Cognitive Impairments in Spinocerebellar Ataxia Type 10 and Their Relation to Cortical Thickness
| Autor: | Juan Fernandez-Ruiz, Carlos R. Hernandez-Castillo, Gabriel Ramirez-Garcia, Amanda Chirino-Pérez, Diana L. Torres, Rosalinda Diaz, Israel Vaca-Palomares |
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| Rok vydání: | 2021 |
| Předmět: |
congenital
hereditary and neonatal diseases and abnormalities Cerebellum medicine.medical_specialty Ataxia Neuropsychological Tests Audiology Spatial memory medicine Humans Spinocerebellar Ataxias Cognitive Dysfunction Effects of sleep deprivation on cognitive performance Cerebral Cortex business.industry Cognitive flexibility medicine.disease Magnetic Resonance Imaging Memory Short-Term medicine.anatomical_structure Neurology Spinocerebellar ataxia Neurology (clinical) medicine.symptom Verbal memory business Executive dysfunction |
| Zdroj: | Movement Disorders. 36:2910-2921 |
| ISSN: | 1531-8257 0885-3185 |
| Popis: | Background Spinocerebellar ataxia type 10 is a neurodegenerative disorder caused by the expansion of an ATTCT pentanucleotide repeat. Its clinical features include ataxia and, in some cases, epileptic seizures. There is, however, a dearth of information about its cognitive deficits and the neural bases underpinning them. Objectives The objectives of this study were to characterize the performance of spinocerebellar ataxia type 10 patients in 2 cognitive domains typically affected in spinocerebellar ataxias, memory and executive function, and to correlate the identified cognitive impairments with ataxia severity and cerebral/cerebellar cortical thickness, as quantified by MRI. Methods Memory and executive function tests were administered to 17 genetically confirmed Mexican spinocerebellar ataxia type 10 patients, and their results were compared with 17 healthy matched volunteers. MRI was performed in 16 patients. Results Patients showed deficits in visual and visuospatial short-term memory, reduced storage capacity for verbal memory, and impaired monitoring, planning, and cognitive flexibility, which were ataxia independent. Patients with seizures (n = 9) and without seizures (n = 8) did not differ significantly in cognitive performance. There were significant correlations between short-term visuospatial memory impairment and posterior cerebellar lobe cortical thickness (bilateral lobule VI, IX, and right X). Cognitive flexibility deficiencies correlated with cerebral cortical thickness in the left middle frontal, cingulate, opercular, and temporal gyri. Cerebellar cortical thickness in several bilateral regions was correlated with motor impairment. Conclusions Patients with spinocerebellar ataxia type 10 show significant memory and executive dysfunction that can be correlated with deterioration in the posterior lobe of the cerebellum and prefrontal, cingulate, and middle temporal cortices. |
| Databáze: | OpenAIRE |
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