Printza Athanasia, Goutsikas Charalambos, Triaridis Stefanos, Kyrgidis Athanasios, Haidopoulou Katerina, Constantinidis Jannis, Pavlou Evagelos
1st Otolaryngology Department, Medical Dept, School of Health Sciences, Aristotle University of Thessaloniki, 54124, Greece.
2nd Paediatric Department, Center for Neuromuscular Diseases, Medical Dept, School of Health Sciences, Aristotle University of Thessaloniki, Greece.
Int J Pediatr Otorhinolaryngol. 2019 Feb;117:198-203. doi: 10.1016/j.ijporl.2018.12.005. Epub 2018 Dec 5.
Dysphagia in progressive muscle diseases is primarily due to muscle weakness. Objective of our study is to investigate the prevalence and phenotypes of dysphagia in patients with childhood onset muscular dystrophy (MD) with the use of a validated questionnaire, the measurement of tongue strength and Flexible Endoscopic Evaluation of Swallowing (FEES).
Prospective observational longitudinal study of dysphagia in a cohort of 58 patients attending the Pediatric Department Center for Neuromuscular Diseases. Control participants were 56 age and sex matched healthy volunteers. Dysphagia was evaluated with the Eating Assessment Tool-10 (EAT-10), and the measurement of Maximal Isometric Tongue Pressure (MITP) and tongue endurance (Iowa Oral Performance Instrument-IOPI). Dysphagic patients were submitted to FEES. Recorded data included demographic and anthropometric characteristics, type of MD, feeding status, and spirometry.
Our patients' cohort consisted of 41 children, 11 adolescents, and 6 adults. Based on EAT-10, 20.7% of the patients were dysphagic: 14.63% of children, 27.3% of adolescents and 50% of adults. The main complain was solid food dysphagia. Spirometry parameters mean values for children and adolescent patients corresponded to lower than the fifth percentile. Means of FVC and FEV1 expressed as % predicted for adult patients were 27.8 (SD:25.05) and 28.8 (SD:28.44) respectively. Reduced tongue strength was measured to children aged 9-10, adolescent and adult MD patients. The main FEES findings were pharyngeal residue, spillage of food before the swallow, and supraglottal penetration.
This is the first study to use a validated questionnaire to evaluate dysphagia in childhood onset MD and report dyphagia prevalence at different patients' age. This is the first study reporting MITP in children and adults with generalised MD. Tongue pressures are reduced well before clinical signs of dysphagia are present.
Screening of potentially dysphagic MD patients can be based on a validated questionnaire. Patients with an EAT-10 score suggestive of dysphagia at regular follow-up can have the MITP measured and in the case of reduced values a thorough dysphagia evaluation with FEES is indicated.
进行性肌肉疾病中的吞咽困难主要是由于肌肉无力。我们研究的目的是通过使用经过验证的问卷、测量舌肌力量和吞咽功能的纤维内镜评估（FEES），来调查儿童期起病的肌营养不良（MD）患者吞咽困难的患病率和表型。
对58名就诊于小儿神经肌肉疾病中心的患者进行吞咽困难的前瞻性观察性纵向研究。对照参与者为56名年龄和性别匹配的健康志愿者。使用进食评估工具-10（EAT-10）、测量最大等长舌压（MITP）和舌耐力（爱荷华口腔功能仪器-IOPI）来评估吞咽困难。吞咽困难患者接受FEES检查。记录的数据包括人口统计学和人体测量学特征、MD类型、喂养状况和肺活量测定。
我们的患者队列包括41名儿童、11名青少年和6名成年人。根据EAT-10，20.7%的患者存在吞咽困难：14.63%的儿童、27.3%的青少年和50%的成年人。主要主诉为固体食物吞咽困难。儿童和青少年患者的肺活量测定参数平均值低于第五百分位数。成年患者的FVC和FEV1以预测值的百分比表示，分别为27.8（标准差：25.05）和28.8（标准差：28.44）。测量发现9至10岁儿童、青少年和成年MD患者的舌肌力量下降。FEES的主要发现为咽部残留、吞咽前食物溢出和声门上穿透。
这是第一项使用经过验证的问卷来评估儿童期起病的MD患者吞咽困难并报告不同患者年龄吞咽困难患病率的研究。这是第一项报告全身性MD儿童和成人MITP的研究。在出现吞咽困难临床体征之前，舌压就已降低。
对潜在吞咽困难的MD患者进行筛查可基于经过验证的问卷。在定期随访中EAT-10评分提示吞咽困难的患者可测量MITP，若值降低，则需用FEES进行全面的吞咽困难评估。
