Characteristics of motor function in children with hereditary neuromuscular disease
Objective To analyze the characteristics of motor function in children with different types of hereditary neuromuscular disease (HNMD). Methods A total of 61 children with HNMD admitted to Peking University First Hospital from January 2018 to January 2024 were enrolled. Manual Muscle Testing was u...
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Tianjin Huanhu Hospital
2025-07-01
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| Series: | Chinese Journal of Contemporary Neurology and Neurosurgery |
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| Online Access: | http://www.cjcnn.org/index.php/cjcnn/article/view/3066 |
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| author | LI Wen-zhu HUANG Zhen FAN Yan-bin XIONG Hui |
| author_facet | LI Wen-zhu HUANG Zhen FAN Yan-bin XIONG Hui |
| author_sort | LI Wen-zhu |
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| description | Objective To analyze the characteristics of motor function in children with different types of hereditary neuromuscular disease (HNMD). Methods A total of 61 children with HNMD admitted to Peking University First Hospital from January 2018 to January 2024 were enrolled. Manual Muscle Testing was used to evaluate the muscle strength of the limbs, and the percentage of limb muscle strength was subsequently calculated. The Motor Function Measure (MFM) was used to evaluate the overall motor function, the Brooke grade and the Vignos grade were respectively used to assess the motor function status of the upper and lower limbs. Pearson correlation analysis and partial correlation analysis were used to explore the relationships between the MFM total score and domain scores and body mass index (BMI), the percentage of limb muscle strength, Brooke grade and Vignos grade. Results A total of 61 cases of children included 18 of congenital muscular dystrophy (CMD group, 29.51%), 17 of spinal muscular atrophy (SMA group, 27.87%), 15 of congenital myopathy (CM group, 24.59%), and 11 of Emery-Dreifuss muscular dystrophy (EDMD group, 18.03%). There were statistically significant differences in the percentage of limb muscle strength (F = 23.749, P = 0.000), MFM total score (F = 17.445, P = 0.000), D1 domain score (F = 18.671, P = 0.000), D2 domain score (F = 10.202, P = 0.017), Brooke grade (F = 9.313, P = 0.025) and Vignos grade (F = 22.967, P = 0.000) among the 4 groups. Correlation analysis showed that the MFM total score was positively correlated with the percentage of limb muscle strength (r = 0.528, P = 0.000), and negatively correlated with Brooke grade (r = - 0.607, P = 0.000) and Vignos grade (r = - 0.511, P = 0.000); D1 domain score was positively correlated with the percentage of limb muscle strength (r = 0.303, P = 0.020), and negatively correlated with Vignos grade (r = - 0.555, P = 0.000); D2 domain score was positively correlated with the percentage of limb muscle strength (r = 0.332, P = 0.010), and negatively correlated with Brooke grade (r = - 0.513, P = 0.000); D3 domain score was negatively correlated with Brooke grade (r = - 0.469, P = 0.000). Conclusions The characteristics of motor function in children with different types of HNMD vary greatly. The specific MFM can assist evaluating the disease severity and analyzing the differences of motor function in children with different types of HNMD. |
| format | Article |
| id | doaj-art-ecb8a7c92fca400a87ac01ce9522834d |
| institution | Kabale University |
| issn | 1672-6731 |
| language | English |
| publishDate | 2025-07-01 |
| publisher | Tianjin Huanhu Hospital |
| record_format | Article |
| series | Chinese Journal of Contemporary Neurology and Neurosurgery |
