Centre for Neuromuscular and Neurological Disorders, Perron Institute for Neurological and Translational Science, UWA, Queen Elizabeth II Medical Centre, Perth, Western Australia, Australia.
Institute for Immunology and Infectious Diseases, Murdoch University, Perth, Western Australia, Australia.
J Neurol Sci. 2018 May 15;388:12-18. doi: 10.1016/j.jns.2018.02.036. Epub 2018 Feb 22.
Benign multiple sclerosis (BMS) is a controversial term that has been used for MS patients with minimal disability decades after disease onset. Herein, we evaluated disease status after 20 years in a Western Australian cohort defined as BMS based on an Expanded Disability Status Scale (EDSS) score ≤ 3.0 at 10 years from onset.
MS patients with an EDSS score ≤ 3.0 at 10 years from onset and minimum of 20 years follow up were included in the study. The 20-year EDSS score was considered the primary outcome. Associations with demographic and clinical characteristics and HLA-DRB1 genotype were investigated.
Among 120 patients with a benign course at 10 years, 78 (65%) remained benign at the 20-year follow up, but patients with an EDSS ≥ 2.5 were more likely to go on to develop more severe disability in the next decade. When considering factors associated with an increase in EDSS score ≤ 1 from 10 to 20 years, indicating limited progression, apart from the EDSS score at 10 years, poly-symptomatic presentation (p = 0.004) and cerebellar/brainstem mono-symptomatic presentation (p = 0.016) were independently associated with more rapid progression compared with other mono-symptomatic presentations. Carriage of the high risk HLA-DRB1*1501 allele was marginally associated with slower progression.
In this geographically isolated MS cohort of predominantly Anglo-Celtic origin clinical progression in the benign MS group was similar to that in other published series from Western countries. These results are in keeping with the view that patients labeled as benign MS are part of a heterogeneous continuum of disease progression and do not possess unique clinical characteristics. Possible genetic determinants of a benign course warrant further investigation.
良性多发性硬化症（BMS）是一个有争议的术语，用于描述疾病发病数十年后仅有轻微残疾的 MS 患者。在此，我们评估了基于发病 10 年后扩展残疾状态量表（EDSS）评分≤3.0 定义为 BMS 的西澳队列患者 20 年后的疾病状况。
纳入 EDSS 评分≤3.0 的 MS 患者，在发病 10 年后随访至少 20 年。20 年 EDSS 评分是主要结局。研究了与人口统计学和临床特征及 HLA-DRB1 基因型的关联。
在 120 名发病 10 年时具有良性病程的患者中，78 名（65%）在 20 年随访时仍保持良性，但 EDSS≥2.5 的患者在接下来的十年中更有可能发展为更严重的残疾。考虑与 EDSS 评分从 10 年到 20 年增加≤1 分相关的因素，表明进展有限，除了 10 年时的 EDSS 评分外，多症状表现（p=0.004）和小脑/脑干单症状表现（p=0.016）与比其他单症状表现相比，与更快速的进展独立相关。携带高危 HLA-DRB1*1501 等位基因与进展缓慢呈边缘相关。
在这个以盎格鲁-撒克逊血统为主的地理位置孤立的 MS 队列中，良性 MS 组的临床进展与其他西方国家发表的系列研究相似。这些结果与认为被标记为良性 MS 的患者是疾病进展异质性连续体的一部分，而不具有独特的临床特征的观点一致。良性病程的可能遗传决定因素值得进一步研究。
