继发进展型多发性硬化的临床诊断进展

Advances in clinical diagnosis of secondary progressive multiple sclerosis

多发性硬化(multiple sclerosis,MS)是一种自身免疫性慢性炎症性中枢神经系统脱髓鞘疾病。约95%的MS患者发病时为复发缓解型(relapsing remitting MS,RRMS),而其中多数RRMS患者会发展为继发进展型多发性硬化(secondary progressive MS,SPMS)。SPMS的发病机制目前尚不完全明确,但研究表明,SPMS的发生与中枢神经系统内缓慢扩张的病变、软脑膜滤泡样结构等机制相关,涉及驻留在中枢神经系统、软脑膜和脑脊液中的免疫细胞,造成神经元持续性变性损害和修复能力下降。在临床上,SPMS主要表现为患者残疾进展,但其诊断通常是回顾性的,仍然具有挑战性,因多达三分之二的具有潜在残疾恶化的患者仍被诊断为RRMS。此类患者继续接受针对RRMS的疾病修饰疗法可能效果欠佳。研究SPMS发病机制的有助于早期识别和诊断。

Multiple sclerosis(MS)is a chronic inflammatory disease of the central nervous system that involves demyelination and axonal degeneration.Usually,MS begins with a relapsing-remitting MS(RRMS)course,and approxi mately will develop into secondary progressive multiple sclerosis(SPMS).At present,the pathogenesis of SPMS is not completely clear,but studies have shown that the occurrence of SPMS is related to the slowly expanding lesions in the central nervous system,leptomeningeal follicle-like structure,chronic inflammation and brain atrophy.In clinical practice,the diagnosis of SPMS is often delayed and retrospective.Up to two-thirds of patients with insidious worsening of disability are still considered to have RRMS.These patients may continue to receive disease-modifying therapies for RRMS,which may not be effective for slowing SPMS.Therefore,early identification and diagnosis of SPMS are essential for their treatment.