Department of Pulmonary Diseases and Home Mechanical Ventilation, Maastricht University Medical Centre+, Maastricht, The Netherlands.
School for Mental Health and Neuroscience (MHeNS), Maastricht University, Maastricht, The Netherlands.
J Neuromuscul Dis. 2024;11(5):1123-1130. doi: 10.3233/JND-240081.
Chronic respiratory failure often occurs in myotonic dystrophy type 1 (DM1) and can be treated with noninvasive home mechanical ventilation (HMV). Treatment adherence with HMV is often suboptimal in patients with DM1, but the reasons for that are not well understood.
The aim of this exploratory study was to gain insight in the prevalence of mild cognitive impairment, affective symptoms, and apathy and to investigate their role in HMV treatment adherence in DM1.
The Montreal Cognitive Assessment (MoCA), the Hospital Anxiety and Depression Scale (HADS), and the Apathy Evaluation Scale (AES) were used to assess cognition, affective symptoms, and apathy in DM1 patients that use HMV. Patients with low treatment adherence (average daily use HMV <5 h or <80% of the days) were compared with patients with high treatment adherence (average daily use of HMV≥5 h and ≥80% of the days).
Sixty patients were included. Abnormal scores were found in 40% of the total group for the MoCA, in 72-77% for the AES, and in 18% for HADS depression. There was no difference between the high treatment adherence group (n = 39) and the low treatment adherence group (n = 21) for the MoCA, AES, and HADS depression. The HADS anxiety was abnormal in 30% of the total group, and was significantly higher in the low treatment adherence group (p = 0.012). Logistic regression analysis revealed that a higher age and a higher BMI were associated with a greater chance of high treatment adherence.
This exploratory study showed that cognitive impairment and apathy are frequently present in DM1 patients that use HMV, but they are not associated with treatment adherence. Feelings of anxiety were associated with low treatment adherence. Higher age and higher BMI were associated with high treatment adherence with HMV.
肌强直性营养不良 1 型（DM1）常发生慢性呼吸衰竭，可采用无创家庭机械通气（HMV）治疗。但 DM1 患者对 HMV 的治疗依从性往往不理想，但其原因尚不清楚。
本探索性研究旨在了解轻度认知障碍、情感症状和淡漠的患病率，并探讨其在 DM1 患者对 HMV 治疗依从性中的作用。
采用蒙特利尔认知评估量表（MoCA）、医院焦虑抑郁量表（HADS）和淡漠评估量表（AES）评估使用 HMV 的 DM1 患者的认知、情感症状和淡漠。将低治疗依从性（平均每天使用 HMV＜5 小时或＜80%的天数）患者与高治疗依从性（平均每天使用 HMV≥5 小时且≥80%的天数）患者进行比较。
共纳入 60 例患者。总组中 MoCA 异常评分占 40%，AES 占 72%-77%，HADS 抑郁占 18%。高治疗依从组（n=39）与低治疗依从组（n=21）之间 MoCA、AES 和 HADS 抑郁评分无差异。总组中 30%的 HADS 焦虑异常，低治疗依从组显著更高（p=0.012）。Logistic 回归分析显示，年龄较大和 BMI 较高与较高的治疗依从性几率相关。
本探索性研究表明，认知障碍和淡漠在使用 HMV 的 DM1 患者中较为常见，但与治疗依从性无关。焦虑感与低治疗依从性相关。年龄较大和 BMI 较高与 HMV 的高治疗依从性相关。
