Cases reported "Huntington Disease"

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1/2. Huntington's disease in thailand: a case report.

    The first documented case of Huntington's disease (HD) in thailand is reported. The patient presented with classical clinical picture, clinical course and family history. Although hereditary chorea is most frequently associated with HD, other hereditary basal ganglion diseases, Syndenham's chorea, and other causes of abnormal movements were considered and excluded by clinical profiles and investigations. Various recent aspects of HD are briefly mentioned.
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keywords = ganglion
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2/2. safety of intrastriatal neurotransplantation for Huntington's disease patients.

    Fetal neural transplantation has been shown to be a feasible, safe, and according to a number of recent reports, effective treatment for Parkinson's disease (PD). Fetal striatal transplantation may be as feasible, safe, and effective a treatment for Huntington's disease (HD), a disorder for which there is currently no effective treatment. This report describes our experience with fetal striatal transplantation to adult striatum in three HD patients. Three moderately advanced, nondemented HD patients received transplantation of fetal striatal tissue. The striatal precursor was selectively obtained from the lateral ganglionic eminence. Each patient received bilateral grafts from five to eight donors, placed into the caudate nucleus (one graft on each side) and the putamen (four grafts on each side). All three patients had HD as documented by family history, dna heterozygosity (17-20 and 48-51 repeats), magnetic resonance imaging (MRI) revealing striatal atrophy, and 2-deoxyglucose positron emission tomography revealing striatal hypometabolism. All patients had been evaluated using the Unified Huntington's disease Rating Scale and appropriate neuropsychological tests for at least 3 months prior to transplantation. One year following transplantation, MRI of all three patients revealed that the grafts survived and grew within the striatum without displacing the surrounding tissue. No patients demonstrated adverse effects of the surgery or the associated cyclosporin immunosuppression, nor did any patient exhibit deterioration following the procedure. The limited experience provided by these three patients indicates that fetal tissue transplantation can be performed in HD patients without unexpected complications.
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keywords = ganglion
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