Bsteh Gabriel, Ehling Rainer, Walchhofer Lisa-Maria, Hegen Harald, Auer Michael, Wurth Sebastian, Di Pauli Franziska, Wagner Michaela, Reindl Markus, Deisenhammer Florian, Berger Thomas
Department of Neurology, Medical University of Innsbruck, Innsbruck, Austria.
Department of Neurology, Clinic for Rehabilitation Muenster, Muenster, Austria.
PLoS One. 2017 Jul 27;12(7):e0181458. doi: 10.1371/journal.pone.0181458. eCollection 2017.
Paroxysmal (PS) and unusual symptoms (US) account for approximately 1.6% of initial manifestations of multiple sclerosis (MS) and have comparable conversion rates to clinically definite MS (CDMS) as classical bout onset symptoms (CS). However, long-term prognosis and clinical outcome of patients experiencing PS or US as first clinical manifestation are unclear.
Clinical, MRI and cerebrospinal fluid data were obtained retrospectively and patients presenting with PS or US were compared to patients with CS presentation.
In a cohort of 532 relapsing onset MS patients followed for a mean period of 11.4 years (SD 3.6), 10 (1.9%) patients initially presented with PS/US. PS/US patients received disease modifying treatment (DMT) in a significantly smaller proportion immediately after the first clinical symptom (30% vs. 61.7%; p = 0.021) and during the observation period (60% vs. 83.5%; p = 0.033). In multivariate models correcting for sex, age at initial symptoms, complete remission of initial symptoms, total number of T2 and contrast-enhancing lesions, presence of oligoclonal bands and DMT exposure, PS/US were not associated with lower annualized relapse rate or lower EDSS over time.
In addition to a similar conversion rate to CDMS, patients presenting with PS/US at disease onset display very similar relapse and disability rates as patients with CS onset. Consequently, initial presentation with PS/US does not indicate benign or atypical MS, but requires DMT initiation based on the same criteria as in CS patients.
发作性(PS)和异常症状(US)约占多发性硬化症(MS)初始表现的1.6%,与经典发作性症状(CS)转化为临床确诊MS(CDMS)的转化率相当。然而,以PS或US作为首发临床表现的患者的长期预后和临床结局尚不清楚。
回顾性收集临床、MRI和脑脊液数据,将表现为PS或US的患者与表现为CS的患者进行比较。
在532例复发型MS患者队列中,平均随访11.4年(标准差3.6年),10例(1.9%)患者最初表现为PS/US。PS/US患者在首次临床症状出现后立即接受疾病修饰治疗(DMT)的比例显著较低(30%对61.7%;p = 0.021),在观察期内接受DMT的比例也较低(60%对83.5%;p = 0.033)。在对性别、初始症状时的年龄、初始症状完全缓解、T2和强化病灶总数、寡克隆带的存在以及DMT暴露进行校正的多变量模型中,PS/US与随时间推移较低的年化复发率或较低的扩展残疾状态量表(EDSS)无关。
除了与CDMS的转化率相似外,疾病发作时表现为PS/US的患者与CS发作患者的复发率和残疾率非常相似。因此,以PS/US作为初始表现并不表明是良性或非典型MS,但需要根据与CS患者相同的标准开始使用DMT。