• Victor Galvez, Gabriel Ramírez-García, Carlos R Hernandez-Castillo, Leo Bayliss, Rosalinda Díaz, María Margarita Lopez-Titla, Aurelio Campos-Romo, and Juan Fernandez-Ruiz.
• 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.
• J. Neurol. Sci. 2018 Feb 15; 385: 22-29.

IntroductionStriatal 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).ObjectiveAnalyze if extrastriatal grey matter decrease in patients with HD correlates with motor performance assessed with the UHDRS and its different domains.MethodTwenty-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.ResultsIn 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.ConclusionIn 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.Copyright © 2017 Elsevier B.V. All rights reserved.

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