期刊
EUROPEAN JOURNAL OF NEUROLOGY
卷 21, 期 8, 页码 1131-1136出版社
WILEY
DOI: 10.1111/ene.12448
关键词
dentate nuclei; Friedreich's ataxia; MRI; T2 relaxometry
资金
- FAPESP (Fundacao de Amparo a Pesquisa do Estado de Sao Paulo)
- CNPq (Conselho Nacional de Pesquisa-BRAZIL)
Background and purpose: In Friedreich's ataxia (FRDA), frataxin deficiency results in iron redistribution in the dentate nuclei (DNC). Clusters of iron cause inhomogeneities in a magnetic field and result in a reduction in T2 relaxation time (T2). Methods: T2 was prospectively evaluated in DNC, putamen, substantia nigra (SN), cerebellar white matter (CWM) and caudate and the correlation with clinical parameters was investigated. Thirty-five patients (range 9-51 years) and 44 controls (12-49 years) underwent T2 multi-echo sequence in a 3T scanner. Twenty-three patients (12-50 years) and 19 controls (14-49 years) were reassessed after 1 year. T2 was evaluated using specialized software (Aftervoxel) and severity of disease was quantified with the Friedreich Ataxia Rating Scale (FARS). Results: T2 of both DNC was significantly shorter in the FRDA group at baseline (right, 58.6 +/- 8.3 ms vs. 63.7 +/- 8.1 ms, P = 0.013; left, 56.7 +/- 7.7 ms vs. 62.6 +/- 6.8 ms, P = 0.001). No significant difference was found between groups regarding the SN, putamen, CWM and caudate T2. DNC T2 values correlated with age, FARS total score and FARS III subscore on both sides. Prospectively, there was a significant reduction of T2 in FRDA patients in right and left DNC (P = 0.001 and 0.009) but not in other structures. Amongst controls, none of the regions significantly changed after 1 year. DNC T2 change over time correlated with GAA expansions and clinical deterioration (expressed by a change in FARS scores). Conclusions: DNC T2 values are abnormal in FRDA, progress over time and correlate with ataxia severity. These results strongly suggest that DNC relaxometry can be a useful neuroimaging marker in FRDA.
作者
我是这篇论文的作者
点击您的名字以认领此论文并将其添加到您的个人资料中。
推荐
暂无数据