Cases reported "Dystonic Disorders"

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1/5. Unilateral lesions of the globus pallidus: report of four patients presenting with focal or segmental dystonia.

    OBJECTIVES: To interpret clinical features after unilateral lesions of the globus pallidus on the basis of physiology of the basal ganglia. methods: Four patients with unilateral lesions in the globus pallidus (GP) were clinically examined and the literature on patients with pallidal lesions was reviewed. RESULTS: Three patients presented with contralateral dystonia largely confined to one arm in one case and one leg in two cases. One patient had predominant contralateral hemiparkinsonism manifested mainly as micrographia and mild dystonia in one arm. The cause of the lesions was unknown in two patients. In the other two symptoms had developed after head trauma and after anoxia. All lesions involved the internal segment of the GP. Two patients, including the patient with hemiparkinsonism, had additional involvement of the external segment of the GP. In the literature reports on 26 patients with bilateral lesions restricted to the GP only two with unilateral lesions were found. The patients with bilateral pallidal lesions manifested with dystonia, parkinsonism, or abulia. One of the patients with unilateral GP lesions had contralateral hemidystonia, the other contralateral arm tremor. CONCLUSION: These cases emphasise the importance of the GP, particularly its internal segment, in the pathophysiology of dystonia.
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keywords = globus pallidus, pallidus, globus
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2/5. risperidone-responsive segmental dystonia and pallidal deep brain stimulation.

    A 67-year-old man with risperidone-responsive segmental dystonia underwent bilateral deep brain stimulation (DBS) of the globus pallidus internus. Prospectively, the authors assessed the Burke-Fahn-Marsden dystonia Rating Scale in medication (M) and stimulation (S) "on"/"off" states. With DBS at 9 months, the score improved by 86% to 8.5 in M-"on"/S-"on" and 12.5 in M-"off"/S-"on." Studies of the effects of DBS and concomitant medication may be warranted in selected patients treated by DBS for dystonia.
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ranking = 0.16666666666667
keywords = globus pallidus, pallidus, globus
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3/5. deep brain stimulation in myoclonus-dystonia syndrome.

    myoclonus-dystonia syndrome (MDS) is an autosomal dominant disorder characterized by bilateral myoclonic jerks. An 8-year-old boy presenting with early onset, medically intractable, MDS due to a mutation in the epsilon-sarcoglycan gene (SGCE) underwent chronic bilateral stimulation of the globus pallidus internus, which eliminates both myoclonus and dystonia. We conclude that deep brain stimulation can be an effective and safe treatment for MDS.
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ranking = 0.16666666666667
keywords = globus pallidus, pallidus, globus
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4/5. Sporadic rapid-onset dystonia-parkinsonism syndrome: failure of bilateral pallidal stimulation.

    We report on a woman who had a severe sporadic nonprogressive dystonia-parkinsonism syndrome with rapid onset of symptoms at age 21. Secondary causes for dystonia were ruled out. No response to levodopa/carbidopa was seen. The patient fulfilled all diagnostic criteria of rapid-onset dystonia-parkinsonism, except for autosomal-dominant inheritance. Bilateral deep brain stimulation of the globus pallidus failed to alleviate her symptoms.
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ranking = 0.16666666666667
keywords = globus pallidus, pallidus, globus
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5/5. Pallidal stimulation relieves myoclonus-dystonia syndrome.

    A patient with myoclonus-dystonia syndrome was treated by implanting electrodes in the internal segment of the globus pallidus (GPi) and applying deep brain stimulation. Surgery was done in two sessions. The most affected limb was treated first and the other limb one year later. Neuronal recordings showed that most pallidal neurones discharged in bursts at a relatively low firing rate (mean (SD), 46 (18) Hz) compared with cells in the GPi in patients with Parkinson's disease. Neurones modified the rate and mode of discharge with dystonic postures and rapid involuntary contractions of limb muscles. Neurological examination at 24 months after surgery showed a decline of 47.8% and 78.5% in the Burke-Fahn-Marsden and disability rating scales, respectively.
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ranking = 0.16666666666667
keywords = globus pallidus, pallidus, globus
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