지원사업
학술연구/단체지원/교육 등 연구자 활동을 지속하도록 DBpia가 지원하고 있어요.
커뮤니티
연구자들이 자신의 연구와 전문성을 널리 알리고, 새로운 협력의 기회를 만들 수 있는 네트워킹 공간이에요.
논문 기본 정보
- 자료유형
- 학술저널
- 저자정보
- 발행연도
- 2013.1
- 수록면
- 21 - 25 (5page)
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초록· 키워드
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Background and Purpose Huntington’s disease (HD) is an autosomal-dominant inherited neurodegenerative disorder. Genetic analysis of abnormal CAG expansion in the IT15 gene allows disease confirmation even in the preclinical stage. However, because there is no treatment to cure or delay the progression of this disease, monitoring of biological markers that predict progression is warranted.
Methods FDG-PET was applied to 13 patients with genetically confirmed HD in the early stage of the disease. We recorded the initial and follow-up statuses of patients using the Independence Scale (IS) of the Unified Huntington’s Disease Rating Scale. The progression rate (PR) was calculated as the annual change in the IS. The patients were divided into two groups with faster and slower progression, using the median value of the PR as the cut-off. FDG-PET data were analyzed using regions of interest, and compared among the two patient groups and 11 age- and sexmatched controls.
Results The mean CAG repeat size in patients was 44.7. The CAG repeat length was inversely correlated with the age at onset as reported previously, but was not correlated with the clinical PR. Compared with normal controls, hypometabolism was observed even at very early stages of the disease in the bilateral frontal, temporal, and parietal cortices on FDG-PET. The decreases in metabolism in the bilateral frontal, parietal, and right temporal cortices were much greater in the faster-progression group than in the slower-progression group.
Conclusions A decrease in cortical glucose metabolism is suggested as a predictor for identifying a more rapid form of progression in patients with early-stage HD.
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참고문헌 (21)
참고문헌 신청
Antonini A / 1996 / Striatal glucose metabolism and dopamine D2 receptor binding in asymptomatic gene carriers and patients with Huntington’s disease / Brain 119 : 2085 ~ 20
Antonini A / 1998 / [11C]raclopride-PET studies of the Huntington’s disease rate of progression: relevance of the trinucleotide repeat length / Annals of Neurology 43 : 253
Ashizawa T / 1994 / CAG repeat size and clinical presentation in Huntington’s disease / Neurology 44 : 1137 ~ 1143
Aylward EH / 1994 / Reduced basal ganglia volume associated with the gene for Huntington’s disease in asymptomatic at-risk persons / Neurology 44 : 823 ~ 828
Aylward EH / 1996 / Basal ganglia volume and proximity to onset in presymptomatic Huntington disease / Archives of neurology 53 : 1293 ~ 1296
Antonini A / 1998 / [11C]raclopride-PET studies of the Huntington’s disease rate of progression: relevance of the trinucleotide repeat length / Annals of Neurology 43 : 253
Ashizawa T / 1994 / CAG repeat size and clinical presentation in Huntington’s disease / Neurology 44 : 1137 ~ 1143
Aylward EH / 1994 / Reduced basal ganglia volume associated with the gene for Huntington’s disease in asymptomatic at-risk persons / Neurology 44 : 823 ~ 828
Aylward EH / 1996 / Basal ganglia volume and proximity to onset in presymptomatic Huntington disease / Archives of neurology 53 : 1293 ~ 1296
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