Ebers George C
Department of Clinical Neurology, Radcliffe Infirmary, University of Oxford, Woodstock Road, Oxford OX2 6HE, UK.
Mult Scler. 2004 Jun;10 Suppl 1:S8-13; discussion S13-5. doi: 10.1191/1352458504ms1025oa.
The relationship of primary progressive multiple sclerosis (PPMS) to relapsing-remitting MS (RRMS) and secondary progressive MS (SPMS) remains unclear. Natural history data from a population-based cohort of patients with PPMS followed for approximately 25 years demonstrate remarkable similarities in the progressive phases of PPMS and SPMS. Immunogenetic and magnetic resonance imaging studies in large numbers of patients also fail to differentiate between the two MS categories. PPMS thus resembles SPMS without the relapses, although the two forms do differ with respect to sex ratio. An unfavourable outcome in PPMS in predicted by rapid early progression of disability and involvement of three or more systems. Natural history studies provide information on likely long-term outcomes and can be used in the design and interpretation of clinical trials in PPMS. The evidence that PPMS is distinct remains weak.
原发性进行性多发性硬化症（PPMS）与复发缓解型多发性硬化症（RRMS）和继发性进行性多发性硬化症（SPMS）之间的关系仍不明确。一项以人群为基础的PPMS患者队列随访约25年的自然史数据表明，PPMS和SPMS的进展阶段存在显著相似性。对大量患者的免疫遗传学和磁共振成像研究也未能区分这两种多发性硬化症类型。因此，PPMS类似于无复发的SPMS，尽管这两种形式在性别比例方面有所不同。PPMS早期残疾进展迅速且累及三个或更多系统预示着不良预后。自然史研究提供了可能的长期预后信息，可用于PPMS临床试验的设计和解读。PPMS具有独特性的证据仍然不足。
