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(Referência obtida automaticamente do SciELO, por meio da informação sobre o financiamento pela FAPESP e o número do processo correspondente, incluída na publicação pelos autores.)

Striatal and extrastriatal atrophy in Huntington's disease and its relationship with length of the CAG repeat

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Autor(es):
H.H. Ruocco ; I. Lopes-Cendes ; L.M. Li ; M. Santos-Silva ; F. Cendes
Número total de Autores: 5
Tipo de documento: Artigo Científico
Fonte: Brazilian Journal of Medical and Biological Research; v. 39, n. 8, p. 1129-1136, Ago. 2006.
Resumo

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 cerebellar and cerebral volumes. There was a significant correlation between age at onset of HD and length of the CAG repeat, as well as clinical disability and age at onset. The degree of basal ganglia atrophy correlated with the length of the CAG repeat. There was no correlation between cerebellar or cerebral volume and length of the CAG repeat. However, there was a tendency to a positive correlation between duration of disease and cerebellar atrophy. While there was a negative correlation of length of the CAG repeat with age at disease onset and with striatal degeneration, its influence on extrastriatal atrophy, including the cerebellum, was not clear. Extrastriatal atrophy occurs later in HD and may be related to disease duration. (AU)

Processo FAPESP: 01/05439-3 - Estudo volumétrico, de textura e espectroscopia por ressonância magnética dos gânglios basais, tálamo e cerebelo em pacientes com Doença de Huntington: correlação com variáveis clínicas e expansão da mutação CAG
Beneficiário:Heloisa Helena Ruocco
Linha de fomento: Bolsas no Brasil - Pós-Doutorado