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Longitudinal Analysis of the Relation Between Clinical Impairment and Gray Matter Degeneration in Spinocerebellar Ataxia Type 7 Patients.

Authors
  • Contreras, Anabel1
  • Ramirez-Garcia, Gabriel2
  • Chirino, Amanda3
  • Morgado-Valle, Consuelo1
  • Pasaye, Erick H4
  • Hernandez-Castillo, Carlos5
  • Díaz, Rosalinda3
  • Fernandez-Ruiz, Juan6, 7
  • Beltran-Parrazal, Luis8
  • 1 Centro de Investigaciones Cerebrales, Universidad Veracruzana, Berlin 7, Fracc. Monte Magno, C.P. 91193, Xalapa, Veracruz, Mexico. , (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. , (Mexico)
  • 3 Laboratorio de Neuropsicología, Departamento de Fisiología, Facultad de Medicina, Universidad Nacional Autónoma de México, Coyoacán, C.P. 04510, Mexico City, Mexico. , (Mexico)
  • 4 Magnetic Resonance Unit, Institute of Neurobiology, Universidad Nacional Autónoma de México campus Juriquilla, Querétaro, Mexico. , (Mexico)
  • 5 CONACyT - Instituto de Neuroetología, Universidad Veracruzana, Xalapa, Veracruz, Mexico. , (Mexico)
  • 6 Laboratorio de Neuropsicología, Departamento de Fisiología, Facultad de Medicina, Universidad Nacional Autónoma de México, Coyoacán, C.P. 04510, Mexico City, Mexico. [email protected] , (Mexico)
  • 7 Facultad de Psicología, Universidad Veracruzana, Xalapa, Veracruz, Mexico. [email protected] , (Mexico)
  • 8 Centro de Investigaciones Cerebrales, Universidad Veracruzana, Berlin 7, Fracc. Monte Magno, C.P. 91193, Xalapa, Veracruz, Mexico. [email protected] , (Mexico)
Type
Published Article
Journal
Cerebellum (London, England)
Publication Date
Jun 01, 2021
Volume
20
Issue
3
Pages
346–360
Identifiers
DOI: 10.1007/s12311-020-01205-8
PMID: 33184781
Source
Medline
Keywords
Language
English
License
Unknown

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.

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