When does Huntington's disease begin?
When does Huntington's disease begin?
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亨廷顿舞蹈症什么时候开始？
DOI：
10.1017/s1355617798455061
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发表时间：
1998
期刊：
影响因子：
--
通讯作者：
Brandt,J
中科院分区：
文献类型：
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作者：
Campodonico,JR;Aylward,E;Codori,AM;Young,C;Krafft,L;Magdalinski,M;Ranen,N;Slavney,PR;Brandt,J
最近的研究发现，基底神经节萎缩的临床无症状的人与基因突变，导致亨廷顿病（HD）。在13名患有IT-15突变的健康成人中，研究了MRI扫描中尾状核和壳核体积的减少是否与认知和神经功能的变化有关。纹状体体积的减小与更严重的神经系统（主要是运动）损伤、更慢的精神处理速度和更差的语言学习有关，尽管没有一个参与者达到HD临床诊断的自由标准。这些相关性与在有症状的HD患者中观察到的惊人相似，可能反映了疾病的早期表现。（医学杂志，1998,4,467 - 473）
Recent studies have detected basal ganglia atrophy in clinically asymptomatic persons with the genetic mutation that causes Huntington's disease (HD). Whether reductions in caudate and putamen volume on MRI scans are associated with changes in cognitive and neurologic functioning was examined in 13 healthy adults with the IT-15 mutation. Reduced striatal volume was found to correlate with greater neurologic (largely motor) impairment, slower mental processing speed, and poorer verbal learning, although none of the participants met even liberal criteria for clinical diagnosis of HD. These correlations are strikingly similar to those observed in symptomatic HD patients, possibly reflecting the earliest manifestations of disease. (JINS, 1998, 4, 467–473.)