Aylward E H, Li Q, Stine O C, Ranen N, Sherr M, Barta P E, Bylsma F W, Pearlson G D, Ross C A
Division of Psychiatric Neuroimaging, Baltimore Huntington's Disease Center, Johns Hopkins University School of Medicine, MD 21287-7362, USA.
Neurology. 1997 Feb;48(2):394-9. doi: 10.1212/wnl.48.2.394.
Cross-sectional MRI studies demonstrating an association between caudate atrophy and symptom severity and duration of symptoms in patients with Huntington's disease (HD) have been assumed to reflect longitudinal changes in basal ganglia, but such neuropathologic progression has never been directly demonstrated. Subjects in the current study were 23 HD patients at various stages of the disorder who had two MRI images at least 10 months apart (mean interimage interval = 20.8 months). We measured volumes of caudate, putamen, and globus pallidus blind to the order of the images. For each structure, we calculated a change score by subtracting the volume obtained on the follow-up imaging from that obtained on the initial imaging. Results indicated significant decreases over time in caudate, putamen, and total basal ganglia volume. Age at onset and length of trinucleotide repeat correlated significantly with amount of volume change in caudate and total basal ganglia, even after controlling for length of interimage interval, duration of disease, and measures of symptom severity. Amount of change in basal ganglia structures was not significantly correlated with neurologic symptom severity at the time of the initial imaging or duration of symptoms. This is the first longitudinal MRI study to document progressive basal ganglia atrophy in HD, and suggests that quantitative neuroimaging with serial MRI may be useful in monitoring effectiveness of potential treatments. In addition, demonstration of greater rate of basal ganglia atrophy in patients with earlier symptom onset suggests that treatment effects may be more quickly observed in this subgroup of patients than in the general HD population.
横断面磁共振成像（MRI）研究表明，亨廷顿舞蹈病（HD）患者的尾状核萎缩与症状严重程度及症状持续时间之间存在关联，人们认为这反映了基底神经节的纵向变化，但这种神经病理学进展从未得到直接证实。本研究的受试者为23名处于疾病不同阶段的HD患者，他们有至少间隔10个月的两张MRI图像（平均图像间隔时间 = 20.8个月）。我们在不知道图像顺序的情况下测量了尾状核、壳核和苍白球的体积。对于每个结构，我们通过用初始成像获得的体积减去随访成像获得的体积来计算变化分数。结果表明，随着时间推移，尾状核、壳核和基底神经节总体积显著减小。发病年龄和三核苷酸重复长度与尾状核和基底神经节总体积变化量显著相关，即使在控制了图像间隔时间、疾病持续时间和症状严重程度指标之后也是如此。基底神经节结构的变化量与初始成像时的神经症状严重程度或症状持续时间无显著相关性。这是第一项记录HD患者基底神经节进行性萎缩的纵向MRI研究，表明连续MRI定量神经成像可能有助于监测潜在治疗的效果。此外，症状发作较早的患者基底神经节萎缩率更高，这表明在该亚组患者中可能比在一般HD人群中能更快观察到治疗效果。
