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11/16. The idiopathic dystonias. A note on their orthopaedic presentation.

    Eight patients suffering from various forms of idiopathic dystonia are described whose initial referral was for an orthopaedic opinion. The diagnoses of these patients, who were seen over a two-year period, comprised dystonia musculorum deformans, dystonia of the foot, spasmodic torticollis and occupational cramps. Although various musculoskeletal sequelae often occur, the primary underlying neurological cause of these unusual conditions is emphasised.
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keywords = deformans
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12/16. Severe dystonia and myoglobinuria.

    myoglobinuria may follow extreme muscular exertion or disorders that cause muscle necrosis. Dystonia has not been implicated previously. We studied an 8-year-old boy of non-Jewish, Mexican-American descent with autosomal-dominant dystonia musculorum deformans who developed rapidly progressive and severe generalized dystonia, hyperpyrexia, myoglobinuria, and renal failure. Curarization was required. Transient improvement was achieved with tetrabenazine and baclofen, but bilateral thalamotomy was then performed. patients with severe dystonia should be observed for evidence of myoglobinuria.
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keywords = deformans
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13/16. dystonia musculorum deformans: implications of therapeutic response to levodopa and carbamazepine.

    Sporadic, autosomal recessive and dominant forms of dystonia musculorum deformans have been recognized. This communication reports the results of treatment of six patients with this condition. Two patients with the recessive form responded to levodopa therapy. Three patients who responded to carbamazepine therapy probably have the dominant form. In one patient, response to carbamazepine therapy was equivocal. It is suggested that such therapeutic responsiveness may reflect underlying biochemical differences in the recessive and dominant forms of the disease.
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ranking = 5
keywords = deformans
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14/16. Fluctuating dystonia and allied syndromes.

    dystonia musculorum deformans is a descriptive diagnosis. A number of other conditions such as Hallervorden-Spatz disease and juvenile paralysis agitans have to be excluded. Then the history of a child's illness may suggest a particular syndrome such as the progressive dystonia with marked diurnal fluctuations. Two case reports are given of children who show fluctuation of symptoms but their histories varied from those previously described. Problems of treatment are discussed and it is suggested that fluctuation of symptoms may indicate a response to levadopa while those with a relentless progression of symptoms may respond to other drugs such as orphenadrine.
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keywords = deformans
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15/16. Simultaneous bilateral pallidoansotomy for idiopathic dystonia musculorum deformans.

    A 17-year-old Russian male with a 9-year diagnosed history of dystonia musculorum deformans manifested as severe tortipelvis, lordosis, and axial and appendicular spastic dystonia, refractory to medical therapy, is reported. This patient underwent a simultaneous bilateral pallidoansotomy with dramatic results. Postoperative evaluation revealed sustained alleviation of all dystonic symptoms and abnormal movements. Rapid recovery of useful strength in all limbs as well as dramatic improvement in coordination occurred. Bilateral posteroventral pallidotomy and pallidoansotomy in the past have proven effective in alleviation of all parkinsonian symptoms, including dyskinesia and dystonia, without the concurrent risk of intransigent side effects associated with bilateral thalamotomy or other stereotactic surgical procedures. Pallidoansotomy may prove to be the treatment of choice for idiopathic torsion dystonia and merits further investigation.
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ranking = 5
keywords = deformans
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16/16. Psychological studies in dystonia musculorum deformans.

    Sixty-eight patients with DMD underwent psychometric assessment before or after thalamic surgery. Statistical comparisonss between categories of patients were undertaken as a function of religion, family history, and age at onset of symptoms as independent variables. It was found that Jewish patients, with negative family history, and age at onset of symptoms from 9 to 13 years, scored significantly higher on IQ tests than did all other groups. Other psychological test data revealed no specific personality patterns in the DMD patients, particularly no pattern of conversion hysteria or hysteria. Pre- and postoperative psychological testing in a small group of patients revealed no evidence of any changes in intellectual, cognitive, or personality functions as a consequence of thalamic surgery, involving up to 10 lesions in some instances.
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keywords = deformans
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