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1/3. dystonia musculorum deformans: three cases treated on a rehabilitation unit.

    dystonia musculorum deformans is a rare involuntary movement disorder of unknown etiology. We treated three brothers in one family on our rehabilitation unit, resulting in definite improvement in their ability to walk and control the dystonic movements, and in their speech and swallowing. All three benefited from orthoses to prevent equinovarus and maintain ankle stability. Intense concentration, such as needlepoint, seemed to reduce the amount of extra motion. Performing another activity during dystonic movements, such as pointing to a communication board, also seemed to give some relief. A special seating system using a collapsible wheelchair, a low center of gravity, and antitip wheels reduced one brother's frequency of falling. Swallowing finely cut foods was not a problem with any of our patients, although one had to be instructed in a special technique. An electronic communication board was very helpful. Our success in these cases should encourage others working in rehabilitation to consider such patients and to apply the principles of rehabilitation, particularly the total team approach, in their care.
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keywords = communication
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2/3. rehabilitation of communication impairment in dystonia musculorum deformans.

    Augmentative and alternative communication (AAC) aids were used in three young, intellectually normal patients with dystonia musculorum deformans (DMD) who had severe speech and writing impediments. These aids included speech therapy, communication boards, and voice synthesizers for verbal communication and typewriters, memowriters, and computer software and printers for written communication. At times customized accessing was needed which required specific adaptive modifications. Implementation of the AAC aids system was determined effective for DMD patients in view of the intellect-sparing nature of the disorder. Improvement was hampered by the progressive nature of the disease and by the emotional stress of accepting the long-term use of AAC. Correct and early diagnosis of communication impediments are crucial for the appropriate AAC aids prescription and implementation. An AAC protocol is suggested to meet the special communication needs of DMD patients.
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keywords = communication
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3/3. 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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keywords = communication
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