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早发型多发性硬化症的病程及预后:与成人发病型的比较。

Course and prognosis in early-onset MS: comparison with adult-onset forms.

作者信息

Simone I L, Carrara D, Tortorella C, Liguori M, Lepore V, Pellegrini F, Bellacosa A, Ceccarelli A, Pavone I, Livrea P

机构信息

Department of Neurological and Psychiatric Sciences, University of Bari, Italy.

出版信息

Neurology. 2002 Dec 24;59(12):1922-8. doi: 10.1212/01.wnl.0000036907.37650.8e.

Abstract

OBJECTIVES

To establish the prognostic role of clinical and demographic factors in a hospital-based cohort of MS patients categorized by age at clinical onset and clinical course.

METHODS

Eighty-three patients with MS had a clinical onset of the disease in childhood (age <16 years; early-onset MS [EOMS]) and 710 in adult age (between 16 and 65 years; adult-onset MS [AOMS]). Patients were followed for a mean period of observation of 5 years. Univariate and multivariate analyses of clinical and demographic predictors for rapid progression and disability were performed using a stepwise Cox regression model with time-dependent covariates.

RESULTS

In EOMS, the Expanded Disability Status Scale (EDSS) evaluated at last clinical examination was lower than in AOMS, despite a longer disease duration. The probability to reach growth disability and progression was significantly lower in EOMS than in AOMS. Median times to reach EDSS score of 4 and secondary progression were longer in EOMS than in AOMS, but the age at both endpoints was significantly lower in EOMS. In EOMS and AOMS, an irreversible disability was related to a secondary progressive course, a sphincteric system involvement at onset, and an older age at onset (in EOMS only for the group >14 years); in AOMS, other unfavorable factors were a pyramidal involvement at onset and a high relapse frequency in the first 2 years. The risk of entering secondary progression was significantly influenced by a high number of relapses in EOMS and by a higher age at onset and a short interattack interval in AOMS.

CONCLUSION

A slower rate of progression of disease characterized EOMS patients, suggesting more plasticity to recover in developing CNS, but the early clinical manifestation cannot be considered a positive prognostic factor.

摘要

目的

在一个以医院为基础的多发性硬化症(MS)患者队列中,确定临床和人口统计学因素对按临床发病年龄和临床病程分类的患者的预后作用。

方法

83例MS患者在儿童期发病(年龄<16岁;早发型MS [EOMS]),710例在成年期发病(16至65岁之间;成年发病型MS [AOMS])。对患者进行了平均5年的观察随访。使用具有时间依赖性协变量的逐步Cox回归模型,对快速进展和残疾的临床及人口统计学预测因素进行单因素和多因素分析。

结果

在EOMS中,尽管病程较长,但在最后一次临床检查时评估的扩展残疾状态量表(EDSS)低于AOMS。EOMS中达到生长性残疾和进展的概率显著低于AOMS。EOMS达到EDSS评分为4和继发进展的中位时间比AOMS长,但两个终点的年龄在EOMS中均显著更低。在EOMS和AOMS中,不可逆残疾与继发进展型病程、发病时括约肌系统受累以及发病年龄较大有关(在EOMS中仅>14岁组);在AOMS中,其他不利因素是发病时锥体受累以及前2年复发频率高。EOMS中复发次数多以及AOMS中发病年龄较大和发作间期短对进入继发进展的风险有显著影响。

结论

EOMS患者疾病进展速度较慢,表明在发育中的中枢神经系统中恢复的可塑性更大,但早期临床表现不能被视为一个积极预后因素。

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