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Striatal and extrastriatal atrophy in Huntington's disease and its relationship with length of the CAG repeat BJMBR
Ruocco,H.H.; Lopes-Cendes,I.; Li,L.M.; Santos-Silva,M.; Cendes,F..
Huntington's disease (HD) is an autosomal dominant neurodegenerative disorder that affects the striatum most severely. However, except for juvenile forms, relative preservation of the cerebellum has been reported. The objective of the present study was to perform MRI measurements of caudate, putamen, cerebral, and cerebellar volumes and correlate these findings with the length of the CAG repeat and clinical parameters. We evaluated 50 consecutive patients with HD using MRI volumetric measurements and compared them to normal controls. Age at onset of the disease ranged from 4 to 73 years (mean: 43.1 years). The length of the CAG repeat ranged from 40 to 69 (mean: 47.2 CAG). HD patients presented marked atrophy of the caudate and putamen, as well as reduced...
Tipo: Info:eu-repo/semantics/article Palavras-chave: Neurodegeneration; Dynamic mutation; Genotype-phenotype correlation; Basal ganglia; Magnetic resonance imaging; Huntington's disease.
Ano: 2006 URL: http://www.scielo.br/scielo.php?script=sci_arttext&pid=S0100-879X2006000800016
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