Striatal And Extrastriatal Atrophy In Huntington's Disease And Its Relationship With Length Of The Cag Repeat.

dc.creatorRuocco, H H
dc.creatorLopes-Cendes, I
dc.creatorLi, L M
dc.creatorSantos-Silva, M
dc.creatorCendes, F
dc.date2006-Aug
dc.date2015-11-27T13:05:40Z
dc.date2015-11-27T13:05:40Z
dc.date.accessioned2018-03-29T01:03:13Z
dc.date.available2018-03-29T01:03:13Z
dc.identifierBrazilian Journal Of Medical And Biological Research = Revista Brasileira De Pesquisas Médicas E Biológicas / Sociedade Brasileira De Biofísica ... [et Al.]. v. 39, n. 8, p. 1129-36, 2006-Aug.
dc.identifier0100-879X
dc.identifier
dc.identifierhttp://www.ncbi.nlm.nih.gov/pubmed/16906288
dc.identifierhttp://repositorio.unicamp.br/jspui/handle/REPOSIP/196875
dc.identifier16906288
dc.identifier.urihttp://repositorioslatinoamericanos.uchile.cl/handle/2250/1297108
dc.descriptionHuntington'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.
dc.description39
dc.description1129-36
dc.languageeng
dc.relationBrazilian Journal Of Medical And Biological Research = Revista Brasileira De Pesquisas Médicas E Biológicas / Sociedade Brasileira De Biofísica ... [et Al.]
dc.relationBraz. J. Med. Biol. Res.
dc.rightsaberto
dc.rights
dc.sourcePubMed
dc.subjectAdolescent
dc.subjectAdult
dc.subjectAged
dc.subjectAtrophy
dc.subjectBrain
dc.subjectCase-control Studies
dc.subjectChild
dc.subjectChild, Preschool
dc.subjectFemale
dc.subjectGenotype
dc.subjectHumans
dc.subjectHuntington Disease
dc.subjectMagnetic Resonance Imaging
dc.subjectMale
dc.subjectMiddle Aged
dc.subjectTrinucleotide Repeats
dc.titleStriatal And Extrastriatal Atrophy In Huntington's Disease And Its Relationship With Length Of The Cag Repeat.
dc.typeArtículos de revistas


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