| spelling | doaj-art-ecb8a7c92fca400a87ac01ce9522834d2025-08-20T03:59:35ZengTianjin Huanhu HospitalChinese Journal of Contemporary Neurology and Neurosurgery1672-67312025-07-0125762963610.3969/j.issn.1672⁃6731.2025.07.010Characteristics of motor function in children with hereditary neuromuscular diseaseLI Wen-zhu0HUANG Zhen1FAN Yan-bin2XIONG Hui3Department of Rehabilitation Medicine, Peking University First Hospital, Beijing 100034, ChinaDepartment of Rehabilitation Medicine, Peking University First Hospital, Beijing 100034, ChinaCenter for Children's Health, Peking University First Hospital, Beijing 100034, ChinaDepartment of Neurology, Beijing Children's Hospital, Capital Medical University; National Center for Children's Health, Beijing 100032, ChinaObjective To analyze the characteristics of motor function in children with different types of hereditary neuromuscular disease (HNMD). Methods A total of 61 children with HNMD admitted to Peking University First Hospital from January 2018 to January 2024 were enrolled. Manual Muscle Testing was used to evaluate the muscle strength of the limbs, and the percentage of limb muscle strength was subsequently calculated. The Motor Function Measure (MFM) was used to evaluate the overall motor function, the Brooke grade and the Vignos grade were respectively used to assess the motor function status of the upper and lower limbs. Pearson correlation analysis and partial correlation analysis were used to explore the relationships between the MFM total score and domain scores and body mass index (BMI), the percentage of limb muscle strength, Brooke grade and Vignos grade. Results A total of 61 cases of children included 18 of congenital muscular dystrophy (CMD group, 29.51%), 17 of spinal muscular atrophy (SMA group, 27.87%), 15 of congenital myopathy (CM group, 24.59%), and 11 of Emery-Dreifuss muscular dystrophy (EDMD group, 18.03%). There were statistically significant differences in the percentage of limb muscle strength (F = 23.749, P = 0.000), MFM total score (F = 17.445, P = 0.000), D1 domain score (F = 18.671, P = 0.000), D2 domain score (F = 10.202, P = 0.017), Brooke grade (F = 9.313, P = 0.025) and Vignos grade (F = 22.967, P = 0.000) among the 4 groups. Correlation analysis showed that the MFM total score was positively correlated with the percentage of limb muscle strength (r = 0.528, P = 0.000), and negatively correlated with Brooke grade (r = - 0.607, P = 0.000) and Vignos grade (r = - 0.511, P = 0.000); D1 domain score was positively correlated with the percentage of limb muscle strength (r = 0.303, P = 0.020), and negatively correlated with Vignos grade (r = - 0.555, P = 0.000); D2 domain score was positively correlated with the percentage of limb muscle strength (r = 0.332, P = 0.010), and negatively correlated with Brooke grade (r = - 0.513, P = 0.000); D3 domain score was negatively correlated with Brooke grade (r = - 0.469, P = 0.000). Conclusions The characteristics of motor function in children with different types of HNMD vary greatly. The specific MFM can assist evaluating the disease severity and analyzing the differences of motor function in children with different types of HNMD.http://www.cjcnn.org/index.php/cjcnn/article/view/3066neuromuscular diseasesgenetic diseases， inbornchildmuscle strengthmotor activity |
| spellingShingle | LI Wen-zhu HUANG Zhen FAN Yan-bin XIONG Hui Characteristics of motor function in children with hereditary neuromuscular disease Chinese Journal of Contemporary Neurology and Neurosurgery neuromuscular diseases genetic diseases， inborn child muscle strength motor activity |
| title | Characteristics of motor function in children with hereditary neuromuscular disease |
| title_full | Characteristics of motor function in children with hereditary neuromuscular disease |
| title_fullStr | Characteristics of motor function in children with hereditary neuromuscular disease |
| title_full_unstemmed | Characteristics of motor function in children with hereditary neuromuscular disease |
| title_short | Characteristics of motor function in children with hereditary neuromuscular disease |
| title_sort | characteristics of motor function in children with hereditary neuromuscular disease |
| topic | neuromuscular diseases genetic diseases， inborn child muscle strength motor activity |
| url | http://www.cjcnn.org/index.php/cjcnn/article/view/3066 |
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