Cases reported "Dystonic Disorders"

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11/91. Differences in somatosensory hand organization in a healthy flutist and a flutist with focal hand dystonia: a case report.

    Focal hand dystonia is a disabling, involuntary disorder of movement that can disrupt a successful musician's career. This problem is difficult to treat, to some extent because we do not fully understand its origin. Somatosensory degradation has been proposed as one etiology. The purpose of this case study was to compare the differences in the somatosensory hand representation of two female flutists, one with focal dystonia of the left hand (digits 4 and 5) and one a healthy subject (the control). Noninvasive magnetic source imaging was performed on both subjects. The somatosensory evoked potentials of controlled taps to the fingers were measured with a 37-channel biomagnetometer and reported in terms of the neuronal organization, latency, amplitude, density, location, and spread of the digits on each axis (x, y, and z). The somatosensory representation of the involved hand of the flutist with dystonia differed from that of the healthy flutist. The magnetic fields evoked from the primary somatosensory cortex had a disorganized pattern of firing, with a short latency and excessive amplitude in the involved digits of the affected hand, as well as inconsistency (decreased density). In addition, the patterns of firing were different in terms of the location of the digits on the x, y, and z axes and sequential organization of the digits. This study confirms that somatosensory evoked magnetic fields can be used to describe the representation of the hand on the somatosensory cortex in area 3b. Degradation in the hand representation of the flutist with focal hand dystonia was evident, compared with the hand representation of the healthy flutist. It is not clear whether the sensory degradation was the cause or the consequence of the dystonia. The questions are whether re-differentiation of the representation could be achieved with aggressive sensory retraining and whether improvement in structure would be correlated with improvement in function.
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keywords = dystonia
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12/91. Pseudodystonic hand posturing contralateral to a metastasis of the parietal association cortex.

    A 56 year-old patient, with a history of surgically removed breast cancer three years earlier, presented with incoordination of hand movements while playing piano. Neurological examination disclosed mild position sensory loss and limb-kinetic apraxia of the distal part of the right upper extremity. The most conspicuous neurological sign was a dystonic posturing of the right hand, which was only elicited when the patient outstretched her arms with the eyes closed. MRI revealed a metastatic lesion involving the left parietal cortex. The association of focal dystonic postures with lesions of the parietal association cortex indicates that dystonia may feature damage of brain cortical areas far from the basal ganglia. In addition, this provides support to the hypothesis that impairment of sensory pathways may play a role in the origin of some hyperkinetic movement disorders, such as dystonia and athetosis.
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ranking = 0.22222222222222
keywords = dystonia
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13/91. Sporadic paroxysmal exercise induced dystonia: report of a case and review of the literature.

    Sporadic paroxysmal exercise induced dystonia (PEID) is a rare condition. So far only fifteen cases have been reported in the world literature. It is characterised by dystonic posture on prolonged exercise, which gets relieved with rest. In general, these are refractory to medical treatment. We report one such case, along with review of the literature. The lower limbs were spared and only right shoulder was tucked up with the head turning to right side. The duration of exercise necessary to bring out the dystonic posture gradually diminished with time, a feature not reported previously.
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ranking = 0.55555555555556
keywords = dystonia
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14/91. SPECT imaging of the dopamine transporter in juvenile-onset dystonia.

    Juvenile-onset dystonia that improves after levodopa may occur in both dopa-responsive dystonia (DRD) and juvenile parkinsonism (JP), clinically similar conditions with different prognoses and management goals. The authors show normal striatal uptake of the dopamine transporter ligand FP-CIT with SPECT in a clinically atypical case of DRD, in contrast to the reduced uptake observed in JP.
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ranking = 0.66666666666667
keywords = dystonia
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15/91. Evidence for altered basal ganglia and cortical functions in transient idiopathic dystonia.

