Cerebral white matter abnormalities in patients with charcot-marie-tooth disease

被引:25
作者
Lee, Mina [1 ,2 ]
Park, Chang-hyun [1 ,2 ]
Chung, Hwa-Kyung [1 ]
Kim, Hyeon Jin [1 ]
Choi, Yunseo [1 ,2 ]
Yoo, Jeong Hyun [3 ,4 ]
Yoon, Young Chul [5 ]
Hong, Young Bin [6 ]
Chung, Ki Wha [8 ]
Choi, Byung-Ok [6 ,7 ]
Lee, Hyang Woon [1 ,2 ]
机构
[1] Ewha Womans Univ, Sch Med, Dept Neurol, 1071 Anyangcheon Ro, Seoul 07985, South Korea
[2] Ewha Womans Univ, Sch Med, Dept Med Sci, 1071 Anyangcheon Ro, Seoul 07985, South Korea
[3] Ewha Womans Univ, Sch Med, Dept Radiol, Seoul 07985, South Korea
[4] Ewha Med Res Inst, 1071 Anyangcheon Ro, Seoul 07985, South Korea
[5] Sungkyunkwan Univ, Sch Med, Samsung Med Ctr, Dept Radiol, Seoul, South Korea
[6] Sungkyunkwan Univ, Sch Med, Samsung Med Ctr, Dept Neurol, Seoul, South Korea
[7] Samsung Med Ctr, Neurosci Ctr, Seoul, South Korea
[8] Kongju Natl Univ, Dept Biol Sci, Gongju, South Korea
基金
新加坡国家研究基金会;
关键词
EARLY-ONSET SEVERE; MUTATIONS; RELIABILITY; VALIDITY;
D O I
10.1002/ana.24824
中图分类号
R74 [神经病学与精神病学];
学科分类号
摘要
Here, we report the structural evidence of cerebral white matter abnormalities in Charcot-Marie-Tooth (CMT) patients and the relationship between these abnormalities and clinical disability. Brain diffusion tensor imaging (DTI) was performed in CMT patients with demyelinating (CMT1A/CMT1E), axonal (CMT2A/CMT2E), or intermediate (CMTX1/DI-CMT) peripheral neuropathy. Although all patients had normal brain magnetic resonance imaging, all genetic subgroups except CMT1A had abnormal DTI findings indicative of significant cerebral white matter abnormalities: decreased fractional anisotropy and axial diffusivity, and increased radial diffusivity. DTI abnormalities were correlated with clinical disability, suggesting that there is comorbidity of central nervous system damage with peripheral neuropathy in CMT patients. ANN NEUROL 2017;81:147-151
引用
收藏
页码:147 / 151
页数:5
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