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Planning deficits in Huntington’s disease: A brain structural correlation by voxel-based morphometry

2021 , Calderon-Villalon, Jesus , Ramirez-Garcia, Gabriel , Fernandez-Ruiz, Juan , Sangri-Gil, Fernanda , Campos-Romo, Aurelio , Gálvez , Víctor , Andre Aleman

Introduction: Early Huntington’s disease (HD) patients begin to show planning deficits even before motor alterations start to manifest. Generally, planning ability is associated with the functioning of anterior brain areas such as the medial prefrontal cortex. However, early HD neuropathology involves significant atrophy in the occipital and parietal cortex, suggesting that more posterior regions could also be involved in these planning deficits. Objective: To identify brain regions associated with planning deficits in HD patients at an early clinical stage. Materials and methods: Twenty-two HD-subjects genetically confirmed with incipient clinical manifestation and twenty healthy subjects were recruited. All participants underwent MRI T1 image acquisition as well as testing in the Stockings of Cambridge (SOC) task to measure planning ability. First, group comparison of SOC measures were performed. Then, correlation voxel-based morphometry analyses were done between gray matter degeneration and SOC performance in the HD group

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Montreal Cognitive Assessment (MoCA) performance in Huntington’s disease patients correlates with cortical and caudate atrophy

2022 , Ramirez-Garcia, Gabriel , Gálvez , Víctor , Diaz, Rosalinda , Campos-Romo, Aurelio , Fernandez-Ruiz, Juan

Huntington's Disease (HD) is an autosomal neurodegenerative disease characterized by motor, cognitive, and psychiatric symptoms. Cognitive impairment develops gradually in HD patients, progressing later into a severe cognitive dysfunction. The Montreal Cognitive Assessment (MoCA) is a brief screening test commonly employed to detect mild cognitive impairment, which has also been useful to assess cognitive decline in HD patients. However, the relationship between MoCA performance and brain structural integrity in HD patients remains unclear. Therefore, to explore this relationship we analyzed if cortical thinning and subcortical nuclei volume differences correlated with HD patients' MoCA performance. Twenty-two HD patients and twenty-two healthy subjects participated in this study. T1-weighted images were acquired to analyze cortical thickness and subcortical nuclei volumes. Group comparison analysis showed a significantly lower score in the MoCA global performance of HD patients. Also, the MoCA total score correlated with cortical thinning of fronto-parietal and temporo-occipital cortices, as well as with bilateral caudate volume differences in HD patients. These results provide new insights into the effectiveness of using the MoCA test to detect cognitive impairment and the brain atrophy pattern associated with the cognitive status of prodromal/early HD patients.

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Extrastriatal degeneration correlates with deficits in the motor domain subscales of the UHDRS

2018 , Gálvez , Víctor , Ramírez-García, Gabriel , Hernandez-Castillo, Carlos R. , Bayliss, Leo , Díaz, Rosalinda , Lopez-Titla, María Margarita , Campos-Romo, Aurelio , Fernandez-Ruiz, Juan

Introduction: 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). Objective: Analyze if extrastriatal grey matter decrease in patients with HD correlates with motor performance assessed with the UHDRS and its different domains. Method: 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.