title: Cerebral white matter abnormalities in patients with charcot-marie-tooth disease.
abstract: 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.
authors.affiliation: Department of Neurology, Ewha Womans University School of Medicine and Ewha Medical Research Institute, Seoul, South Korea.|Department of Medical Science, Ewha Womans University School of Medicine and Ewha Medical Research Institute, Seoul, South Korea.
authors.forename: Mina
authors.initials: M
authors.lastname: Lee
authors.affiliation: Department of Neurology, Ewha Womans University School of Medicine and Ewha Medical Research Institute, Seoul, South Korea.|Department of Medical Science, Ewha Womans University School of Medicine and Ewha Medical Research Institute, Seoul, South Korea.
authors.forename: Chang-Hyun
authors.initials: CH
authors.lastname: Park
authors.affiliation: Department of Neurology, Ewha Womans University School of Medicine and Ewha Medical Research Institute, Seoul, South Korea.
authors.forename: Hwa-Kyung
authors.initials: HK
authors.lastname: Chung
authors.affiliation: Department of Neurology, Ewha Womans University School of Medicine and Ewha Medical Research Institute, Seoul, South Korea.
authors.forename: Hyeon Jin
authors.initials: HJ
authors.lastname: Kim
authors.affiliation: Department of Neurology, Ewha Womans University School of Medicine and Ewha Medical Research Institute, Seoul, South Korea.|Department of Medical Science, Ewha Womans University School of Medicine and Ewha Medical Research Institute, Seoul, South Korea.
authors.forename: Yunseo
authors.initials: Y
authors.lastname: Choi
authors.affiliation: Department of Radiology, Ewha Womans University School of Medicine and Ewha Medical Research Institute, Seoul, South Korea.
authors.forename: Jeong Hyun
authors.initials: JH
authors.lastname: Yoo
authors.affiliation: Department of Radiology, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, South Korea.
authors.forename: Young Chul
authors.initials: YC
authors.lastname: Yoon
authors.affiliation: Department of Neurology, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, South Korea.
authors.forename: Young Bin
authors.initials: YB
authors.lastname: Hong
authors.affiliation: Department of Biological Science, Kongju National University, Gongju, South Korea.
authors.forename: Ki Wha
authors.initials: KW
authors.lastname: Chung
authors.affiliation: Department of Neurology, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, South Korea.|Neuroscience Center, Samsung Medical Center, Seoul, South Korea.
authors.forename: Byung-Ok
authors.initials: BO
authors.lastname: Choi
authors.affiliation: Department of Neurology, Ewha Womans University School of Medicine and Ewha Medical Research Institute, Seoul, South Korea.|Department of Medical Science, Ewha Womans University School of Medicine and Ewha Medical Research Institute, Seoul, South Korea.
authors.forename: Hyang Woon
authors.initials: HW
authors.lastname: Lee
mesh_terms: D016880:Anisotropy; D016022:Case-Control Studies; D002607:Charcot-Marie-Tooth Disease / Q000235:genetics / Q000473:pathology*; D056324:Diffusion Tensor Imaging; D004185:Disability Evaluation; D005260:Female; D006801:Humans; D008297:Male; D009154:Mutation; D059906:Neuroimaging; D010523:Peripheral Nervous System Diseases / Q000473:pathology*; D066127:White Matter / Q000473:pathology*