Deng Xiao, Hao Ying, Xiao Bin, Tan Eng-King, Lo Yew-Long
Department of Neurology, National Neuroscience Institute, Singapore General Hospital, Singapore 169608, Singapore.
Health Service Research Unit, Division of Medicine, Singapore General Hospital, Singapore 169608, Singapore.
J Clin Neurosci. 2017 May;39:137-141. doi: 10.1016/j.jocn.2016.12.018. Epub 2017 Jan 12.
Motor neuron disease (MND) is a devastating degenerative disorder. Amyotrophic Lateral Sclerosis (ALS) is the most common and severe form of MND. Respiratory failure arising from ventilator musculature atrophy is the most common cause of death for ALS patients. Exploring the factors correlated with respiratory failure can contribute to disease management.
To characterize the clinical features of MND and determine the factors that may affect respiratory failure of MND patients.
The case records of all MND patients seen in Singapore General Hospital (SGH) between January 2004 and December 2014 were examined. Demographic, clinical information were collected by reviewing case records. Mortality data, if not available from records, were obtained via phone call interview of family members. Demographic data and clinical treatments were compared between Respiratory support group and Non-respiratory support group.
There were 73 patients included in our study. 49 (67.1%) patients died during follow-up. The mean age of onset was 58±11.1years. With regard to treatment, 63% needed feeding support, and 42.5% required ventilation aid. The median overall survival was 36months from symptom onset. Chi-square tests showed there was significantly higher percentage of respiratory support needed in Chinese than in other races (P=0.016). Compared with non-feeding support patients, patients with feeding support were more likely to require assisted ventilation (P=0.001).
We report for the first time that the need of feeding support is significantly associated with assisted ventilation. Chinese MND patients may be more inclined to require respiratory support.
运动神经元病（MND）是一种毁灭性的退行性疾病。肌萎缩侧索硬化症（ALS）是MND最常见且最严重的形式。呼吸肌萎缩导致的呼吸衰竭是ALS患者最常见的死亡原因。探索与呼吸衰竭相关的因素有助于疾病管理。
描述MND的临床特征，并确定可能影响MND患者呼吸衰竭的因素。
检查了2004年1月至2014年12月期间在新加坡总医院（SGH）就诊的所有MND患者的病例记录。通过查阅病例记录收集人口统计学和临床信息。如果记录中没有死亡率数据，则通过电话采访家庭成员获得。比较了呼吸支持组和非呼吸支持组的人口统计学数据和临床治疗情况。
我们的研究纳入了73例患者。49例（67.1%）患者在随访期间死亡。平均发病年龄为58±11.1岁。在治疗方面，63%的患者需要营养支持，42.5%的患者需要通气辅助。从症状出现开始计算，总体生存期中位数为36个月。卡方检验显示，华人患者需要呼吸支持的比例显著高于其他种族（P = 0.016）。与不需要营养支持的患者相比，需要营养支持的患者更有可能需要辅助通气（P = 0.001）。
我们首次报告，营养支持需求与辅助通气显著相关。华人MND患者可能更倾向于需要呼吸支持。
