Tintoré Mar
Unitat de Neuroimmunologia Clinica (UNIC), Edif. Escola d'Infermeria Planta 2, Hospital Universitari Vall d'Hebron, Pg Vall d'Hebron 119-129, 08035, Barcelona, Spain.
J Neurol. 2008 Mar;255 Suppl 1:37-43. doi: 10.1007/s00415-008-1006-4.
The McDonald diagnostic criteria have allowed the formal diagnosis of multiple sclerosis in patients presenting with clinically isolated syndromes to be brought forward. Evidence from research suggests that many patients with clinically isolated syndromes or early multiple sclerosis should be treated with disease-modifying drugs at an early stage, since disease experience during the first few years are likely to have significant impact on the long-term evolution of the disease. Histopathological studies have demonstrated the presence of axonal transection in patients with less than five years of disease duration, and especially during the first twelve months. Natural history studies have shown that the number of relapses occurring during the first few years of the disease is related to the time to accrued disability. Moreover, longitudinal studies on patients with clinically isolated syndromes have shown that the presence of even a very small number of baseline MRI lesions is associated with an increased risk of developing clinically definite multiple sclerosis and, more importantly, that the increase in volume of the lesions seen in the first five years correlates with the degree of disability in the longer term. For example, long-term follow-up of a large cohort of patients presenting with a clinically isolated syndrome in Barcelona has shown that the number of Barkhof criteria fulfilled at baseline was correlated with the risk of relapse, EDSS disability scores at five years and the risk of reaching given EDSS disability thresholds. Three randomised clinical trials in patients with a clinically isolated syndrome and abnormal brain MRI have shown significant benefit of initiating early therapy with beta-interferons, and a similar study is underway with glatiramer acetate. It is concluded that choosing the right time to introduce treatment is critically important for outcome and the earlier treatment is initiated, the better the outcome.
麦克唐纳诊断标准使得对表现为临床孤立综合征的患者进行多发性硬化症的正式诊断得以提前。研究证据表明，许多患有临床孤立综合征或早期多发性硬化症的患者应在疾病早期就接受疾病修正药物治疗，因为最初几年的疾病经历可能对疾病的长期发展产生重大影响。组织病理学研究表明，病程不足五年的患者，尤其是在最初十二个月内，存在轴突横断现象。自然史研究显示，疾病最初几年的复发次数与累积残疾时间相关。此外，对临床孤立综合征患者的纵向研究表明，即使仅有极少量的基线磁共振成像（MRI）病灶，也会增加发展为临床确诊多发性硬化症的风险，更重要的是，最初五年内病灶体积的增加与长期残疾程度相关。例如，对巴塞罗那一大群表现为临床孤立综合征的患者进行长期随访发现，基线时满足巴克霍夫标准的数量与复发风险、五年时的扩展残疾状态量表（EDSS）残疾评分以及达到特定EDSS残疾阈值的风险相关。三项针对临床孤立综合征且脑部MRI异常患者的随机临床试验表明，早期使用β-干扰素治疗有显著益处，一项关于醋酸格拉替雷的类似研究正在进行中。结论是，选择合适的治疗时机对治疗结果至关重要，治疗开始得越早，结果越好。
