What IS Torsion Dystonia Disease;( Dystonia Musculorum Deformans)

Torsion Dystonia is a rare disease characterized by inter sustained torsion spasms of the musculature w resultant marked abnormalities of bodily postu. The dystonic movements may involve any or of the musculature, but have a predilection the trunk and shoulder and pelvic girdles. Though dystonic movements occur with hep a lenticular degeneration, postencephalitic Parson’s, and tumors or other diseases involving basal ganglia, dystonia musculorum deformans a distinct clinical entity. The cause and mor1 anatomy of this condition are unknown. A here tary basis has been suggested for some forms this disorder. Glial scarring in the basal ganglia thalamus, and cortex has been described in so: cases, and some have been associated with sta: marmoratus and dysmyelination, as indicated the article on Athetosis.

The onset of the disease is variable and gradu and usually occurs between the ages of 5 and 1. years. Most frequently it begins with intermittent. spasmodic inversion of the foot, so t on walking the child finds difficulty in placing the heel on the ground. Bizarre stepping or a bowing gait may be noted when the dystonic movements affect the more proximal muscles the leg or the spine. As the movements become’ more intense and the proximal musculature is more prominently involved, lordosis and to pelvis appear. If the muscles of the neck and shoulder girdle are involved, torticollis is an early finding. Facial grimacing and difficulties in s become evident as the muscles subserving the functions become involved. The continuous spa. over a period of time result in marked distort: of the body of a degree rarely seen in any o disease process. Although muscle tone and p; appear to be normal, the involuntary movements interfere with function to such a d as to make them useless. No changes in the tendon reflexes occur, and mentation re; normal.

You Must know The Early stages of Torsion Dystonia Disease

In its early stages dystonia musculorum mans must be differentiated from other ment disorders in which dystonic pheno may occur. The history and physical findings out hepatolenticular degeneration and ep encephalitis. The age at onset and the involve­ment of proximal musculature in the movements are enough to differentiate it from athetosis. Chorea is characterized by movements more rapid and of shorter duration than those of dys­tonia. Hysteria is often a consideration: if it is not positively established by the personality characteristics of the ‘ patient, the course of the disease will soon suggest the correct diagnosis.

There is extreme variability in the rate of pro­gression and eventual disability. In its early phases there may be complete remission of symp­toms or lack of progression of initial symptoms for intervals of up to five years. Because the natural history 15 subject to wide variations, evaluation of the effects of treatment is difficult. Dystonic movements have been effectively con­trolled for varying periods of time by both drugs and surgical intervention. The medicines effec­tive are anticholinergic agents such as those indicated for the treatment of parkinsonism, com­bined with sedative agents such as phenobarbital.

In mild cases a combination of these drugs with some general supportive psychotherapy may be enough to control the patient’s symptoms for years. In more serious cases thalamotomy has produced encouraging results. However, because rather extensive lesions must be placed in the thalamic nuclei, multiple operative proce­dures are required to produce the desired effect. Although the operations are well tolerated by most patients, they do carry an element of risk, and are indicated only in selective cases. Levo­dopa has been reported as being effective in se­lected cases. In general a lower daily dose (3 grams per day) than that used in parkinsonism has been required.

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