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Diffusion tensor imaging in blepharospasm and blepharospasm-oromandibular dystonia

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Abstract

Patterns of white matter (WM) abnormalities and correlation with clinical features in patients with blepharospasm (BSP) and patients with blepharospasm-oromandibular dystonia (BOM) remain unknown. Using voxel-based analysis, diffusion behaviors of WM including fractional anisotropy (FA), mean diffusivity (MD) and eigenvalues were compared between 20 BSP patients vs. 11 healthy controls (HCs) and 11 patients with BOM vs. 11 HCs. Correlation analyses were performed to assess possible association between diffusion behaviors of significantly different areas and clinical measures. Compared with HCs, BSP patients showed significant FA reductions in the left anterior lobe of cerebellum. Significant increases of MD and radial diffusivity (RD) were detected in right lentiform nucleus and thalamus. Significantly decreased FA in the right precuneus of parietal lobe, increased MD in the right lentiform nucleus and insula, and increased axial diffusivity in the right insula were observed in BOM patients. The FA values in the WM of left cerebellum negatively correlated with disease severity in BSP patients measured by JRS (r = −0.655, p = 0.002). The FA values in the right parietal WM negatively correlated with disease duration in BOM patients (r = −0.745, p = 0.008). Both BSP and BOM are related to microstructural abnormalities of WM in the basal ganglia. WM changes outside the basal ganglia may present trait features that are specific for individual dystonia phenotype. The correlation between FA abnormalities and symptom severity suggests that DTI parameters might be of clinical value in assessing and following disability in BSP patients.

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Acknowledgments

This study was supported by the National Science Foundation of China (Grant No. 30973149). We thank the technical staff of the Department of Radiology for their collaboration and assistance.

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All the authors declare no conflict of interest.

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Authors and Affiliations

  1. Department of Neurology, West China Hospital, Sichuan University, Chengdu, 610041, Sichuan, China

    Jing Yang, ChunYan Luo, Wei Song, XiaoYan Guo, Bi Zhao, XuePing Chen & Hui-Fang Shang

  2. Department of Radiology, Huaxi MR Research Center (HMRRC), West China Hospital, Sichuan University, Chengdu, 610041, Sichuan, China

    XiaoQi Huang & QiYong Gong

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  1. Jing Yang
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  2. ChunYan Luo
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  3. Wei Song
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Correspondence to QiYong Gong or Hui-Fang Shang.

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J. Yang and C. Luo contributed equally to this study.

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Yang, J., Luo, C., Song, W. et al. Diffusion tensor imaging in blepharospasm and blepharospasm-oromandibular dystonia. J Neurol 261, 1413–1424 (2014). https://doi.org/10.1007/s00415-014-7359-y

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  • DOI: https://doi.org/10.1007/s00415-014-7359-y

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