Department of Rehabilitation Sciences and Physiotherapy, Faculty of Medicine and Health Science, University of Antwerp, Antwerp, Belgium.
Department of Pediatric Neurology, Antwerp University Hospital, University of Antwerp, Edegem, Belgium.
Dev Med Child Neurol. 2019 Aug;61(8):950-956. doi: 10.1111/dmcn.14147. Epub 2019 Jan 15.
The aim of this study is to describe the course of motor development in children with Dravet syndrome.
Forty-three participants (21 males, 22 females; mean age at last assessment 53.89mo±42.50mo) met the inclusion criteria of having a confirmed diagnosis of Dravet syndrome and presence of data on motor development. All data between 1985 and 2018 were derived retrospectively from their medical records. Gross motor milestones and motor age equivalents were used to describe motor development. Standardized neurodevelopmental assessment and the Bayley Scales of Infant Development defined the overall motor development. Peabody Developmental Motor Scales, Bruininks-Oseretsky Test of Motor Proficiency, and the Beery-Buktenica Developmental Test of Visual-Motor Integration were used to describe development in specific motor domains.
Children with Dravet syndrome showed a delay in both sitting (seven out of 14) and walking independently (11 out of 25). Overall motor age equivalents revealed a delay in 29 out of 38 assessments (age 9-115mo). All assessments of children older than 2 years (16 out of 16) showed a delay. Gross motor delay was present in seven out of seven and fine motor delay in 10 out of 13 assessments (age 19-167mo).
Motor development is delayed in the majority of children with Dravet syndrome older than 2 years and increases with age.
A delay in motor development is present in most children with Dravet syndrome older than 2 years. Large diversity in early gross motor milestones confirms heterogeneity in Dravet syndrome.
本研究旨在描述 Dravet 综合征患儿的运动发育过程。
43 名参与者（男 21 名，女 22 名；末次评估时的平均年龄为 53.89±42.50 个月）符合 Dravet 综合征的确诊标准，并存在运动发育数据。所有数据均为回顾性地从他们的病历中获得，时间范围为 1985 年至 2018 年。粗大运动里程碑和运动年龄当量用于描述运动发育。标准化神经发育评估和贝利婴幼儿发育量表定义了整体运动发育。使用 Peabody 发育运动量表、Bruininks-Oseretsky 运动能力测试和 Beery-Buktenica 视觉运动整合发育测试来描述特定运动领域的发育。
Dravet 综合征患儿存在独坐（14 例中有 7 例）和独立行走（25 例中有 11 例）延迟。总体运动年龄当量显示 38 次评估中有 29 次（年龄 9-115 个月）延迟。所有年龄大于 2 岁的儿童评估（16 例中的 16 例）均显示延迟。粗大运动延迟见于 7 例（7 例），精细运动延迟见于 10 例（19-167 个月）。
大多数年龄大于 2 岁的 Dravet 综合征患儿的运动发育均延迟，且随年龄增长而增加。
大多数年龄大于 2 岁的 Dravet 综合征患儿的运动发育延迟。早期粗大运动里程碑的多样性较大，证实了 Dravet 综合征的异质性。
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