Instituto de Neuroetología, Universidad Veracruzana, Mexico; Unidad Periférica de Neurociencias, Facultad de Medicina, Universidad Nacional Autónoma de México, Instituto Nacional de Neurología y Neurocirugía "MVS", Mexico; Universidad Panamericana, Escuela de Psicología, Laboratorio de Neurociencias cognitivas y desarrollo, Mexico.
Unidad Periférica de Neurociencias, Facultad de Medicina, Universidad Nacional Autónoma de México, Instituto Nacional de Neurología y Neurocirugía "MVS", Mexico.
J Neurol Sci. 2018 Feb 15;385:22-29. doi: 10.1016/j.jns.2017.11.040. Epub 2017 Dec 1.
Striatal degeneration has significant behavioral effects in patients with Huntington's disease (HD). However, there is scant evidence of the possible contribution of extrastriatal regions to the motor alterations assessed within the different domains of the Unified Huntington's Disease Rating Scale (UHDRS).
Analyze if extrastriatal grey matter decrease in patients with HD correlates with motor performance assessed with the UHDRS and its different domains.
Twenty-two molecular diagnosed patients with incipient HD, and twenty-two control participants matched for sex and age participated in this study. Voxel-based morphometry (VBM) analyses were done to identify grey matter decrease in the HD patients, and its relationship with the motor deterioration measured with the UHDRS motor scale. To further explore this relationship, a principal component analysis (PCA) was done on the UHDRS domains scores. Then the average of each component was used as a covariate in a VBM analysis. Finally, individual sub-scores from each domain were also tested for correlations with the VBM results.
In addition to the striatal degeneration, the VBM analysis showed significant negative correlations between the global UHDRS scores and the cerebellum, insula and precuneus atrophy. The UHDRS PCA showed component-related negative correlations suggesting a specific impact of individual degnerations. Further analyses with the individual sub-scores showed more specific corelations, including: chorea, with right caudate and left posterior cingulate gyrus; ocular pursuit, with left precentral gyrus, left superior temporal gyrus, cerebellum culmen and right temporal lobe. Saccadic movements with left postcentral gyrus and left middle occipital gyrus.
In the early stages of HD, it is possible to find correlations between behavioral alterations as measured with the UHDRS motor domains, and extrastriatal regions, including specific areas of the cerebellum, and insular, parietal and frontal cortices. These areas could contribute to the HD related impairments along with the classical deficits associated with the striatal degeneration.
亨廷顿病（HD）患者的纹状体退化会对其行为产生显著影响。然而，目前仅有很少的证据表明，纹状体以外的区域可能会对统一亨廷顿病评定量表（UHDRS）不同领域评估的运动改变产生影响。
分析亨廷顿病患者的纹状体以外的灰质减少是否与 UHDRS 及其不同领域评估的运动表现相关。
本研究纳入了 22 名分子诊断为亨廷顿病的早期患者和 22 名性别和年龄相匹配的对照组参与者。采用基于体素的形态测量学（VBM）分析来识别亨廷顿病患者的灰质减少，并分析其与 UHDRS 运动量表测量的运动恶化之间的关系。为了进一步探索这种关系，对 UHDRS 各领域的评分进行了主成分分析（PCA）。然后，将每个成分的平均值用作 VBM 分析的协变量。最后，还测试了每个领域的个体子评分与 VBM 结果之间的相关性。
除了纹状体退化外，VBM 分析还显示，UHDRS 总评分与小脑、脑岛和楔前叶萎缩之间存在显著的负相关。UHDRS PCA 显示出与成分相关的负相关，表明了个别退化的特定影响。进一步对个体子评分进行分析，显示出了更具体的相关性，包括：舞蹈症与右侧尾状核和左侧后扣带回 gyrus 相关；眼球追踪与左侧中央前回、左侧颞上回、小脑蚓部和右侧颞叶相关；扫视运动与左侧中央后回和左侧中枕叶相关。
在亨廷顿病的早期阶段，就有可能在 UHDRS 运动领域所测量的行为改变与纹状体以外的区域之间发现相关性，包括小脑、脑岛以及顶叶和额叶皮质的特定区域。这些区域可能会与纹状体退化相关的缺陷一起，对亨廷顿病相关的损害产生影响。
