van den Engel-Hoek Lenie, de Swart Bert J M, Erasmus Corrie E, de Groot Imelda J M
Department of Rehabilitation, Radboud University Nijmegen Medical Centre, Nijmegen, The Netherlands.
J Child Neurol. 2008 Aug;23(8):919-21. doi: 10.1177/0883073808315418. Epub 2008 Apr 10.
A child with spinal muscular atrophy type 2 was referred for evaluation of eating and swallowing problems. The dysphagia evaluation demonstrated coughing during eating and drinking and occasionally stertorous when eating solid food. The videofluoroscopic swallow study showed a late upper esophageal sphincter opening with hypopharyngeal residue, more with solid food than with thin liquid. His lumbar lordosis associated with anterior tilted pelvis and his problems with head balance due to weak neck musculature caused compensatory behavior like a retracted neck and mandible. This position negatively influences the opening of the upper esophageal sphincter. This case supported the idea that dysphagia in spinal muscular atrophy type 2 is caused by both a bulbar component as well as a treatable posture component.
一名患有2型脊髓性肌萎缩症的儿童因进食和吞咽问题前来接受评估。吞咽困难评估显示，该患儿在进食和饮水时会咳嗽，吃固体食物时偶尔会打鼾。电视荧光吞咽造影研究显示，食管上括约肌开口延迟，下咽有残留物，固体食物比稀液体时更多。他的腰椎前凸伴有骨盆前倾，由于颈部肌肉无力导致头部平衡问题，从而引发了如缩颈和下颌后缩等代偿行为。这种姿势对上食管括约肌的开口产生负面影响。该病例支持了这样一种观点，即2型脊髓性肌萎缩症的吞咽困难是由延髓因素以及可治疗的姿势因素共同引起的。
