Characteristics of neuropsychological development level in  children with spinal muscular atrophy

PENG Xin-yue; XUE Yang; SHAN Ling; JIA Fei-yong; DU Lin

doi:10.11852/zgetbjzz2022-1262

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Chinese Journal of Child Health Care ›› 2023, Vol. 31 ›› Issue (2) : 195-199. DOI: 10.11852/zgetbjzz2022-1262

Clinical Research

Characteristics of neuropsychological development level in children with spinal muscular atrophy

PENG Xin-yue¹, XUE Yang², SHAN Ling², JIA Fei-yong², DU Lin²

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Abstract

Objective To explore the characteristics of neuropsychological development in children with spinal muscular atrophy (SMA), so as to provide theoretical basis for promoting the overall development of SMA children and further enriching and improving the rehabilitation treatment plan for SMA children. Methods From January 2019 to August 2022, 16 children diagnosed with SMA in the Department of Developmental Behavior Pediatrics of the First Hospital of Jilin University were selected into SMA group, and 19 healthy children matched by age and gender were enrolled as the control group.The Griffiths Mental Development Scales-Chinese (GDS-C) was used to evaluate the developmental levels of the children, and the differences were analyzed between the two groups.HammerSmith Functional Motor Scale Expanded (HFMSE), the Children′s Hospital of Philadelphia Infant Test of Neuromuscular Disorders(CHOP INTEND) and Revised Upper Limb Module(RULM) were used to assess the motor function of SMA children. Results The development levels of motor (t=-27.592), personal-social(t=-7.485), hearing and language(t=-3.216), hand-eye coordination(t=-4.709), performance(t=-4.681) and reasoning(t=-2.468) in SMA children were significantly lower than those of the healthy control(P＜0.05).The development quotients of motor and personal-social in GDS-C were significantly correlated with age of SMA children(r=-0.625, -0.611, P＜0.05).The HFMSE score was positively correlated with motor(r=0.805), hand-eye coordination(r=0.503) and performance(r=0.590) development quotient in GDS-C(P＜0.05).CHOP INTEND score was positively correlated with motor(r=0.564), hand-eye coordination(r=0.587) and performance(r=0.759) development quotient in GDS-C(P＜0.05). RULM score was positively correlated with performance(r=0.643) development quotient in GDS-C(P＜0.05). Conclusion Compared with healthy children with the same age, SMA children have developmental delay in other areas apart from motor development.

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spinal muscular atrophy / development / Griffiths Mental Development Scales-Chinese / children

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PENG Xin-yue, XUE Yang, SHAN Ling, JIA Fei-yong, DU Lin. Characteristics of neuropsychological development level in children with spinal muscular atrophy[J]. Chinese Journal of Child Health Care. 2023, 31(2): 195-199 https://doi.org/10.11852/zgetbjzz2022-1262

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