Radboud University Medical Center, Donders Institute for Brain, Cognition and Behaviour, Department of Rehabilitation, Nijmegen, Netherlands.
Radboud University Medical Center, Donders Institute for Brain, Cognition and Behaviour, Department of Neurology, Nijmegen, Netherlands.
J Neuromuscul Dis. 2020;7(4):483-494. doi: 10.3233/JAD-200511.
Oculopharyngeal muscular dystrophy (OPMD) is a late onset progressive neuromuscular disorder. Although dysphagia is a pivotal sign in OPMD it is still not completely understood.
The aim of this study was to systematically investigate oropharyngeal functioning in a large OPMD population.
Forty-eight genetically confirmed OPMD patients completed questionnaires, performed clinical tests on swallowing, chewing, speaking, tongue strength and bite force, and underwent videofluoroscopy of swallowing. Descriptive statistics was used for all outcomes and logistic regression to investigate predictors of abnormal swallowing.
Eighty-two percent reported difficulties with swallowing, 27% with chewing and 67% with speaking. Patients performed significantly worse on all oropharyngeal tests compared to age-matched controls except for bite force. Also asymptomatic carriers performed worse than controls: on chewing time, swallowing speed and articulation rate. During videofluoroscopy, all patients (except one asymptomatic) had abnormal residue and 19% aspirated. Independent predictors of abnormal residue were reduced swallowing capacity for thin liquids (OR 10 mL = 0.93; 20 mL = 0.95) and reduced tongue strength for thick liquids (OR 10 mL = 0.95); 20 mL = 0.90). Aspiration of thin liquids was predicted by disease duration (OR = 1.11) and post-swallow residue with 20 mL (OR = 4.03).
Next to pharyngeal dysphagia, chewing and speaking are also frequently affected in OPMD patients, even in asymptomatic carriers. Residue after swallowing is a very early sign, while aspiration is a later sign in OPMD. For clinical follow-up monitoring of subjective complaints, swallowing capacity and tongue strength seems relevant.
眼咽型肌营养不良症（OPMD）是一种迟发性进行性神经肌肉疾病。尽管吞咽困难是 OPMD 的一个重要特征，但它仍未被完全理解。
本研究旨在系统研究大量 OPMD 人群的口咽功能。
48 名经基因证实的 OPMD 患者完成了问卷调查，进行了吞咽、咀嚼、说话、舌力和咬合力的临床测试，并进行了吞咽录像检查。所有结果均采用描述性统计，逻辑回归分析用于研究异常吞咽的预测因素。
82%的患者报告吞咽困难，27%的患者报告咀嚼困难，67%的患者报告说话困难。与年龄匹配的对照组相比，患者在所有口咽测试中表现明显更差，除咬合力外。无症状携带者的咀嚼时间、吞咽速度和发音率也比对照组差。在录像检查中，所有患者（除一名无症状携带者外）均有异常残留，19%的患者有吸入。异常残留的独立预测因素为稀薄液吞咽量减少（OR 10 mL = 0.93；20 mL = 0.95）和浓液舌力减弱（OR 10 mL = 0.95；20 mL = 0.90）。稀薄液吸入的预测因素为疾病持续时间（OR = 1.11）和 20 毫升后吞咽残留（OR = 4.03）。
除咽肌吞咽困难外，咀嚼和说话在 OPMD 患者中也经常受到影响，即使在无症状携带者中也是如此。吞咽后残留是 OPMD 的一个早期特征，而吸入是 OPMD 的一个晚期特征。对于临床随访监测，主观症状、吞咽能力和舌力似乎是相关的。
