A new MRI marker of ataxia with oculomotor apraxia
A new MRI marker of ataxia with oculomotor apraxia
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DOI：
10.1016/j.ejrad.2018.11.035
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发表时间：
2019-01-01
影响因子：
3.3
通讯作者：
Tilikete, Caroline
中科院分区：
文献类型：
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作者：
Ronsin, Solene;Hannoun, Salem;Tilikete, Caroline
目的：评价磁敏感加权成像（SWI）齿状核（DN）低信号消失在眼神经性失用症（AOA）患者中的特异性和敏感性。在这项前瞻性研究中，27例常染色体遗传性共济失调患者，（AOA（n = 11），弗里德赖希共济失调和共济失调伴维生素E缺乏（n = 4），和显性遗传性共济失调（n = 12））沿着15名健康对照。对MRI进行定性分类，以确定FLAIR和SWI序列上是否存在DN低信号。然后对MRI进行定量研究，通过手动描绘测量DN与脑干白色物质信号强度的比值。机构审查委员会批准了本研究，并获得了书面知情同意书。结果：11例AOA患者均无DN SWI和FLAIR低信号，3例显性遗传性共济失调患者SWI DN呈中度低信号，FLAIR低信号缺失，13例AOA患者SWI和FLAIR DN均正常。DN SWI低信号缺失对AOA的敏感性和特异性为100%。定量信号强度比AOA组的（平均值+/-标准差）（98.96 ± 5.37%）显著高于对照组（76.40 ± 8.34%; p < 0.001），显性遗传性共济失调组（81.15 +/- 9.94%; p < 0.001），Friedreich共济失调和共济失调伴维生素E缺乏组（87.56 +/- 2.78%; p < 0.02）。这项小型研究表明，在3 T下SWI和FLAIR成像上齿状核正常低信号的丢失是一种高度敏感和特异的方法。AOA的生物标志物。
Purpose: Evaluate the specificity and sensitivity of disappearance of susceptibility weighted imaging (SWI) dentate nuclei (DN) hypointensity in oculomotor apraxia patients (AOA).Method: In this prospective study, 27 patients with autosomal genetic ataxia (AOA (n = 11), Friedreich ataxia and ataxia with vitamin E deficit (n = 4), and dominant genetic ataxia (n = 12)) were included along with fifteen healthy controls. MRIs were qualitatively classified for the presence or absence of DN hypointensity on FLAIR and SWI sequences. The MRIs were then quantitatively studied, with measurement of a ratio of DN over brainstem white matter signal intensity through manual delineation. The institutional review board approved this study, and written informed consent was obtained. In the cross-sectional analysis, the Mann-Whitney test was applied.Results: Qualitatively, the eleven AOA patients presented absence of both DN SWI and FLAIR hyposignals; three dominant genetic ataxia patients had moderate SWI DN hyposignal and absent FLAIR hyposignal; the thirteen remaining subjects presented normal SWI and FLAIR DN hyposignal. Absence of DN SWI hypointensity was 100% sensitive and specific to AOA. Quantitative signal intensity ratio (mean +/- standard deviation) of the AOA group (98.96 +/- 5.37%) was significantly higher than in control subjects group (76.40 +/- 8.34%; p < 0.001), dominant genetic ataxia group (81.15 +/- 9.94%; p < 0.001), and Friedreich ataxia and ataxia with vitamin E deficit group (87.56 +/- 2.78%; p < 0.02).Conclusion: This small study shows that loss of the normal hypointensity in the dentate nucleus on both SWI and FLAIR imaging at 3T is a highly sensitive and specific biomarker for AOA.