Cases reported "Movement Disorders"

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1/364. Osmotic demyelination syndrome with two-phase movement disorders: case report.

    Osmotic demyelination syndrome (ODS) is characterized by regions of demyelination throughout the brain, which are most prominent in the pons. This demyelinating disease is associated with electrolyte disturbances and typically occurs in patients who are alcoholic or malnourished. movement disorders are not frequently recognized in patients with ODS. This report describes a 22-year-old woman with ODS after correction of profound hyponatremia. The main neurologic symptom was two-phase movement disorder. First, she had acute onset dystonia, then the movement disorder transformed to generalized rigidity and tremors in the delayed second phase. magnetic resonance imaging in the first phase revealed demyelinating lesions in the central pons, bilateral thalami and basal ganglia. In the second phase, the previous myelinolysis had been partially resolved. The clinical course of the two-phase movement disorder did not correlate with the resolving feature of neuroradiologic findings. During the second-phase movement disorder, the patient had a good response to propranolol and trihexyphenidyl.
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2/364. Facial dyskinesia induced by auditory stimulation: a report of four cases.

    INTRODUCTION: The Vibroacoustic disease (VAD) is an occupational pathology induced by long-term (>10 yr) exposure to large pressure amplitude (> or = 90 dB SPL) and low frequency (< or = 500 Hz) noise. During studies contributing to the characterization of VAD, abnormal facial movements induced by repetitive auditory stimuli were observed in one individual employed as an aeronautical mechanic. The goal of this study was to investigate the existence of movement disorders triggered by auditory stimuli in a group of VAD-diagnosed patients. methods: Sixty VAD patients received auditory and median nerve stimulation. Simultaneously, EEG monitoring was performed. RESULTS: Abnormal myoclonus movements were triggered by auditory stimuli in four patients. EEG recordings were normal in all 60 patients. Stimulation of the median nerves did not trigger any abnormal movements. CONCLUSION: These data suggest that this facial dyskinesia has a subcortical origin.
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3/364. A novel method for locomotion training.

    This article describes a novel therapeutic system for locomotion training and learning for patients with a wide range of neurological and musculoskeletal disorders. The technique embraces the notion that locomotion therapy should be goal oriented and task specific. The task specificity includes a partial weight-bearing device that permits the posture/equilibrium, movement, and weight-bearing components of gait function to operate concurrently, even in patients with serious deficits. In addition, it allows interaction with therapists and others to facilitate locomotion control, particularly during the early stages of gait therapy. Neurobiological bases for this technique and early clinical results are discussed, and two case studies of patients with traumatic brain injury (TBI) are presented. Although well-designed efficacy studies are needed, clearly this therapeutic approach to locomotor disorders among TBI patients meets the various criteria for recovery of gait function established in this article.
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4/364. Selected legal issues in movement disorders.

    This article explores the long-standing question of whether trauma causes Parkinson's disease, and discusses the impact of informed consent and confidentiality in issues of genetic testing for Huntington's disease. Neurologists are appropriately concerned about the legal aspects of genetic testing, and this article attempts to address that subject from a medical-legal perspective.
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5/364. movement disorders following nonfunctional neurosurgery.

    OBJECT: knowledge is scarce about movement disorders that follow neurosurgical operations other than functional stereotactic surgery. The cases of 14 patients who suffered from movement disorders secondary to craniocerebral or spinal surgery are analyzed. None of these patients was initially treated by any of the authors. methods: Twelve patients underwent surgery for cerebral diseases. Nine of these patients harbored tumors and three patients had neurovascular disorders. Two patients underwent spinal surgery for cervicothoracic ependymoma or for multiple cervical disc herniations. Twelve of the 14 patients had immediate postoperative side effects such as hemiparesis, ataxia, and somnolence. In all but two patients, movement disorders became manifest only after a delay. Dystonic movement disorders developed in eight patients, unilateral tremors in three patients, unilateral facial myokymia in one patient, and hemichorea-hemiballism in two patients. The mean delay of onset for tremor was 5 weeks and that for dystonic movement disorders was 5.5 months. movement disorders were transient in three patients; however, they were persistent in 11 patients at a mean follow-up period of 5 years. These movement disorders caused marked persistent disability in four patients. Lesions of the contralateral striatum were identified in patients with dystonic syndromes and lesions of the dentatothalamic outflow in patients with tremors. In three patients who had postoperative basal ganglia lesions after partial removal of astrocytomas, tumor regrowth was later documented. Medical treatment in patients with persistent movement disorders rendered only limited benefit. Two patients improved with botulin injections. In one patient postoperative hemidystonia was alleviated by contralateral thalamotomy. CONCLUSIONS: Dystonic syndromes and tremors are the most common movement disorders that occur after craniocerebral and spinal surgery. Postoperative movement disorders can lead to various degrees of functional disability. The pathoanatomical correlations are similar to those described in other patients with secondary movement disorders.
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6/364. Generalized freezing in Hallervorden-Spatz syndrome: case report.

