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

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• 作者：Mina Lee ; Chang-hyun Park ; Hwa-Kyung Chung ; Hyeon Jin Kim ; Yunseo Choi ; Jeong Hyun Yoo ; Young Chul Yoon ; Young Bin Hong ; Ki Wha Chung ; Byung-Ok Choi and Hyang Woon Lee
• 刊名：Annals of Neurology
• 出版年：2017
• 出版时间：January 2017
• 年：2017
• 卷：81
• 期：1
• 页码：147-151
• 全文大小：266K
• ISSN：1531-8249

文摘

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