    Idiopathic dystonia with onset in the first year of life has been described as a transient movement disorder, in contrast to other forms of idiopathic dystonia We report on a girl who showed, from her 5th month, episodes of dystonic postures of her neck and arm, which lasted for seconds and occurred several times a day. Neurologic findings and the psychomotor development were and remained normal. Neurometabolic screening tests and cerebral magnetic resonance imaging showed normal results. Functional cerebral imaging showed decreased perfusion of the basal ganglia and the left temperomesial cortex using single photon emission computed tomography (SPECT with technetium 99m hexamethylpropyleneamine oxime [99mTc-HMPAO]) and decreased glucose metabolism in the basal ganglia and the cerebellum using positron emission tomography (PET with [18F]fluorodeoxyglucose [18FDG]). Follow-up revealed that the episodes disappeared at the age of 16 months. The findings of PET and SPECT give evidence of an alteration in basal ganglia function but also in functions of other central nervous system regions, which may, however, be temporary.
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ranking = 0.6666830669322
keywords = dystonia, idiopathic
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16/91. Thalamic hand dystonia: an MRI anatomoclinical study.

    Focal dystonia has been attributed to lesions involving the basal ganglia and/or thalamus. hand dystonia was studied in a patient with a unilateral thalamic infarction documented by MRI. A 18-year-old girl presented with severe isolated dystonia of the right hand as a sequel of perinatal infarction. MRI scan revealed infarction affecting part of the dorsomedian, lateral posterior, ventral lateral, ventral posterior lateral nuclei, and centromedian-parafascicular nucleus of the contralateral thalamus. The unique MRI anatomoclinical presentation of this case, taken together with the literature data, could provide evidence that a lesion affecting one or several thalamic nuclei, including the centromedian nucleus, can induce hand dystonia.
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ranking = 0.88888888888889
keywords = dystonia
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17/91. Cervical dystonia in monozygotic twins: case report and review of the literature.

    We report on monozygotic male twins of German origin with early onset cervical dystonia. No other family members were affected. Although identical in age of onset and clinical course, there were phenotypic variations. While the second-born, more affected twin suffered from a symmetric retrocollis, the twin brother mainly presented with a spasmodic torticollis to the left. dystonia remained focal in both siblings. A DYT1 gene deletion was excluded. Including our patients, hitherto only four twin pairs with idiopathic dystonia have been reported. Although dystonia in twins can be remarkably uniform in some cases, major differences in terms of disease progression, remissions, and disability may be found. These observations underline the role of inheritance in the pathogenesis of idiopathic dystonia but also indicate that some other factors contribute to the clinical presentation of dystonia.
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ranking = 1.0000065601062
keywords = dystonia, idiopathic
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18/91. Neurostimulation of the ventral intermediate thalamic nucleus in inherited myoclonus-dystonia syndrome.

    We report on the effects of bilateral neurostimulation of the ventral intermediate thalamic nucleus (VIM) in a patient with medically intractable and progressing inherited myoclonus dystonia syndrome (IMDS). Postoperatively, the patient improved by approximately 80% on the modified version of a myoclonus score without any significant change in the dystonic symptoms. This suggests that neurostimulation of the VIM may be an effective treatment for myoclonus in pharmacologically intractable IMDS.
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ranking = 0.55555555555556
keywords = dystonia
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19/91. Playing harp, another unusual task-specific dystonia.

    Herein we report a task-specific dystonia in a 48-year-old woman, with an unusual association between a familial harp-playing dystonia and essential tremor.
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ranking = 0.66666666666667
keywords = dystonia
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20/91. Atypical presentation of dopa-responsive dystonia: generalized hypotonia and proximal weakness.

    Dopa-responsive dystonia (DRD) is an autosomal dominant disorder typically presenting as dystonia with diurnal variability. Described is an 8-year-old boy who had had waddling gait, generalized hypotonia, and proximal weakness since early childhood. He responded well to low-dose L-dopa. He had a point mutation of the gtp cyclohydrolase I gene. The patient's father and sister had the same mutation but did not have proximal weakness. gtp cyclohydrolase I deficiency can present with hypotonia and weakness.
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ranking = 0.66666666666667
keywords = dystonia
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