11/17. Cerebral metabolism of glucose in benign hereditary chorea.Benign hereditary chorea (BHC) is an autosomal dominant disorder characterized by chorea of early onset with little or no progression. There is marked clinical variability in this disease with some subjects having onset in infancy and others with onset in early adulthood. In contrast to Huntington's disease (HD), there is no dementia. Computed tomography is normal in all subjects with no evidence of caudate nucleus atrophy. We present the results of positron emission tomography using 18F-2-fluorodeoxyglucose on three patients with this disorder from two families. Cerebral glucose metabolism in one patient was decreased in the caudate nucleus, as previously reported in HD. The other two persons from a second family showed a relative decrease in metabolic rates of glucose in the caudate when compared with the thalamus. It appears that caudate hypometabolism is not specific for HD. These findings suggest that the caudate nucleus may play a significant role in the pathophysiology of some persons with BHC.- - - - - - - - - - ranking = 1keywords = nucleus (Clic here for more details about this article) |
12/17. cerebellum and brain stem atrophy in a child with Huntington's chorea.An 11-yr-old girl with Huntington's chorea since the age of 4 had mental deterioration, chorea, rigidity, generalized convulsions and cerebellar ataxia. Computerized tomography (CT) showed atrophy of the cerebellum and brain stem in addition to atrophy of the caudate nucleus and cerebral cortex.- - - - - - - - - - ranking = 0.33333333333333keywords = nucleus (Clic here for more details about this article) |
13/17. Fetal striatal homotransplantation for Huntington's disease: first two case reports.Based on the successful use of fetal striatal brain grafting in the restoration of striatal function in rat and nonhuman primate models of Huntington's disease, as well as on the evidence for the clinical potential of fetal brain grafting in the treatment of Parkinson's disease, homotopic fetal striatal homotransplantations were performed in two huntingtonians. Case 1 was a 37 year-old female with moderate to severe Huntington's disease of 9 years evolution; case 2 was a 29 year-old male with mild Huntington's disease of 5 years evolution. Using open microsurgery, each patient was implanted to the ventricular wall of the right caudate nucleus with both striata from a 13 week-old and a 12 week-old human fetus, respectively. Since surgery both patients were kept on cyclosporine A. Surgery produced no damaging effect to either patient. The time course of the neurological progression of their disease, spanning 33 months for case 1, and 16 months for case 2, reveal that the disease in both patients has progressed more slowly in relation to their preoperative state. Although presently it is not possible to determine to what extent, surgery has modified the course of their disease, or if it will continue to have an effect on it, these surgeries represent the first step towards the development of brain grafting for Huntington's disease.- - - - - - - - - - ranking = 0.33333333333333keywords = nucleus (Clic here for more details about this article) |
14/17. The neuropathological features of neuroacanthocytosis.In this article we describe the neuropathological changes in three patients with neuroacanthocytosis and review the neuropathology of the other eight cases reported in the literature. Macroscopically the brains showed enlargement of the lateral ventricles, especially the frontal horns. The most severely and consistently affected brain areas were the caudate nucleus and putamen, which were atrophic and showed by light microscopy marked neuronal loss and gliosis. Small and medium-sized striatal neurons were particularly depleted. The globus pallidus was almost as severely involved as the striatum. In some cases the thalamus, substantia nigra, and anterior horns of the spinal cord showed pathology, mainly neuronal loss and mild gliosis. Brain areas with no pathology included the subthalamic nucleus, cerebral cortex, cerebellum, pons, and medulla. The preservation of these areas may help in the neuropathological distinction of neuroacanthocytosis from Huntington's disease.- - - - - - - - - - ranking = 0.66666666666667keywords = nucleus (Clic here for more details about this article) |
15/17. Neuropsychological and neuroradiological study of a case of early-onset Huntington's chorea.The authors report a case of Huntington's disease in an 11-year-old boy with onset at six years of age. The neurological signs and symptoms were midway between the hyperkinetic and rigid forms of chorea. Intellectual development was characterized by a medium-grade deficit. MRI revealed marked atrophy of the head of the caudate nucleus, with diffuse hyperintensity of the putamen. The most characteristic neuropsychological feature was ideomotor apraxia. Neuropsychological and neuroradiological data are discussed in relation to the role of the basal nuclei and frontal cortex in the organization of movement.- - - - - - - - - - ranking = 0.33333333333333keywords = nucleus (Clic here for more details about this article) |
16/17. SPECT, CT and MRI in a Turkish family with Huntington's disease.A Turkish family with Huntington's disease documented on CT, MRI and SPECT is reported. Whereas in clinically definite cases CT and MRI are of limited value and SPECT does not add anything of value, in one asymptomatic subject SPECT showed moderate caudate nucleus hypoperfusion, underlining the hypothesis that SPECT may have a role in predicting Huntington's disease.- - - - - - - - - - ranking = 0.33333333333333keywords = nucleus (Clic here for more details about this article) |
17/17. 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.- - - - - - - - - - ranking = 1.120236839539keywords = ganglion, nucleus (Clic here for more details about this article) |
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