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Estimation of ataxia severity in children with ataxia-telangiectasia using ankle-worn sensors

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Data availability

The data that support the findings of this study are available on request from the corresponding author. The data are not publicly available due to privacy or ethical restrictions.

References

  1. Levy A, Lang AE (2018) Ataxia-telangiectasia: a review of movement disorders, clinical features, and genotype correlations: Ataxia-Telangiectasia. Mov Disord 33:1238–1247. https://doi.org/10.1002/mds.27319

    Article  PubMed  Google Scholar 

  2. Bürk K, Sival DA (2018) Scales for the clinical evaluation of cerebellar disorders. In: Handbook of Clinical Neurology. Elsevier, pp 329–339

  3. Brandsma R, Spits AH, Kuiper MJ et al (2014) Ataxia rating scales are age-dependent in healthy children. Dev Med Child Neurol 56:556–563. https://doi.org/10.1111/dmcn.12369

    Article  PubMed  Google Scholar 

  4. Lawerman TF, Brandsma R, Burger H et al (2017) Age-related reference values for the pediatric Scale for Assessment and Rating of Ataxia: a multicentre study. Dev Med Child Neurol 59:1077–1082. https://doi.org/10.1111/dmcn.13507

    Article  PubMed  Google Scholar 

  5. Ilg W, Seemann J, Giese M et al (2020) Real-life gait assessment in degenerative cerebellar ataxia: toward ecologically valid biomarkers. Neurology 95:e1199–e1210. https://doi.org/10.1212/WNL.0000000000010176

    Article  CAS  PubMed  Google Scholar 

  6. Lee J, Oubre B, Daneault J-F et al (2022) Analysis of gait sub-movements to estimate ataxia severity using ankle inertial data. IEEE Trans Biomed Eng 69:2314–2323. https://doi.org/10.1109/TBME.2022.3142504

    Article  PubMed  Google Scholar 

  7. Shah VV, Rodriguez-Labrada R, Horak FB et al (2021) Gait variability in spinocerebellar ataxia assessed using wearable inertial sensors. Mov Disord 36:2922–2931. https://doi.org/10.1002/mds.28740

    Article  PubMed  Google Scholar 

  8. Miranda JGV, Daneault J-F, Vergara-Diaz G et al (2018) Complex upper-limb movements are generated by combining motor primitives that scale with the movement size. Sci Rep 8:12918. https://doi.org/10.1038/s41598-018-29470-y

    Article  CAS  PubMed Central  PubMed  Google Scholar 

  9. Oubre B, Daneault J-F, Whritenour K et al (2021) Decomposition of reaching movements enables detection and measurement of ataxia. Cerebellum 20:811–822. https://doi.org/10.1007/s12311-021-01247-6

    Article  PubMed Central  PubMed  Google Scholar 

  10. Gupta AS, Luddy AC, Khan NC et al (2022) Real-life wrist movement patterns capture motor impairment in individuals with ataxia-telangiectasia. Cerebellum 22:261–271. https://doi.org/10.1007/s12311-022-01385-5

    Article  PubMed Central  PubMed  Google Scholar 

  11. Schmahmann JD, Gardner R, MacMore J, Vangel MG (2009) Development of a brief ataxia rating scale (BARS) based on a modified form of the ICARS: Brief Ataxia Rating Scale. Mov Disord 24:1820–1828. https://doi.org/10.1002/mds.22681

    Article  PubMed Central  PubMed  Google Scholar 

  12. Hickey A, Din SD, Rochester L, Godfrey A (2016) Detecting free-living steps and walking bouts: validating an algorithm for macro gait analysis. Physiol Meas 38:N1–N15. https://doi.org/10.1088/1361-6579/38/1/n1

    Article  PubMed  Google Scholar 

  13. Summa S, Tartarisco G, Favetta M et al (2020) Validation of low-cost system for gait assessment in children with ataxia. Comput Methods Programs Biomed 196:105705. https://doi.org/10.1016/j.cmpb.2020.105705

    Article  CAS  PubMed  Google Scholar 

  14. Milne SC, Kim SH, Murphy A et al (2021) The responsiveness of gait and balance outcomes to disease progression in friedreich ataxia. Cerebellum 21:963–975. https://doi.org/10.1007/s12311-021-01348-2

    Article  PubMed  Google Scholar 

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Acknowledgements

This study was supported by A-T Children’s Project and National Institute of Neurological Disorders and Stroke (NINDS) R01 NS117826. The authors would like to thank Jennifer Thornton and Sara Reiling of the A-T Children’s Project for support throughout the study; Mary Donovan, Winnie Ching, and Nergis Khan for recruitment and data collection; and the children and families who collaborated with us on this project.

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Authors and Affiliations

  1. College of Information and Computer Sciences, University of Massachusetts Amherst, 140 Governors Dr, Amherst, MA, USA

    Juhyeon Lee, Brandon Oubre & Sunghoon Ivan Lee

  2. Department of Rehabilitation and Movement Sciences, Rutgers University, 65 Bergen St, Newark, NJ, USA

    Jean-Francois Daneault

  3. Department of Neurology, Massachusetts General Hospital, Harvard Medical School, 100 Cambridge St, Boston, MA, USA

    Brandon Oubre & Anoopum S. Gupta

Authors
  1. Juhyeon Lee
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  2. Brandon Oubre
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  3. Jean-Francois Daneault
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  4. Sunghoon Ivan Lee
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  5. Anoopum S. Gupta
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Corresponding authors

Correspondence to Sunghoon Ivan Lee or Anoopum S. Gupta.

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Conflicts of interest

The authors report that they have no conflict of interest.

Ethical standards

The study protocol was approved by the Institutional Review Board at Massachusetts General Hospital, and all participants provided written informed assent and/or consent.

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Lee, J., Oubre, B., Daneault, JF. et al. Estimation of ataxia severity in children with ataxia-telangiectasia using ankle-worn sensors. J Neurol 270, 5097–5101 (2023). https://doi.org/10.1007/s00415-023-11786-z

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  • DOI: https://doi.org/10.1007/s00415-023-11786-z

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