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Results for “DYSTONIA MUSCULORUM DEFORMANS”

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At least 19 recordsLinked to original sources
PubMed1990

[Torticollis as an initial symptom of adult-onset dystonia musculorum deformans].

Dystonia musculorum deformans (DMD) is an idiopathic movement disorder which usually involves pediatric age group and progresses to the generalized type. On the contrary in adult-onset DMD, dystonia is usually confined to an upper extremity and its clinical course is benign. The authors report seven patients with adult-onset DMD whose initial symptom was confined to the neck. Diagnosis of idiopathic spasmodic torticollis had been made in all of them. Average ages at the onsets of torticollis and extranuchal dystonia were 49 +/- 13 and 54 +/- 9 years (mean +/- SD) respectively. The duration between these onsets was 2-3 years in five patients and 10-17 years in two younger patients. Two patients finally developed generalized dystonia and one patient became hemidystonic type. These findings suggest that some patients diagnosed as idiopathic spasmodic torticollis are in an early stage of DMD and that this particular type progresses more likely to the generalized form than other types of adult-onset DMD.

Adult↗
PubMed1979

MMPI characteristics associated with cerebral palsy and dystonia musculorum deformans.

The origins of dystonia musculorum deformans are now considered to be organic. However, misdiagnosis of dystonia as a functional psychiatric disorder--usually conversion reaction--has persisted. The present study describes personality traits as measured by the Minnesota Multiphasic Personality Inventory in 30 persons with dystonia and in a control group of 37 persons with cerebral palsy. The data, examined by diagnosis, level of disability, and sex, showed no differences for diagnostic groups or levels of disability. Males scored in the direction of greater psychopathology than did females. The male dystonics showed the highest elevations of MMPI scales of all the groups. Although only one person with dystonia musculorum deformans and none with cerebral palsy produced the profile usually associated with conversion reaction, 36% of all profiles showed two scales above a T score of 70. This finding suggested that young adults with a physically disabling disease may be at higher risk for developing maladaptive personality traits.

Adult↗
PubMed1985

[Experience with L-DOPA treatment of children with dystonia musculorum deformans and dystonic hyperkineses in infantile cerebral palsy].

Nineteen children with dystonia musculorum deformans and dystonic hyperkineses were treated by L-DOPA. A mild positive effect was obtained in 6 children, a considerable positive effect in 9 children. A more marked positive effect was obtained in children with dystonia musculorum deformans. The results indicate the efficiency of L-DOPA in the treatment of dystonia musculorum deformans and dystonic hyperkineses against the residual organic background. The advisability of 2-3 month long courses of therapy with L-DOPA 2-3 times a year is discussed. Some patients may need a continuous therapy with maintenance doses of the drug.

Adolescent↗
PubMedDate not supplied

Relationship between acquired spastic talipes equinovarus and dystonia musculorum deformans.

Acquired spastic talipes equinovarus associated with dystonia musculorum deformans (DMD) presents orthopaedic surgeons with a difficult treatment problem. We reviewed the natural history, diagnostic evaluation, and treatment of this deformity in 12 patients. Two patients initially had the generalized form of dystonia, and 10 patients had the focal form (in which acquired spastic talipes equinovarus was the only manifestation). With time, five of these 10 developed the generalized form. A positive family history and age < 14 years at onset were prognostic of this progression.

Adolescent↗
PubMed2001

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.

Adolescent↗
PubMed1988

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.

Adult↗
PubMedDate not supplied

[Diagnostic and therapeutic problems in dystonia musculorum deformans].

Taking as starging point three patients who unsuccessfully underwent both medical treatment and different neurosurgical interventions, the Authors discuss the problem of the dystonia musculorum deformans nosographic arrangement and its present therapeutical possibilities. They believe that the dystonia musculorum deformans must be considered, rather than as a syndrome, as a real disease, to be distinguished in particular from the various infantile dystonic syndromes and from spasmodic torticollis. In fact in these last morbid forms, at least from a neurosurgical standpoint, the therapeutical possiblities are not so uncertain as those of the dystonia musculorum deformans.

Adolescent↗
PubMed1986

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.

Acetyltransferases↗
PubMed1983

Polysomnographic findings in dystonia musculorum deformans.

Twenty-five cases from the literature and four personal cases with dystonia musculorum deformans are described. The polysomnographic EEG findings of severely involved patients were characterized by over abundance of stage 2 sleep, with a characteristic pattern of spindle activity, increased latency to sleep, and reduced sleep efficiency. The similarities between these patients may underscore the importance of sleep spindles as a feature of the dystonia process and may be useful in the investigation of other basal ganglia disorders.

Adult↗
PubMed1996

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.

Adolescent↗
PubMed1983

[Dystonia musculorum deformans. Favorable effect of bromocriptine].

A 25 year-old woman suffered from a severe dystonia musculorum deformans since the age of 14. No similar cases were recorded in the family. Examination showed torsion spasm and a cogwheel phenomenon in both superior limbs. Signs disappeared on bromocriptine 22,5 mg/day then reappeared when dosages where progressively reduced. With 12.5 mg/day after 1 year of treatment, the patient leads a normal life including university studies.

Adult↗
PubMed1982

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.

Adult↗
PubMedDate not supplied

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.

Adolescent↗