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[临床孤立综合征:转化为多发性硬化症及启动疾病修饰治疗的预后标志物]

[Clinically isolated syndrome: prognostic markers for conversion to multiple sclerosis and initiation of disease-modifying therapy].

作者信息

Kohriyama Tatsuo

机构信息

Department of Neurology, Hiroshima City Hospital.

出版信息

Rinsho Shinkeigaku. 2011 Mar;51(3):179-87. doi: 10.5692/clinicalneurol.51.179.

DOI:10.5692/clinicalneurol.51.179
PMID:21485162
Abstract

Eighty-five percent of patients with multiple sclerosis (MS) initially present with a single demyelinating event, referred to as a clinically isolated syndrome (CIS) of the optic nerves, brainstem, or spinal cord. Following the onset of CIS, 38 to 68% of patients develop clinically definite MS (CDMS). Clinically silent brain lesions are seen on MRI in 50 to 80% of patients with CIS at first clinical presentation and 56 to 88% of CIS patients with abnormal MRI are at high risk of conversion to CDMS. Axonal damage, that is considered to underlie the development of persistent disability in MS, occurs in the CIS stage. Treatment with disease-modifying therapies (DMTs), that might prevent axonal damage and result in slowing the progression of disability, should be initiated early during the disease course. Clinical trials demonstrated that early treatment of CIS patients with the standard dose of interferon beta (IFNbeta) significantly reduced the risk of progression to CDMS by 44 to 50%. After 5 years of followup, the results of the IFNbeta treatment extension studies confirmed that the risk of conversion to CDMS was significantly reduced by 35 to 37% in patients receiving early treatment compared to that in those receiving delayed treatment. However, not every patient with CIS will progress to CDMS; the IFNbeta treatment is appropriately indicated for CIS patients who are diagnosed with MS by McDonald diagnostic criteria based on MRI findings of dissemination in space and time and are at high risk for conversion to CDMS. Development of more reliable prognostic markers will enable DMTs to be targeted for those who are most likely to benefit.

摘要

85%的多发性硬化症(MS)患者最初表现为单一脱髓鞘事件,称为视神经、脑干或脊髓的临床孤立综合征(CIS)。CIS发病后,38%至68%的患者会发展为临床确诊的MS(CDMS)。在初次临床表现时,50%至80%的CIS患者MRI可见临床无症状脑损伤,MRI异常的CIS患者中有56%至88%有转化为CDMS的高风险。轴索损伤被认为是MS持续性残疾发展的基础,在CIS阶段就已发生。使用疾病修正疗法(DMTs)进行治疗,可能预防轴索损伤并减缓残疾进展,应在病程早期开始。临床试验表明,用标准剂量的干扰素β(IFNβ)早期治疗CIS患者可使进展为CDMS的风险显著降低44%至50%。经过5年随访,IFNβ治疗扩展研究结果证实,与延迟治疗的患者相比,早期治疗的患者转化为CDMS的风险显著降低35%至37%。然而,并非每个CIS患者都会进展为CDMS;IFNβ治疗适用于根据空间和时间播散的MRI表现,按照麦克唐纳诊断标准被诊断为MS且有转化为CDMS高风险的CIS患者。开发更可靠的预后标志物将使DMTs能够针对最可能受益的患者。

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