Proximal nerve magnetization transfer MRI relates to disability in Charcot-Marie-Tooth diseases
Proximal nerve magnetization transfer MRI relates to disability in Charcot-Marie-Tooth diseases
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DOI：
10.1212/wnl.0000000000000919
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发表时间：
2014-10-21
期刊：
影响因子：
9.9
通讯作者：
Li, Jun
中科院分区：
文献类型：
--
作者：
Dortch, Richard D.;Dethrage, Lindsey M.;Li, Jun
目的：本研究的目的是（1）开发一种新的近端坐骨神经（SN）的磁化传递比（MTR）MRI测定，其通过当前用于评估周围神经的工具是不可及的，以及（2）评估所得MTR值作为Charcot-Marie-Tooth（CMT）疾病患者中髓鞘含量变化的潜在生物标志物。在CMT 1A型（CMT 1A，n = 10）、CMT 2A型（CMT 2A，n = 3）、遗传性压力性神经病（n = 3）和健康对照（n = 21）患者的SN中测量MTR。还纳入了其他没有遗传学证实亚型的患者（n = 4），但其家族史和电生理学检查与CMT一致。MTR和临床神经病变评分之间的关系进行了评估，和interscan和inter-rater可靠性MTRestimated.Results：平均体积MTR值显着降低，在SN的患者CMT 1A（33.8 +/-3.3%单位）和CMT 2A（31.5 +/-1.9%单位）相对于对照组（37.2 +/-2.3%单位）。还检测到MTR和残疾评分之间的显著关系（仅遗传学确诊患者p = 0.01，所有患者p = 0.04）。从interscan和inter-rater可靠性分析，近端神经MTR值是可重复的切片和平均体积levels.Conclusions：MTR测量可能是一个可行的生物标志物近端神经病理CMT患者。
Objective: The objectives of this study were (1) to develop a novel magnetization transfer ratio (MTR) MRI assay of the proximal sciatic nerve (SN), which is inaccessible via current tools for assessing peripheral nerves, and (2) to evaluate the resulting MTR values as a potential biomarker of myelin content changes in patients with Charcot-Marie-Tooth (CMT) diseases.Methods: MTR was measured in the SN of patients with CMT type 1A (CMT1A, n = 10), CMT type 2A (CMT2A, n = 3), hereditary neuropathy with liability to pressure palsies (n = 3), and healthy controls (n = 21). Additional patients without a genetically confirmed subtype (n = 4), but whose family histories and electrophysiologic tests were consistent with CMT, were also included. The relationship between MTR and clinical neuropathy scores was assessed, and the interscan and inter-rater reliability of MTR was estimated.Results: Mean volumetric MTR values were significantly decreased in the SN of patients with CMT1A (33.8 +/- 3.3 percent units) and CMT2A (31.5 +/- 1.9 percent units) relative to controls (37.2 +/- 2.3 percent units). A significant relationship between MTR and disability scores was also detected (p = 0.01 for genetically confirmed patients only, p = 0.04 for all patients). From interscan and inter-rater reliability analyses, proximal nerve MTR values were repeatable at the slicewise and mean volumetric levels.Conclusions: MTR measurements may be a viable biomarker of proximal nerve pathology in patients with CMT.