Abstract
Williams syndrome (WS) is a genetically based neurodevelopmental disorder characterized by intellectual disability and impaired visuospatial recognition. The aim of this study was to analyze the gait characteristics of WS children with impaired visuospatial recognition using a three-dimensional gait analysis (3DGA) to clarify the gait adaptation needed to compensate for it. 3DGA was performed in 8 WS children with impaired visuospatial recognition (mean age, 11.8 years) and 9 age-, sex-, height-, and weight-matched controls. Clinical data, fundamental motor tests, and gait variables while walking on a flat surface and walking up a mat were compared between the two groups, and the correlations between variables were analyzed in the WS children. WS children showed impairment of balance function without muscle weakness. In walking on a flat surface, the WS group showed reduced walking speed, short step length, increased variability of step length, increased knee flexion throughout the stance phase, increased horizontal pelvic range of motion (ROM), and a low Gait Deviation Index and a high Gait Profile Score, which are indices of gait quality. In walking up a mat, the WS group showed further reduced walking speed and decreased sagittal hip flexion and ankle dorsiflexion ROM in the swing phase. Impaired balance function was significantly correlated with increased variability of step length and decreased sagittal ankle dorsiflexion ROM in the swing phase. The detailed gait pattern of WS children with impaired visuospatial recognition was presented. These findings show that impaired visuospatial recognition and balance function contribute to gait adaptation.
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Data availability
The datasets generated during and/or analyzed during the current study are available from the corresponding author on reasonable request.
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This work was supported by Grant-in-Aid for Scientific Research (C) 15K09609 and 19K02637 by the Ministry of Education, Science, Sports and Culture, Japan.
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Conception and design: IY, IT, NM. Acquisition of data: IY, IT, KN, ON, NK, NM. Analysis and interpretation of data: IY, IT. Drafting the article: IY. Critically revising the article: IT, NM. Reviewed submitted version of manuscript: KN, ON, NK, SH, MS, KH, NJ. Administrative/technical/material support: ON, NK, SH, MS, KH, NJ, NM.
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Jun Natsume belongs to the Department of the Developmental Disability Medicine in Nagoya University Graduate School of Medicine, which is the laboratory endowed by Aichi Prefecture. The authors declare that they have no known competing financial interests or personal relationships that could have appeared to influence the work reported in this paper.
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This cross-sectional study was approved by the research ethics board of Aichi Prefecture Mikawa Aoitori Medical and Rehabilitation Center for Developmental Disabilities (approval number RH30001).
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After written, informed consent was obtained from the parents, participants in this study were enrolled between November 2018 and August 2019 at our hospital.
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Communicated by Francesco Lacquaniti.
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Ito, Y., Ito, T., Kurahashi, N. et al. Gait characteristics of children with Williams syndrome with impaired visuospatial recognition: a three-dimensional gait analysis study. Exp Brain Res 238, 2887–2895 (2020). https://doi.org/10.1007/s00221-020-05946-0
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DOI: https://doi.org/10.1007/s00221-020-05946-0