Cases reported "Tremor"

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11/75. intention tremor, parkinsonism, and generalized brain atrophy in male carriers of fragile X.

    The authors report five elderly men with the fragile X premutation who had a progressive action tremor associated with executive function deficits and generalized brain atrophy. These individuals had elevated fragile X mental retardation 1 gene (FMR1) messenger rna and normal or borderline levels of FMR1 protein. The authors propose that elevations of FMR1 messenger rna may be causative for a neurodegenerative syndrome in a subgroup of elderly men with the FMR1 premutation.
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ranking = 1
keywords = group
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12/75. Deep brain stimulator electrodes used for lesioning: proof of principle.

    OBJECTIVE: patients with chronically implanted deep brain stimulator (DBS) electrodes can encounter complications requiring hardware removal. We assessed the safety and efficacy of using implanted DBS electrodes to create a therapeutic lesion before their removal. methods: Revision or removal of the DBS electrodes was required in two patients who had previously undergone DBS implantation. We conducted a series of in vitro experiments to confirm that the DBS electrodes could be used to generate radiofrequency lesions and to assess the relationship between radiofrequency parameters and lesion size. With this information, and with the approval of the hospital ethical review board, implanted electrodes were used to create incremental radiofrequency lesions in the thalamus in one patient and in the subthalamic nucleus in another. The procedures were performed under local anesthesia with contiguous contacts of the DBS lead connected to the active and reference sites of the RF generator to create a bipolar lesion. RESULTS: A 51-year-old man with essential tremor and a thalamic DBS required repeated battery changes secondary to tolerance and high voltage demands. Rather than replacing the battery, a radiofrequency thalamotomy was performed by using the existing left DBS electrode. At the 6-month follow-up examination, successful lesioning provided near complete tremor control. A second patient, a 50-year-old man with Parkinson's disease who had undergone bilateral subthalamic deep brain stimulation, developed skin erosion over the DBS hardware. A subthalamic nucleus lesion was made through the right DBS electrode. Lesion position and size were confirmed with magnetic resonance imaging. CONCLUSION: Lesions can be made through chronically implanted DBS electrodes in a safe, graded fashion and can produce therapeutic benefit.
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ranking = 4119726.3605392
keywords = thalamic nucleus, nucleus
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13/75. Electrophysiological confirmation of the zona incerta as a target for surgical treatment of disabling involuntary arm movements in multiple sclerosis: use of local field potentials.

    Lesioning or chronic deep brain stimulation (DBS) of the nucleus ventralis intermedius results in abolition of tremor in the contralateral limbs in Parkinson's disease (PD) and also in essential tremor. Recently, chronic DBS of the subthalamic nucleus has also proved to be very effective in reducing contralateral limb tremor in PD. These targets have been less effective in controlling the complex limb tremor often seen in multiple sclerosis (MS). Consequently, other targets have been sought in cases of MS with tremor. We describe a patient with MS with disabling proximal and distal involuntary arm movements in whom we were able to obtain sustained control of contralateral arm tremor and achieve functional improvement of the affected arm by chronic DBS of the region of the zona incerta. We also highlight the important role played by local field potentials recorded from the brain, with simultaneous recording of corresponding EMGs, in target localisation.
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ranking = 2069173.564341
keywords = thalamic nucleus, nucleus
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14/75. Reorganization of somatic sensory function in the human thalamus after stroke.

    A patient with thalamic stroke underwent microelectrode-guided stereotactic thalamic exploration during surgery for control of tremor. The results of somatic sensory mapping in this patient were compared with explorations carried out during stereotactic surgery for the control of essential tremor (70 patients). There was evidence both of somatotopic reorganization and of anatomic reorganization of the representation of deep structures in the principal somatic sensory nucleus of the thalamus and the nuclei located anterior to it. This case demonstrates that thalamic reorganization can occur after a thalamic stroke and may play a role in recovery from such a stroke.
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ranking = 9310.3840713806
keywords = nucleus
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15/75. Hemiparkinsonism-somatic hemiatrophy syndrome.

    PURPOSE: This paper highlights the hemiparkinsonism-hemiatrophy (HPHA) syndrome as a unique presentation of the parkinsonian state. Clinically relevant diagnostic and treatment aspects are reviewed. METHOD: We report a case of HPHA, in a 21-year-old, otherwise healthy, woman. Clinical and radiographic features of our case are presented. We also review the current literature on the clinical, radiological and pathophysiological mechanisms of HPHA. RESULTS: In our case, despite the lack of benefit from anticholinergics and dopamine agonists (the patient declined treatment with levodopa), the patient showed a dramatic improvement with subthalamic nucleus stimulator (STN) implantation. There are no reported cases of use of STN stimulator in HPHA. CONCLUSION: Hemiparkinsonism-hemiatrophy is a distinct entity that may be clinically and pathogenetically different from idiopathic Parkinson's disease; hence, HPHA needs to be considered as a possible syndrome in patients that have persistent unilateral parkinsonism. As medications are reported to be unhelpful in HPHA cases, early surgical intervention may be an option, such as in our case.
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ranking = 2059863.1802696
keywords = thalamic nucleus, nucleus
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16/75. Posttraumatic tremor without parkinsonism in a patient with complete contralateral loss of the nigrostriatal pathway.