    We report the case of a female patient who was exhibiting slowly progressive, severe, generalized freezing of voluntary movement and disequilibrium. brain magnetic resonance imaging showed a very low signal intensity in both pallidal nuclei, with a high signal intensity in the central portion, the so called 'eye-of-the-tiger' sign. Despite the unusual clinical features, we believe that this is a case of Hallervorden-Spatz syndrome, although without neuropathological examination we were unable to confirm the diagnosis with certainty. This case further demonstrates the complex role of the pallidum in voluntary movement.
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7/364. Neuronal activity in the basal ganglia in patients with generalized dystonia and hemiballismus.

    Microelectrode recording was performed in the basal ganglia of 3 patients with generalized dystonia and 1 patient with hemiballismus secondary to a brainstem hemorrhage. Neuronal activity was recorded from the internal and external segments of the globus pallidus and assessed for mean discharge rate and pattern of spontaneous activity. The responses of neurons in the internal segment of the globus pallidus to passive and active movements were also evaluated. Mean discharge rates of neurons in both segments of the pallidum in patients with dystonia and the patient with hemiballismus were considerably lower than those reported for patients with idiopathic Parkinson's disease. In addition, the pattern of spontaneous neuronal activity was highly irregular, occurring in intermittent grouped discharges separated by periods of pauses. Although receptive fields in the dystonia patients were widened and less specific than those reported in normal monkeys, neuronal responses to movement were uncommon in the hemiballismus patient. Before surgery, patients with dystonia experienced abnormal posturing and involuntary movements. Coactivation of agonist-antagonist muscle groups was observed both at rest and during the performance of simple movements. After pallidotomy there was a significant reduction in the involuntary movement associated with these disorders and a more normal pattern of electromyographic activity during rest and movement. Given the improvement in dystonic and hemiballistic movements in these patients after ablation of the sensorimotor portion of the internal segment of the globus pallidus, we suggest that pallidotomy can be an effective treatment for patients with dystonia and also for patients with medically intractable hemiballismus. Based on the finding of decreased neuronal discharge rates in pallidal neurons, we propose that physiologically dystonia most closely resembles a hyperkinetic movement disorder. A model for dystonia is proposed that incorporates the observed changes in the rate and pattern of neuronal activity in the pallidum with data from neuroimaging with positron emission tomography and 2-deoxyglucose studies.
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8/364. Familial paroxysmal kinesigenic choreoathetosis: an electrophysiologic and genotypic analysis.

    PURPOSE: We report a pedigree of familial paroxysmal kinesigenic choreoathetosis (PKC) in which five of 18 members are affected. The pathophysiologic basis for PKC is still uncertain; reflex epilepsy versus dysfunction of basal ganglia. We examined (a) whether there were ictal discharges during the attacks, and (b) a linkage between PKC and possible dna markers linked to several familial epileptic or movement disorders. methods: Video-monitoring EEG was performed in two patients with PKC during attacks elicited by movements of the lower extremities. blood samples for dna studies were obtained from 15 members of the pedigree. Fourteen polymorphic markers on chromosomes 1p, 2q, 6p, 10q, and 20q were genotyped, and two-point lod scores were calculated for each marker under a dominant model. RESULTS: No ictal discharges were found during the attacks in both patients. We could not obtain significant linkage of PKC with any marker examined. CONCLUSIONS: The video-monitoring EEG findings in our cases strongly suggested that the etiology of PKC should be considered distinct from that of reflex epilepsy. However, the patients in this pedigree had experienced generalized convulsions in their infancies; thus we could not deny the possibility of an epileptogenic basis for PKC. There was no strong evidence for a linkage of the gene for PKC with the candidate regions on 1p, 2q, 6p, 10q, or 20q.
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9/364. Bilateral ballism in a patient with overlapping Fisher's and Guillain-Barre syndromes.

    A 29 year old woman developed diplopia and ataxic gait. Neurological examination showed total ophthalmoplegia, cerebellar ataxia, and areflexia. Moreover, there was muscle weakness in all four limbs. An overlap of Fisher's and Guillain-Barre syndromes was dignosed. On day 5 she suddenly developed involuntary flinging movements that affected the face and four limbs. Surface EMG showed 1.5-2 Hz rhythmic grouping discharges. The involuntary movements were considered ballism. This is the first report of a patient with guillain-barre syndrome and a related disorder who showed ballism.
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10/364. An 'annoying' foot: unilateral painful legs and moving toes syndrome.

    The syndrome of painful legs and moving toes is an uncommon condition causing distressing leg pain and is recognised chiefly by the characteristic involuntary movements. This case report describes an unusual unilateral clinical presentation, which led to a delay in diagnosis.
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