Istituto per la Ricerca e l'Innovazione Biomedica (IRIB), Consiglio Nazionale delle Ricerche (CNR), Palermo, Italy.
Unione Italiana contro la Distrofia Muscolare (UILDM), Palermo, Italy.
J Neuromuscul Dis. 2023;10(5):955-962. doi: 10.3233/JND-230055.
In Duchenne muscular dystrophy (DMD), dysphagia is a common but often overlooked symptom, which may affect quality of life (QoL). Its possible causes are progressive deterioration of muscle groups involved in swallowing function (oropharyngeal, inspiratory muscles) or impairment of autonomic function.
In adult patients with DMD, we aimed to identify predictors of swallowing-related QoL and to compare swallowing-related QoL at different ages.
Forty-eight patients aged 30.0±6.6 years were enrolled. Questionnaires were administered: the Swallowing Quality of Life questionnaire (SWAL-QOL) for swallowing-related QoL assessment, and the Compass 31 for autonomic symptoms assessment. The Brooke Upper Extremity Scale was used for upper limbs muscular function assessment. Respiratory and muscle function tests were performed, including spirometry, arterial blood gases, polysomnography, maximal inspiratory pressure (MIP), maximal expiratory pressure and sniff nasal inspiratory pressure.
An abnormal composite SWAL-QOL score (≤86) was found in 33 patients. Autonomic symptoms were mild, while a severe impairment was shown by the Brooke Upper Extremity Scale. Spirometry and muscle strength tests demonstrated severe alterations, while diurnal and nocturnal blood gases were normal, due to effective use of noninvasive ventilation. Independent predictors of the composite SWAL-QOL score were age, MIP and Compass 31. A MIP < 22 had an accuracy of 92% in predicting altered swallowing-related QoL. The composite SWAL-QOL score was worse in subjects > 30 years old than in younger patients (64.5±19.2 vs 76.6±16.3, p < 0.02), due to worse scores in items pertinent to mental and social functioning; scores in domains pertinent to the physical function were similar in both groups.
In adult DMD, swallowing-related QoL, which is altered in most patients, can be predicted by age, inspiratory muscles strength and autonomic dysfunction symptoms. While swallowing function is already altered in young patients, swallowing-related QoL can progressively worsen with advancing age due to psychological and social factors.
在杜氏肌营养不良症（DMD）中，吞咽困难是一种常见但经常被忽视的症状，可能会影响生活质量（QoL）。其可能的原因是参与吞咽功能的肌肉群（口咽、吸气肌）进行性恶化或自主功能受损。
在成年 DMD 患者中，我们旨在确定与吞咽相关的 QoL 的预测因素，并比较不同年龄的吞咽相关 QoL。
纳入 48 名年龄 30.0±6.6 岁的患者。进行问卷调查：吞咽相关 QoL 评估采用吞咽质量问卷（SWAL-QOL），自主症状评估采用 Compass 31。使用 Brooke 上肢量表评估上肢肌肉功能。进行呼吸和肌肉功能测试，包括肺功能检查、动脉血气分析、多导睡眠图、最大吸气压力（MIP）、最大呼气压力和嗅探鼻吸气压力。
33 名患者的复合 SWAL-QOL 评分（≤86）异常。自主症状较轻，但 Brooke 上肢量表显示严重受损。肺功能和肌肉力量测试显示严重改变，而日间和夜间血气正常，因为有效使用了无创通气。复合 SWAL-QOL 评分的独立预测因素为年龄、MIP 和 Compass 31。MIP<22 预测吞咽相关 QoL 改变的准确性为 92%。年龄较大（>30 岁）的患者复合 SWAL-QOL 评分较年轻患者差（64.5±19.2 对 76.6±16.3，p<0.02），原因是与精神和社会功能相关的项目评分较差；两组在与身体功能相关的领域的评分相似。
在成年 DMD 中，大多数患者的吞咽相关 QoL 改变，可由年龄、吸气肌力量和自主神经功能障碍症状来预测。尽管年轻患者的吞咽功能已经受损，但随着年龄的增长，由于心理和社会因素，吞咽相关 QoL 可能会逐渐恶化。
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