    We present a patient with posttraumatic tremor who did not show any [(123)I]FP-CIT uptake in the contralateral putamen and caudate. The absence of hypokinesia and rigidity is surprising in the presence of a striatal dopaminergic denervation that is even more severe than in Parkinson's disease. An explanation, therefore, could be that the lesion in the subthalamic nucleus in our patient prevented the onset of a Parkinson syndrome.
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ranking = 2059863.1802696
keywords = thalamic nucleus, nucleus
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17/75. Lesioning the thalamus for dyskinesia.

    Recent advances on understanding the pallidothalamic relation lead us to perform Vim-Vo thalamotomy (combined thalamic lesion in ventralis intermedius nucleus and ventralis oralis nucleus) for cases with dyskinesia. In our recent series of thalamotomies, there are 12 cases of dyskinesia caused by various etiologies. Therefore the clinical manifestation of the involuntary movement was different in each case, including, more or less, some elements of irregular involuntary hyperkinetic movement. Stereotactic operation was performed using Leksell's apparatus aided by Surgiplan and MRI. The Vim nucleus was identified by physiological study using microelectrodes. High background activity and kinesthetic neurons are reliable indicators of Vim nucleus (but only for the lateral part). Then, selective coagulation was made by dual coagulation needles. Since the Vo nucleus is located just rostral to the Vim nucleus, the coagulation needle was turned toward the anterior part to partly cover the Vo nucleus. Thus, selective Vim-Vo thalamotomy was shown to be quite successful for the treatment of dyskinesia.
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ranking = 65172.688499664
keywords = nucleus
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18/75. A clinical and pathological study of a Japanese case of amyotrophic lateral sclerosis/Parkinsonism-dementia Complex with family history.

    This report concerns a Japanese family with neuropathological findings consistent with amyotrophic lateral sclerosis/parkinsonism-dementia complex (ALS/PDC) in the Island of guam. The proband was a 68-year-old woman with an 8-year history of parkinsonism which was followed by psychiatric symptoms and neurogenic amyotrophy 5 years after the onset. She had a family history of parkinsonism associated with dementia in all of her three siblings. They grew up in the Hobara village, a focus of amyotrophic lateral sclerosis in the Kii Peninsula of japan in their childhood. Their parents were not consanguineous nor natives of the Kii Peninsula. The brain weight was 1040 g and there were mild frontal lobe atrophy, moderate atrophy of pes hippocampi, decoloration of the substantia nigra and locus coeruleus, and atrophy of the anterior root of the spinal cord. The microscopic examinations revealed degeneration of CA1 portion of the hippocampus to the parahippocampus gyrus, substantia nigra, locus coeruleus and spinal anterior horn with Bunina bodies. The spinal pyramidal tracts also mildly degenerated. neurofibrillary tangles (NFT) were observed in the cerebral cortex, especially in the cortices from hippocampus to lateral occipitotemporal gyri, basal nucleus of Mynert, basal ganglia, thalamus, substantia nigra and widespread regions of the central nervous system through the brainstem to spinal cord including the nucleus of Onufrowitcz. In spite of a small amount of the senile plaques in the cerebral cortex and lewy bodies in the substantia nigra and locus coeruleus, abundant NFT were distributed mainly in the third layer of the cerebral cortex, which is the characteristic feature of ALS/PDC. Thus, this was likely to be an ALS/PDC case outside the guam Island. A tau mutation was not found on dna analysis.
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ranking = 18620.768142761
keywords = nucleus
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19/75. Eyelid tremor in a patient with a unilateral paramedian thalamic lesion.

    A patient with a circumscribed infarction of the right paramedian thalamus developed a tremor of both eyelids on voluntary eye closure. Co-registration of the magnetic resonance image to a stereotactic atlas of the human thalamus revealed that the lesion was confined to a small subgroup of paramedian nuclei, including the parvocellular part of the mediodorsal nucleus. It is concluded that this region provides inhibitory input to cortical and/or subcortical regions controlling eyelid movements. Voluntary eye closure may involve direct cortico-nuclear connections and indirect pathways through the paramedian thalamus, most probably through the mediodorsal nucleus.
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ranking = 18621.768142761
keywords = nucleus, group
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20/75. Thalamic deep brain stimulation for disabling tremor after excision of a midbrain cavernous angioma. Case report.

    Thalamic deep brain stimulation (DBS) has been demonstrated to be effective for the treatment of parkinsonian or essential tremor. To date, however, few data exist to support the application of this method to treat midbrain tremor. A 24-year-old right-handed man underwent radiosurgery and subsequent resection of a recurrently hemorrhaging cavernous angioma located in the left side of the midbrain. The surgery exacerbated severe choreoathetotic resting and action tremors of his right extremities and trunk. The patient underwent placement of a deep brain stimulator into the left ventral intermediate nucleus of the thalamus (Vim). Postoperatively, decreased truncal ataxia and right-sided choreoathetotic tremor were demonstrated, with a 57% increase in dexterity as measured by task testing. The authors demonstrate that DBS can be an effective treatment modality for disabling tremor after resection of a midbrain cavernous angioma.
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ranking = 9310.3840713806
keywords = nucleus
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