Longitudinal Analysis of the Relation Between Clinical Impairment and Gray Matter Degeneration in Spinocerebellar Ataxia Type 7 Patients.
Adolescent
Adult
Atrophy
Brain
/ pathology
Cerebellum
/ diagnostic imaging
Cerebral Cortex
/ diagnostic imaging
Disease Progression
Female
Follow-Up Studies
Gray Matter
/ diagnostic imaging
Humans
Image Processing, Computer-Assisted
Longitudinal Studies
Magnetic Resonance Imaging
Male
Mental Status and Dementia Tests
Middle Aged
Neurodegenerative Diseases
/ diagnostic imaging
Pons
/ diagnostic imaging
Spinocerebellar Ataxias
/ diagnostic imaging
Verbal Learning
Young Adult
Cognitive assessment
Effect size
Motor deterioration
Rate of atrophy
SCA7
Journal
Cerebellum (London, England)
ISSN: 1473-4230
Titre abrégé: Cerebellum
Pays: United States
ID NLM: 101089443
Informations de publication
Date de publication:
Jun 2021
Jun 2021
Historique:
accepted:
13
10
2020
pubmed:
14
11
2020
medline:
15
12
2021
entrez:
13
11
2020
Statut:
ppublish
Résumé
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.
Identifiants
pubmed: 33184781
doi: 10.1007/s12311-020-01205-8
pii: 10.1007/s12311-020-01205-8
doi:
Types de publication
Journal Article
Langues
eng
Sous-ensembles de citation
IM
Pagination
346-360Subventions
Organisme : Premio a la investigación interdisciplinaria en torno al Plan de restructuración estratégica del CONACYT 2018" (0001)
ID : 0001
Organisme : CONACYT
ID : 326042
Organisme : Universidad Nacional Autónoma de México
ID : PAPIIT-UNAM
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