Department of Neurology, Mayo Clinic College of Medicine, Rochester, MN, USA.
Mult Scler. 2011 Oct;17(10):1262-4. doi: 10.1177/1352458510387183. Epub 2010 Dec 6.
Primary progressive multiple sclerosis (PPMS), relapsing remitting MS (RRMS) and acute disseminated encephalomyelitis (ADEM) are clinically and immunopathogenetically distinct phenotypes of inflammatory demyelinating disorders of the central nervous system. Progression following RRMS is well described as secondary progressive MS. We report a patient with unexpected transition from long established PPMS to clinically and radiologically active RRMS after an ADEM-like fulminant demyelinating episode despite an immunosuppressive treatment preceding relapses. We note clearly accelerated brain atrophy after the RRMS course ensues. The unique disease course in this patient illustrates the dissociation of the biology and disability impact of relapses and progression.
原发性进展型多发性硬化症（PPMS）、复发缓解型多发性硬化症（RRMS）和急性播散性脑脊髓炎（ADEM）是中枢神经系统炎症性脱髓鞘疾病在临床上和免疫发病机制上不同的表现型。RRMS 后的进展被很好地描述为继发性进展型多发性硬化症。我们报告了一例患者，在经历了类似 ADEM 的暴发性脱髓鞘发作后，从长期确立的 PPMS 意外转变为临床上和影像学上活跃的 RRMS，尽管在此之前进行了免疫抑制治疗。我们注意到 RRMS 发作后大脑萎缩明显加速。该患者的独特疾病过程说明了复发和进展的生物学和残疾影响的分离。
