Journal of neurology · 2024
Tract-specific spinal damage in SCA2, SCA3 and SCA6.
СтатьяНазвание и аннотация: Перевод в очереди
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de Borba Fabrício Castro, Fernandes Joyce Macedo Sanches, de Rezende Thiago Junqueira Ribeiro, González-Salazar Carelis, de Melo Teixeira Branco Lucas, Wolmer Paulo Schneider, Pedroso José Luiz, Barsottini Orlando Graziani Povoas, França Junior Marcondes Cavalcante
Аннотация
BACKGROUND: Spinocerebellar ataxias (SCAs) are a group of neurodegenerative disorders characterized by progressive ataxia. Although previous studies have focused on cerebral and cerebellar damage, spinal cord involvement in SCAs remains underexplored.
OBJECTIVES: This study aims to characterize spinal cord abnormalities in SCA2, SCA3, and SCA6 and to identify its phenotypic correlates.
METHODS: We conducted a multimodal spinal neuroimaging study on 26 SCA3, 16 SCA2, and 14 SCA6 patients, along with matched healthy controls. MRI scanning was performed using a 3 Tesla device, and the Spinal Cord Toolbox (SCT) was employed for morphometric and diffusivity analyses of the cervical spinal cord.
RESULTS: Our findings revealed significant spinal cord atrophy and altered white matter microstructural metrics in SCA3 and SCA2 patients compared to controls, with no abnormalities in SCA6. A strong negative correlation was observed between cross-sectional cord area and disease duration in SCA2, suggesting its potential as a biomarker for disease progression.
CONCLUSIONS: This study highlights the importance of spinal cord imaging in understanding the pathophysiology of SCAs and demonstrates the utility of MRI-based metrics in identifying structural deviations and their clinical correlates. Further longitudinal studies are needed to validate these findings and explore their implications for clinical trials and therapeutic interventions.
OBJECTIVES: This study aims to characterize spinal cord abnormalities in SCA2, SCA3, and SCA6 and to identify its phenotypic correlates.
METHODS: We conducted a multimodal spinal neuroimaging study on 26 SCA3, 16 SCA2, and 14 SCA6 patients, along with matched healthy controls. MRI scanning was performed using a 3 Tesla device, and the Spinal Cord Toolbox (SCT) was employed for morphometric and diffusivity analyses of the cervical spinal cord.
RESULTS: Our findings revealed significant spinal cord atrophy and altered white matter microstructural metrics in SCA3 and SCA2 patients compared to controls, with no abnormalities in SCA6. A strong negative correlation was observed between cross-sectional cord area and disease duration in SCA2, suggesting its potential as a biomarker for disease progression.
CONCLUSIONS: This study highlights the importance of spinal cord imaging in understanding the pathophysiology of SCAs and demonstrates the utility of MRI-based metrics in identifying structural deviations and their clinical correlates. Further longitudinal studies are needed to validate these findings and explore their implications for clinical trials and therapeutic interventions.