Dentate nuclei T2 relaxometry is a reliable neuroimaging marker in Friedreich's ataxia
Dentate nuclei T2 relaxometry is a reliable neuroimaging marker in Friedreich's ataxia
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DOI：
10.1111/ene.12448
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发表时间：
2014-08-01
影响因子：
5.1
通讯作者：
Franca, M. C., Jr.
中科院分区：
文献类型：
--
作者：
Bonilha da Silva, C.;Bergo, F. P. G.;Franca, M. C., Jr.
背景和目的：在弗里德赖希共济失调（FRDA）中，共济失调蛋白缺乏导致齿状核（DNC）中铁的重新分布。方法：采用前瞻性研究方法，对DNC、壳核、黑质、小脑白色质和尾状核的T2进行了测定，并分析了T2与临床参数的相关性。35例患者（范围9-51岁）和44例对照（12-49岁）在3 T扫描仪中接受了T2多回波序列。23例患者（12-50岁）和19例对照（14-49岁）在1年后重新评估。使用专门的软件对T2进行评价结果：法尔斯组基线时两种DNC的T2均明显短于正常对照组（P < 0. 05（右侧，58.6 ± 8.3 ms vs. 63.7 ± 8.1 ms，P = 0.013;左侧，56.7 ± 7.7 ms vs. 62.6 ± 6.8 ms，P = 0.001）。两组间SN、壳核、CWM和尾状核T2无显著差异。双侧DNC T2值与年龄、法尔斯总分及法尔斯III分相关。此外，FRDA患者右侧和左侧DNC的T2显著降低（P = 0.001和0.009），但在其他结构中没有。在对照组中，1年后没有任何区域发生显著变化。DNC T2随时间的变化与GAA扩张和临床恶化（由法尔斯评分的变化表示）相关。结论：DNC T2值在FRDA中异常，随时间进展并与共济失调严重程度相关。这些结果强烈表明，DNC弛豫可以是一个有用的神经影像学标记FRDA。
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.