Progressive white matter degeneration in patients with spinocerebellar ataxia type 2.

Purpose: Spinocerebellar ataxia type 2 (SCA2) is a progressive neurodegenerative disorder characterized by cerebellar atrophy. However, studies to elucidate the longitudinal progression of the neuropathology are limited. We sought to identify brain macrostructural and microstructural alterations in...

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Publicado en:Neuroradiology Vol. 66; no. 1; pp. 101 - 109
Autores principales: Tu, Ye, Li, Zheng, Xiong, Fei, Gao, Feng
Formato: diagnostic images research tables/charts Journal Article
Publicado: Springer Nature Jan2024
Acceso en línea:Ver este registro en EBSCOhost
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      dt: Jan2024
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      pub: Springer Nature
      place: New York, New York
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        10.1007/s00234-023-03260-4
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        atl: Progressive white matter degeneration in patients with spinocerebellar ataxia type 2.
      aug:
        au:
          Tu, Ye
          Li, Zheng
          Xiong, Fei
          Gao, Feng
        affil: Department of Anesthesiology, Tongji Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, China
      sug:
        subj:
          Spinocerebellar Ataxias Classification
          Spinocerebellar Ataxias Radiography
          Brain Anatomy and Histology
          Disease Progression Diagnosis
          White Matter Radiography
          White Matter Pathology
          Magnetic Resonance Imaging Methods
          Human
          Prospective Studies
          Paired T-Tests
          Descriptive Statistics
          Brain Stem Pathology
      ab: Purpose: Spinocerebellar ataxia type 2 (SCA2) is a progressive neurodegenerative disorder characterized by cerebellar atrophy. However, studies to elucidate the longitudinal progression of the neuropathology are limited. We sought to identify brain macrostructural and microstructural alterations in patients with SCA2 using fixel-based analysis (FBA) to better understand its distribution patterns and progression. Methods: We enrolled 9 patients with SCA2 and 16 age- and gender-matched controls. Longitudinal clinical and imaging data were collected at baseline, and 3.5 years later. Fiber density (FD), fiber-bundle cross-section (FC), and a combination of FD and FC (FDC) were calculated. The paired t-test was used to examine longitudinal differences. The associations between fixel-based metrics and clinical variables were explored in SCA2 patients. Results: At baseline, patients with SCA2 displayed multiple white matter tracts with significantly decreased FD, FC, and FDC in the corticospinal tract, cerebellar peduncles, brainstem, corpus callosum, thalamus, striatum, and prefrontal cortex, compared to controls. Over time, many of these macrostructural and microstructural alterations progressed, manifesting lower FD, FC, and FDC in corticospinal tract, middle cerebellar peduncle, brainstem, striatum, fornix, and cingulum. No significant brain white matter alterations were found in the healthy controls over time. There was no association between the FBA-derived metrics and clinical variables in SCA2. Conclusion: This study provides evidence of brain macrostructural and microstructural alterations and of progression over time in SCA2. The FBA-derived metrics may serve as potential biomarkers of SCA2 progression.
      pubtype: Academic Journal
      doctype:
        diagnostic images
        research
        tables/charts
        Journal Article
      ougenre: Article
    language: English
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