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原发进展型和继发进展型多发性硬化症的自然史比较揭示了差异与相似之处。

Natural history comparisons of primary and secondary progressive multiple sclerosis reveals differences and similarities.

作者信息

Tremlett Helen, Zhao Yinshan, Devonshire Virginia

机构信息

Dept. of Medicine (Neurology), rm S178, 2211 Wesbrook Mal, University of British Columbia, Vancouver, BC V6T 2B5, Canada.

出版信息

J Neurol. 2009 Mar;256(3):374-81. doi: 10.1007/s00415-009-0039-7. Epub 2009 Mar 18.

Abstract

BACKGROUND

Similarities in the onset age of progression in secondary-progressive (SP) and primary-progressive multiple sclerosis (PPMS) have been previously reported. However, with longer follow-up, more relapsing-remitting (RRMS) patients reach SPMS, such that the baseline characteristics, including age at progression may shift. We aimed to examine how this phenomenon impacts on demographic and clinical comparisons made between PP and SPMS.

METHODS AND RESULTS

Patients with definite MS, onset by July 1988 and > or = 1 Expanded Disability Status Scale (EDSS) score were selected from the British Columbia-wide MS database (n = 2837). Of these, 353 (12.4 %) had PPMS and 1445/2484 (58.2 %) of the RRMS population reached SPMS at study close (July 2003). Females predominated in the SPMS population regardless of follow-up time (p < or = 0.032). From Kaplan-Meier analysis (all RR, SP and PP patients considered), the estimated median onset age of progression was greater in SPMS (49.0 years; 95 % CI: 48.3-49.7) than PPMS (41.0 years; 95 % CI: 39.7-42.4), p < 0.0005. If the RR patients (who had not developed SPMS) were excluded, median age of onset of SPMS was still greater (43.1 years (95 % CI: 42.3-43.9, p < 0.0005).

CONCLUSIONS

Although there were some similarities between SPMS and PPMS, the former had a later onset age in our British Columbian MS cohort.

摘要

背景

先前已有报道称继发进展型(SP)多发性硬化症和原发进展型多发性硬化症(PPMS)在疾病进展起始年龄方面存在相似性。然而,随着随访时间延长,更多复发缓解型(RRMS)患者发展为继发进展型多发性硬化症(SPMS),使得包括疾病进展时年龄在内的基线特征可能发生变化。我们旨在研究这一现象如何影响PPMS和SPMS之间的人口统计学及临床比较。

方法与结果

从不列颠哥伦比亚省范围的多发性硬化症数据库中选取1988年7月前发病且扩展残疾状态量表(EDSS)评分≥1分的确诊多发性硬化症患者(n = 2837)。其中,353例(12.4%)患有PPMS,在研究结束时(2003年7月),RRMS人群中有1445/2484例(58.2%)发展为SPMS。无论随访时间长短,SPMS人群中女性均占主导(p≤0.032)。通过Kaplan-Meier分析(纳入所有RR、SP和PP患者),SPMS患者疾病进展的估计中位起始年龄(49.0岁;95%可信区间:48.3 - 49.7)大于PPMS患者(41.0岁;95%可信区间:39.7 - 42.4),p < 0.0005。若排除未发展为SPMS的RR患者,SPMS的中位发病年龄仍然更大(43.1岁(95%可信区间:42.3 - 43.9,p < 0.0005)。

结论

尽管SPMS和PPMS之间存在一些相似性,但在我们不列颠哥伦比亚省的多发性硬化症队列中,前者的发病年龄较晚。

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