Longitudinal Analysis of the Relation Between Clinical Impairment and Gray Matter Degeneration in Spinocerebellar Ataxia
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ORIGINAL ARTICLE
Longitudinal Analysis of the Relation Between Clinical Impairment and Gray Matter Degeneration in Spinocerebellar Ataxia Type 7 Patients Anabel Contreras 1 & Gabriel Ramirez-Garcia 2 & Amanda Chirino 3 & Consuelo Morgado-Valle 1 & Erick H. Pasaye 4 & Carlos Hernandez-Castillo 5 & Rosalinda Díaz 3 & Juan Fernandez-Ruiz 3,6 & Luis Beltran-Parrazal 1 Accepted: 13 October 2020 # Springer Science+Business Media, LLC, part of Springer Nature 2020
Abstract Spinocerebellar ataxia type 7 (SCA7) is a neurodegenerative disease characterized by progressive ataxia and retinal degeneration. Previous cross-sectional studies show a significant decrease in the gray matter of the cerebral cortex, cerebellum, and brainstem. However, there are no longitudinal studies in SCA7 analyzing whole-brain degeneration and its relation to clinical decline. To perform a 2-year longitudinal characterization of the whole-brain degeneration and clinical decline in SCA7, twenty patients underwent MRI and clinical evaluations at baseline. Fourteen completed the 2-year follow-up study. A healthy-matched control group was also included. Imaging analyses included volumetric and cortical thickness evaluation. We measured the cognitive deterioration in SCA7 patients using MoCA test and the motor deterioration using the SARA score. We found statistically significant differences in the follow-up compared to baseline. Imaging analyses showed that SCA7 patients had severe cerebellar and pontine degeneration compared with the control group. Longitudinal follow-up imaging analyses of SCA7 patients showed the largest atrophy in the medial temporal lobe without signs of a progression of cerebellar and pontine atrophy. Effect size analyses showed that MRI longitudinal analysis has the largest effect size followed by the SARA scale and MoCA test. Here, we report that it is possible to detect significant brain atrophy and motor and cognitive clinical decline in a 2-year follow-up study of SCA7 patients. Our results support the hypothesis that longitudinal analysis of structural MRI and MOCA tests are plausible clinical markers to study the natural history of the disease and to design treatment trials in ecologically valid contexts. Keywords SCA7 . Rate of atrophy . Effect size . Cognitive assessment . Motor deterioration
Anabel Contreras, Gabriel Ramirez-Garcia and Amanda Chirino contributed equally to this work. Supplementary Information The online version contains supplementary material available at https://doi.org/10.1007/s12311-02001205-8. * Juan Fernandez-Ruiz [email protected] * Luis Beltran-Parrazal [email protected] 1
Centro de Investigaciones Cerebrales, Universidad Veracruzana, Berlin 7, Fracc. Monte Magno, C.P. 91193 Xalapa, Veracruz, Mexico
2
Unidad Periférica de Neurociencias, Facultad de Medicina, Universidad Nacional Autónoma de México, Instituto Nacional de Neurología y Neurocirugía “Manuel Velasco Suárez”, Mexico City, Mexico
3
Laboratorio de Neuropsicología, Departamento de Fisiología, Facultad de Medicina, Universidad Nacional Aut
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