van den Engel-Hoek L, Erasmus C E, van Bruggen H W, de Swart B J M, Sie L T L, Steenks M H, de Groot I J M
Department of Speech Language Therapy, Children Hospital of the Radboud University Nijmegen Medical Centre, Nijmegen, The Netherlands.
Neurology. 2009 Nov 24;73(21):1787-91. doi: 10.1212/WNL.0b013e3181c34aa6.
In patients with spinal muscular atrophy (SMA) type II, feeding problems and dysphagia are common, but the underlying mechanisms of these problems are not well defined. This case control study was designed to determine the underlying mechanisms of dysphagia in SMA type II.
Six children with SMA type II and 6 healthy matched controls between 6.4 and 13.4 years of age were investigated during swallowing liquid and solid food in 2 different postures using surface EMG (sEMG) of the submental muscle group (SMG) and a video fluoroscopic swallow study (VFSS).
The VFSS showed postswallow residue of solid food in the vallecula and above the upper esophageal sphincter (UES), which can be responsible for indirect aspiration. Better results in swallowing were achieved in a more forward head position. These findings were supported by the sEMG measurements of the SMG during swallowing.
Dysphagia in spinal muscular atrophy type II is due to a neurologic dysfunction (lower motor neuron problems from the cranial nerves in the brainstem) influencing the muscle force and efficiency of movement of the tongue and the submental muscle group in combination with a biomechanical component (compensatory head posture). The results suggest an integrated treatment with an adapted posture during meals and the advice of drinking water after meals to prevent aspiration pneumonias.
在Ⅱ型脊髓性肌萎缩症（SMA）患者中，喂养问题和吞咽困难很常见，但这些问题的潜在机制尚未明确。本病例对照研究旨在确定Ⅱ型SMA吞咽困难的潜在机制。
对6名Ⅱ型SMA儿童和6名年龄在6.4至13.4岁之间的健康匹配对照者，在两种不同姿势下吞咽液体和固体食物时，使用颏下肌群（SMG）的表面肌电图（sEMG）和视频荧光吞咽造影检查（VFSS）进行研究。
VFSS显示，会厌谷和食管上括约肌（UES）上方存在固体食物吞咽后残留，这可能导致间接误吸。头部位置更靠前时吞咽效果更好。吞咽过程中SMG的sEMG测量结果支持了这些发现。
Ⅱ型脊髓性肌萎缩症的吞咽困难是由于神经功能障碍（来自脑干中颅神经的下运动神经元问题）影响了舌部和颏下肌群的肌肉力量及运动效率，同时伴有生物力学因素（代偿性头部姿势）。研究结果表明，应采取综合治疗措施，包括进餐时采用合适姿势以及建议饭后饮水以预防吸入性肺炎。
