Predictors of participation restriction over a 9-year period in adults with myotonic dystrophy type 1

For slowly progressive neuromuscular disease, prognostic approach and long-term monitoring of participation is a crucial part of rehabilitation services. To improve the prognostic approach, professionals must identify individuals at risk of having higher participation restriction. This study aimed to identify personal and environmental predictors of participation restriction over nine years in adults with myotonic dystrophy type 1 (DM1). A secondary analysis of a longitudinal design comparing baseline with a follow-up nine years later was used with a multidimensional assessment of participation and personal and environmental factors. Based on theoretical models, multiple linear regressions were used. One hundred and fourteen adults with DM1 were included in the study (63.2% women; 78.9% adult onset; mean (SD) age of 43.5 (10.4) years). When age, sex, phenotype, and education were controlled for, participation restriction was predicted by a longer time to stand and walk, lower grip strength, higher body mass index, absence of perceived impact of myotonia in daily living, use of adapted transportation from community services, and perception of obstacle in physical environment (<i>p</i> &lt; 0.001, adjusted <i>R</i>² = 0.50). The majority of predictors of participation restriction can be advantageously modified by rehabilitation and environmental changes, such as politics targeting community services provision or physical environment and services accessibility.Implications for rehabilitationPredictors could better inform rehabilitation professional to recognize individuals at risk of higher participation restriction over time and to target specific interventions based on a prognostic approach.Rehabilitation professionals could inform the people living with myotonic dystrophy type 1 and their relatives of the multifactorial nature of occurrence of participation restriction to diminish the “fatality” associated with a genetic progressive disorder.Predictors allow professionals to assess and intervene in the management of specific factors depending on the rehabilitation goal.Identifying individual with myotonic dystrophy with higher risk of participation restriction could help implement a long-term community based rehabilitation intervention plan targeting both personal and environmental factors. Predictors could better inform rehabilitation professional to recognize individuals at risk of higher participation restriction over time and to target specific interventions based on a prognostic approach. Rehabilitation professionals could inform the people living with myotonic dystrophy type 1 and their relatives of the multifactorial nature of occurrence of participation restriction to diminish the “fatality” associated with a genetic progressive disorder. Predictors allow professionals to assess and intervene in the management of specific factors depending on the rehabilitation goal. Identifying individual with myotonic dystrophy with higher risk of participation restriction could help implement a long-term community based rehabilitation intervention plan targeting both personal and environmental factors.

对于慢性进展性神经肌肉疾病而言，预后评估方案与参与功能的长期监测是康复服务的核心组成部分。为优化预后评估流程，临床康复专业人员需识别存在更高参与限制风险的个体。
本研究旨在识别1型强直性肌营养不良（myotonic dystrophy type 1, DM1）成人患者9年内参与限制的个人与环境预测因素。本研究采用纵向研究设计的二次分析方法，对比基线评估与9年后的随访结果，并对参与功能、个人及环境因素开展多维度评估。基于相关理论模型，本研究采用多元线性回归分析。
本研究共纳入114名1型强直性肌营养不良成人患者，其中女性占比63.2%，成人起病者占78.9%，平均年龄为43.5岁（标准差10.4岁）。在控制年龄、性别、表型与教育水平的前提下，以下因素可预测参与限制：起立与行走耗时更长、握力更低、身体质量指数更高、未感知肌强直对日常生活的影响、使用社区服务提供的适配交通工具，以及感知到物理环境存在障碍（p<0.001，校正后R²=0.50）。
多数参与限制的预测因素可通过康复干预与环境改造得到有效改善，例如制定针对社区服务供给、物理环境与服务可及性的相关政策。

康复实践启示
预测因素可帮助康复专业人员更好地识别随时间推移存在更高参与限制风险的个体，并基于预后评估方案制定针对性干预措施。康复专业人员可向1型强直性肌营养不良患者及其家属告知参与限制发生的多因素本质，以减轻该遗传性进展性疾病相关的不良结局影响。预测因素可帮助专业人员根据康复目标评估并干预特定影响因素。识别存在较高参与限制风险的1型强直性肌营养不良患者，有助于制定兼顾个人与环境因素的长期社区康复干预计划。
预测因素可帮助康复专业人员更好地识别随时间推移存在更高参与限制风险的个体，并基于预后评估方案制定针对性干预措施。康复专业人员可向1型强直性肌营养不良患者及其家属告知参与限制发生的多因素本质，以减轻该遗传性进展性疾病相关的不良结局影响。预测因素可帮助专业人员根据康复目标评估并干预特定影响因素。识别存在较高参与限制风险的1型强直性肌营养不良患者，有助于制定兼顾个人与环境因素的长期社区康复干预计划。