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1/16. Functional recovery after bilateral pallidotomy for the treatment of early-onset primary generalized dystonia.

    This report describes the successful treatment of dystonia musculorum deformans with bilateral stereotactic pallidotomy in a 14-year-old girl in whom the dystonia was diagnosed when she was 7 years old. The patient presented with dystonia of the right upper extremity that progressed to generalized dystonia. Preoperatively, she required maximal assistance with all activities of daily living and transfers. She was not a functional ambulator. Postoperatively, she had remarkable functional recovery. At discharge, she was at modified independence level for all basic activities of daily living and required supervision for household ambulation. No postoperative complications were noted. We propose that bilateral stereotactic lysis of globus pallidus interna may be an alternative treatment for dystonia musculorum deformans. The technique of bilateral pallidotomy and theories of its effectiveness are discussed.
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2/16. Spinal lordosis with marked opisthotonus secondary to dystonia musculorum deformans: case report with surgical management.

    STUDY DESIGN: A case report of severe spinal lordosis with marked opisthotonus and retrocollis secondary to dystonia musculorum deformans is presented. OBJECTIVE: To describe a case of dystonia musculorum deformans with progressive spinal lordosis and its surgical treatment. SUMMARY OF BACKGROUND DATA: Four patients with correction of coronal spinal deformity associated with dystonia musculorum deformans have been reported in the literature. No reports of sagittal spinal deformity treated with surgical instrumentation and fusion were found. methods: A retrospective chart and radiographic review of a single case was conducted. RESULTS: Orthotic management and pharmacologic therapy with botulinum toxin injections were unsuccessful in controlling the deformity. Severe spinal lordosis (170 degrees ) from occiput to sacrum was corrected surgically, allowing an upright posture. CONCLUSION: dystonia musculorum deformans is a rare condition resulting in coronal or sagittal plane deformities. When other treatment methods are unsuccessful, surgical instrumentation and arthrodesis may correct the deformity and improve function.
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3/16. brain neurotransmitters in dystonia musculorum deformans.

    We examined histologically and biochemically the brains of two patients with generalized childhood-onset dystonia musculorum deformans. We found no important histologic changes in the basal ganglia, cerebral cortex, higher brain-stem nuclei, locus ceruleus, or raphe nuclei. Similarly, the activity of choline acetyltransferase and the levels of gamma-aminobutyric acid and glutamic acid in the cerebral cortex and basal ganglia were within the control range. In contrast, the norepinephrine concentrations were markedly and consistently decreased in the lateral and posterior hypothalamus, mamillary body, subthalamic nucleus, and locus ceruleus. The serotonin level was subnormal in the dorsal raphe nucleus, as was the dopamine level in the nucleus accumbens and, in one of the two cases, in the striatum. Elevated concentrations of norepinephrine were found in the septum, thalamus, colliculi, red nucleus, and dorsal raphe nucleus; of serotonin, in the globus pallidus, subthalamic nucleus, and locus ceruleus; and of 5-hydroxyindoleacetic acid, in the globus pallidus, subthalamic nucleus, and nuclei raphe centralis inferior and obscurus. The level of homovanillic acid showed little consistent change in the regions examined. We conclude that some of these monoamine changes, especially the pronounced apparent disturbance of noradrenergic brain mechanisms, may represent a basic neurochemical abnormality in dystonia musculorum deformans and may thus be relevant to the pathoneurophysiology and treatment of this disorder.
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keywords = deformans
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4/16. Behavioral covariation in the treatment of chronic pain.

    The successful use of operant procedures to alter behaviors associated with various medical conditions suggests that such behaviors may be learned and that the principles of learning may be applied not only to treatment but also to the study of the pathogenesis of illness behavior. The present study, conducted within an ongoing neuromuscular research project, assessed the covariation of behaviors associated with chronic pain within and across behavioral and drug approaches to treatment. Problems of screaming and five other behaviors (including self-reports of pain) were measured across conditions of varying behavioral contingencies (noncontingent reinforcement vs the removal of reinforcement contingent upon screaming) and varying administration (time since medication and dosage) of Parsidol during attempts to treat the muscle pain of a 24-year-old male with a severe, chronic neuromuscular disorder diagnosed as dystonia musculorum deformans (DMD). Results indicated that: (a) pain behaviors covaried during behavioral and drug conditions even though the behavioral intervention only targeted screaming; (b) effects were greater on nontargeted behaviors during periods that followed rather than preceded drug administration; (c) in contrast to behavioral observation data, physiological measures of neuromuscular activity (EMG) did not differ across conditions. These results suggest that functional response-response relationships exist in patients as the result of their illness experience.
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5/16. 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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ranking = 2.5
keywords = deformans
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6/16. 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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ranking = 2.5
keywords = deformans
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7/16. Orthotic technique for dystonia musculorum deformans.

    Tone reducing, inhibitive casting, and orthoses have been effectively used in patients with cerebral palsy and head injury to improve gait patterns and decrease tone. We present a patient with dystonia musculorum deformans who had severe inversion and supination of his left foot with weight bearing. He did poorly with metal double-upright ankle-foot orthoses with lateral T-strap. A tone-reducing ankle-foot orthosis (TRAFO) was successful in decreasing problems with abrasions and allowing him to walk without assistive devices.
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ranking = 2.5
keywords = deformans
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8/16. pathology in brainstem regions of individuals with primary dystonia.

    Examination of brains from four individuals with the clinical diagnosis of primary dystonia revealed histopathologic abnormalities in two cases. A 29-year-old man with a 15-year history of dystonia musculorum deformans (DMD) had numerous neurofibrillary tangles (NFT) and mild neuronal loss within the locus ceruleus; occasional NFT were also recognized in the substantia nigra pars compacta, pedunculopontine nucleus, and dorsal raphe nucleus. A 68-year-old man with a 35-year history of meige syndrome had moderate-to-severe neuronal loss in several brainstem nuclei, including the substantia nigra pars compacta, locus ceruleus, raphe nuclei, and pedunculopontine nucleus. Infrequent NFT were also noted in substantia nigra. An examination of these and other brain regions in a 10-year-old boy with a 6-year history of DMD and a 50-year-old woman with a 3-year history of spasmodic torticollis did not disclose similar abnormalities.
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9/16. Autosomal recessive idiopathic torsion dystonia in a kindred of mixed ancestry.

    A family with idiopathic torsion dystonia (dystonia musculorum deformans) was seen in a peripheral clinic in the Richtersveld in the north-western Cape. This rare inherited form of neurological disease has maximal prevalence in individuals of Ashkenazi Jewish ancestry, and there is controversy regarding the exact mode of inheritance. The kindred documented in this study was of mixed ancestry (Cape Coloured), and autosomal recessive inheritance was suggested by the finding of affected male and female siblings born to normal parents in an isolated inbred community.
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keywords = deformans
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10/16. Idiopathic torsion dystonia associated with lesions of the basal ganglia.

    We report two siblings who are suffering from a dystonic syndrome, clinically indistinguishable from idiopathic torsion dystonia (dystonia musculorum deformans) but with cranial computerized tomographic scan findings of basal ganglia lesions, similar to that reported in Wilson's disease. The occurrence of the disorder in the same sibship suggests an autosomal recessive mode of inheritance and may represent another variety of the syndrome of idiopathic torsion dystonia.
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