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Cortical Activation Reorganization of Cerebral Regions in Charcot– Marie–Tooth Patients: A Task-state Functional Magnetic Resonance Imaging Study

Author(s): Zhifeng Wang, Ce Han, Chengjie Yuan, Yiyuan Shen, Xiang Geng, Chen Wang, Chao Zhang, Jiazhang Huang, Xin Ma, Hanqiu Liu and Xu Wang

Background: Evidence of altered brain functional activation in response to different tasks has been reported in some peripheral neuropathies. The aim of this study was to investigate possible central nervous system modifications using task-state Functional Magnetic Resonance Imaging (FMRI) in Charcot–Marie–Tooth patients.

Methods: A design of ankle dorsiflexion-plantarflexion and fist clutching paradigm was adopted at a frequency of 1 Hz in the FMRI portion in CMT patients and healthy controls. We acquired 3.0T MRI brain scans. The brain activation MR signals were recorded and a paired voxel-wise t-test was performed. The correlations between FMRI measurement and clinical variables were calculated.

Results: Compared with control group during the ankle dorsiflexionplantarflexion movement, the voxel-wise independent sample t-test revealed that the CMT patients demonstrated statistically more activation of the contralateral precentral gyrus (M1, t=5.01, P<0.001), supplementary motor area (SMA, t=4.58, P=0.038), postcentral gyrus (PCG) (t=4.46, P=0.031), and ipsilateral PCG (t=4.14, P=0.002), temporal lobe (TL) (t=4.73, P<0.001). A significant positive correlation emerged between the CMTNS and increased activated voxels with SMA(r=0.71111, P<0.001), with a significant negative correlation between handgrip strength and increased activated voxels with SMA(r=-0.7073, P<0.001).

Conclusion: This study demonstrated the differences of cortical activation in CMT patients during ankle dorsiflexion-plantarflexion movement and its correlation with clinical severity. These findings give further understanding of the potential mechanism in central nervous system underlying the peripheral nerve pathology in CMT patients, and lay the foundation for longitudinal research and further mechanism investigation.

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