and chorea athetosis.
There are also a few drugs which leads to dystonia. Acute cases of facial dystonia is caused by intake of loxapine, haloperidol ( antipsychotic),metoclopramide (antiemetic), metyrosine, risperidone and excessive doses of levodopa. Dystonia may also related to 3 types of phenothiazines (drugs). Carphenazine and acetophenazine are piperazine phenothiazines drugs which is frequently caused dystonia. Other drugs such as piperidines and chlorpromazine rarely cause dystonia.
In children, dystonia is rarely present. Children typically don’t show any sign of dystonia before they able to walk or before 10 years of age. In children dystonia is commonly caused by athetoid cerebral palsy, Fahr’s syndrome, the residual effect of anoxia at birth and dystonia musculorum deformans.
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