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Association Between Body Mass Index and Disease Severity in Chinese Spinocerebellar Ataxia Type 3 Patients

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Abstract

Spinocerebellar ataxia type 3 (SCA3), the most common subtype of SCA worldwide, is caused by mutation of CAG repeats expansion in ATXN3. Body mass index (BMI) is an important modulatory factor in the progression of neurodegenerative disorders such as Huntington disease and amyotrophic lateral sclerosis. However, its relevance in SCA3 is not well understood. In this study, BMI was investigated in 134 molecularly confirmed SCA3 patients and 136 healthy controls from China. The multivariable linear regression models were performed to establish the putative risk factors for BMI, and whether BMI could affect the severity of ataxia. We found that BMI was significantly lower in the case group than that in the control group. The age at onset (positive correlation) and severity of ataxia (negative correlation) were the risk factors affecting BMI. Conversely, BMI along with the disease duration, the age at onset, and the numbers of CAG repeats could also have influence on the severity of ataxia. In conclusion, SCA3 patients had lower BMI than matched controls and BMI is a predictor of disease progression in SCA3. Nutritional intervention to promote weight gain could be a promising strategy to impede SCA3 progression.

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Acknowledgements

The authors sincerely thank the participants for their help and willingness to participate in this study. We also thank the reviewers for their helpful comments.

Funding

This work was supported by The National Natural Science Foundation of China to S-R Gan (81100851, Beijing), N Wang (U1505222, Beijing), and X-P Chen (11601083, Beijing). This work was also supported by the Natural Science Foundation of Fujian Province to X-P Chen (2016J05002, Fuzhou) and J-S Yang (2017J05123, Fuzhou). This work was also supported by the Startup Fund for scientific research, Fujian Medical University to J-S Yang (2016QH067, Fuzhou).

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Author notes

  1. Jin-Shan Yang and Ping-Ping Chen contributed equally to this work.

Authors and Affiliations

  1. Department of Neurology and Institute of Neurology, First Affiliated Hospital of Fujian Medical University, 20 Chazhong Road, Fuzhou, 350005, China

    Jin-Shan Yang, Ping-Ping Chen, Min-Ting Lin, Mei-Zhen Qian, Hui-Xia Lin, Dan-Ni Wang, Yu-Chao Chen, Bin Jiang, Yi-Jun Chen, Ning Wang, Wan-Jin Chen & Shi-Rui Gan

  2. Fujian Key Laboratory of Molecular Neurology, 20 Chazhong Road, Fuzhou, 350005, China

    Jin-Shan Yang, Ping-Ping Chen, Min-Ting Lin, Mei-Zhen Qian, Hui-Xia Lin, Dan-Ni Wang, Yu-Chao Chen, Bin Jiang, Yi-Jun Chen, Ning Wang, Wan-Jin Chen & Shi-Rui Gan

  3. Interdisciplinary Institute of Neuroscience and Technology, Zhejiang University, Hangzhou, China

    Mei-Zhen Qian

  4. School of Mathematics and Computer Science & FJKLMAA, Fujian Normal University, Fuzhou, China

    Xiao-Ping Chen

  5. Department of Neurology, Yijishan Hospital of Wannan Medical College, Wuhu, China

    Xian-Jin Shang

  6. Department of Neurology, The First Affiliated Hospital of Xiamen University, Xiamen, China

    Bin Jiang

Authors
  1. Jin-Shan Yang
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  2. Ping-Ping Chen
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  3. Min-Ting Lin
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  5. Hui-Xia Lin
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  7. Xian-Jin Shang
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  8. Dan-Ni Wang
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  9. Yu-Chao Chen
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  10. Bin Jiang
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  11. Yi-Jun Chen
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  12. Ning Wang
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  13. Wan-Jin Chen
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  14. Shi-Rui Gan
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Contributions

Dr. J-S Yang: study concept and design, statistical analysis and interpretation, writing the manuscript, critical revision of the manuscript for important intellectual content

Dr. P-P Chen: study concept and design, statistical analysis and interpretation, critical revision of the manuscript for important intellectual content

Bs. M-T LIN: acquisition of data

Dr. M-Z Qin: study concept and design, acquisition of data

Dr. H-X Lin: study concept and design, acquisition of data

Prof. X-P Chen: statistical analysis and interpretation

Dr. X-J Shang: acquisition of data

Dr. D-N Wang: statistical analysis and interpretation

Dr. Y-C Chen: acquisition of data

Dr. B Jiang: acquisition of data

Dr. Y-J Chen: acquisition of data

Dr. N Wang: study concept and design, acquisition of data, study supervision

Dr. W-J Chen: study concept and design, acquisition of data, critical revision of the manuscript for important intellectual content, study supervision

Dr. S-R Gan: study concept and design, acquisition of data, analysis and interpretation, critical revision of the manuscript for important intellectual content

Corresponding authors

Correspondence to Wan-Jin Chen or Shi-Rui Gan.

Ethics declarations

The study was approved by the ethics committee of the First Affiliated Hospital of Fujian Medical University. Written informed consent forms were signed by all subjects.

Conflict of Interest

The authors declare that they have no conflict of interest.

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Cite this article

Yang, JS., Chen, PP., Lin, MT. et al. Association Between Body Mass Index and Disease Severity in Chinese Spinocerebellar Ataxia Type 3 Patients. Cerebellum 17, 494–498 (2018). https://doi.org/10.1007/s12311-018-0929-2

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  • DOI: https://doi.org/10.1007/s12311-018-0929-2

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