Metabolic dysfunction is an important factor in the pathogenesis of motor neuron disease, but its prevalence and association with survival in this disorder is unknown. We hypothesized that patients with motor neuron disease would show a higher prevalence of metabolic syndrome compared to the general New Zealand population, and that metabolic syndrome would be associated with worsened survival. We undertook a retrospective analysis in 109 motor neuron disease patients diagnosed and treated at Waikato Hospital from 2013 to 2020. Demographic, clinical, and laboratory data were collected. Survival was defined as the date of initial symptom onset to the date of death. Of 104 eligible patients, 34 patients (33%) had metabolic syndrome (33% of Europeans, 46% of Māori). Mean survival in motor neuron disease patients with metabolic syndrome was significantly reduced compared to patients without metabolic syndrome (38 vs. 61 months, P = 0.044), with a 5-year survival rate of 21% for the former and 38% for the latter (P = 0.012). Compared with the general New Zealand population, metabolic syndrome is highly prevalent amongst motor neuron disease patients in the Waikato region and it is associated with worsened survival. Metabolic dysfunction may be a key factor underlying the pathogenesis of motor neuron disease.

代谢功能障碍是运动神经元疾病发病机制的一个重要因素，但其患病率及其与该疾病生存的关系尚不清楚。我们假设，与新西兰普通人群相比，运动神经元疾病患者的代谢综合征患病率更高，并且代谢综合征与生存率恶化相关。我们对 2013 年至 2020 年在怀卡托医院诊断和治疗的 109 名运动神经元疾病患者进行了回顾性分析。收集了人口统计学、临床和实验室数据。生存期定义为初始症状出现日期至死亡日期。在 104 名符合条件的患者中，34 名患者 (33%) 患有代谢综合征（欧洲人为 33%，毛利人为 46%）。与无代谢综合征的患者相比，患有代谢综合征的运动神经元疾病患者的平均生存期显着降低（38 个月与 61 个月，P  = 0.044），前者的 5 年生存率为 21%，后者的 5 年生存率为 38% （P  = 0.012）。与新西兰普通人群相比，代谢综合征在怀卡托地区的运动神经元疾病患者中非常普遍，并且与生存率恶化有关。代谢功能障碍可能是运动神经元疾病发病机制的关键因素。