Kantarci O, Siva A, Eraksoy M, Karabudak R, Sütlaş N, Ağaoğlu J, Turan F, Ozmenoğlu M, Toğrul E, Demirkiran M
Department of Neurology, Istanbul University, Cerrahpaşa School of Medicine, Turkey.
Neurology. 1998 Sep;51(3):765-72. doi: 10.1212/wnl.51.3.765.
To examine the natural history, survival, and prognostic factors in a sample of Turkish MS patients.
This multicenter study included 1,259 definite MS patients diagnosed according to the criteria of Poser et al. Actuarial analysis of selected disability levels of 3, 6, 8, and 10 achieved with the Expanded Disability Status Scale (EDSS); a multivariate Cox regression analysis for prognostic factors related to time to reach EDSS > or = 6; and Pearson's correlation coefficient for individual factors were performed.
The survival (+/- SE) at 15 years from onset was 94.6 +/- 2.9%, and at 25 years was 89.0 +/- 5.8%. The disability reached by 15 years was EDSS > or = 3 in 66.4%, EDSS > or = 6 in 41.2%, EDSS > or = 8 in 10.5%, and EDSS = 10 in 5.4%. The most significant unfavorable prognostic factors were progressive course (relative risk [RR], 3.73; CI, 2.71 to 5.13) and sphincter symptoms at onset (RR, 1.86; CI, 1.23 to 2.82), followed by male sex, motor symptoms at onset, and a high attack frequency within the first 5 years. Primary progressive disease was correlated positively with male sex (r = 0.0895, p = 0.001), older age (r = 0.1807, p = 0.000), and motor (r = 0.1433, p = 0.000) or sphincter symptoms (r = 0.1001, p = 0.000) at onset, unlike relapsing-remitting and secondary progressive disease.
Although a slightly better prognosis is observed in the Turkish MS population, early prognostic factors are similar to most of the previous Western series. Primary progressive disease, mostly seen in older men with motor and sphincter involvement at onset, has a worse prognosis and may represent a distinct behavioral variant of MS.
研究一组土耳其多发性硬化症(MS)患者的自然病程、生存率及预后因素。
这项多中心研究纳入了1259例根据波泽等人的标准确诊的明确MS患者。对采用扩展残疾状态量表(EDSS)达到的3、6、8和10级选定残疾水平进行精算分析;对与达到EDSS≥6的时间相关的预后因素进行多变量Cox回归分析;并计算个体因素的Pearson相关系数。
起病后15年的生存率(±标准误)为94.6±2.9%,25年时为89.0±5.8%。15年时达到的残疾程度为:EDSS≥3的占66.4%,EDSS≥6的占41.2%,EDSS≥8的占10.5%,EDSS = 10的占5.4%。最显著的不良预后因素是病程呈进展型(相对风险[RR],3.73;可信区间[CI],2.71至5.13)和起病时出现括约肌症状(RR,1.86;CI,1.23至2.82),其次是男性、起病时出现运动症状以及前5年内发作频率高。原发性进展型疾病与男性(r = 0.0895,p = 0.001)、年龄较大(r = 0.1807,p = 0.000)以及起病时的运动(r = 0.1433,p = 0.000)或括约肌症状(r = 0.1001,p = 0.000)呈正相关,这与复发缓解型和继发进展型疾病不同。
尽管土耳其MS患者群体的预后稍好,但早期预后因素与之前大多数西方研究系列相似。原发性进展型疾病主要见于起病时出现运动和括约肌受累的老年男性,预后较差,可能代表MS的一种独特行为变异型。