Yong Loo Lin School of Medicine, National University of Singapore, Singapore.
Department of Neurology, Division of Nursing, National Neuroscience Institute, Tan Tock Seng Hospital, Singapore.
Singapore Med J. 2022 Jul;63(7):371-375. doi: 10.11622/smedj.2021001. Epub 2021 Jan 21.
A clinical-based staging model would guide physicians in the prompt management of the evolving symptoms and functional needs of patients with amyotrophic lateral sclerosis (ALS).
We aimed to delineate the clinical trajectory of ALS in Singapore and test the degree of congruity of King's College staging for ALS (King's staging) among Singapore patients. In this retrospective cohort study, clinical milestones used for staging were identical to King's staging: stage 1 corresponded to symptom onset; stage 2A corresponded to diagnosis; stage 2B corresponded to two central nervous system (CNS) regions; stage 3B corresponded to three CNS regions; stage 4A corresponded to requirement of supportive enteric feeding; and stage 4B corresponded to requirement of non-invasive ventilation, of which bulbar, diaphragmatic, upper and lower limb pyramidal involvements each constituted one CNS region. Standardised timings from disease onset (0) to death (1) among Singapore patients with ALS were measured.
46 patients with ALS were reviewed. Results were largely congruous with King's staging. Results for patients with limb-onset ALS were: diagnosis (0.35); two CNS region involvement (0.42); three CNS region involvement (0.63); diaphragmatic involvement (0.81); and bulbar involvement (0.73). Results for patients with bulbar-onset ALS were: diagnosis (0.14); two CNS region involvement (0.28); three CNS region involvement (0.42); diaphragmatic involvement (0.62); and bulbar involvement (0.67).
King's staging can be used to model ALS trajectory in Singapore due to the large degree of congruity seen. Easily remembered and accessible knowledge of ALS staging will allow prompt management of the evolving needs of patients with ALS.
基于临床的分期模型将指导医生及时处理肌萎缩侧索硬化症（ALS）患者不断变化的症状和功能需求。
我们旨在描绘新加坡 ALS 的临床轨迹，并测试 King's 大学 ALS 分期（King's 分期）在新加坡患者中的一致性程度。在这项回顾性队列研究中，用于分期的临床里程碑与 King's 分期完全相同：第 1 阶段对应症状发作；第 2A 阶段对应诊断；第 2B 阶段对应两个中枢神经系统（CNS）区域；第 3B 阶段对应三个 CNS 区域；第 4A 阶段对应需要支持性肠内喂养；第 4B 阶段对应需要非侵入性通气，其中延髓、膈肌、上下肢锥体受累各构成一个 CNS 区域。测量新加坡 ALS 患者从疾病发作（0）到死亡（1）的标准化时间。
共回顾了 46 例 ALS 患者。结果与 King's 分期基本一致。对于肢体起病的 ALS 患者：诊断（0.35）；两个 CNS 区域受累（0.42）；三个 CNS 区域受累（0.63）；膈肌受累（0.81）；延髓受累（0.73）。对于延髓起病的 ALS 患者：诊断（0.14）；两个 CNS 区域受累（0.28）；三个 CNS 区域受累（0.42）；膈肌受累（0.62）；延髓受累（0.67）。
由于一致性程度很高，King's 分期可用于模拟新加坡 ALS 的轨迹。易于记忆和获取的 ALS 分期知识将允许及时处理 ALS 患者不断变化的需求。
