McDonald W I, Ron M A
Royal College of Physicians, London, UK.
Philos Trans R Soc Lond B Biol Sci. 1999 Oct 29;354(1390):1615-22. doi: 10.1098/rstb.1999.0506.
Multiple sclerosis is an immune-mediated inflammatory demyelinating disease of the central nervous system clinically characterized by relapses and remissions of neurological disturbance. A typical relapse, exemplified by optic neuritis, increases in severity over a week or two and after approximately one month begins to remit. Resolution takes place over the course of two to three months. In the early stages, clinical recovery is virtually complete, though persistent abnormalities of conduction can usually be detected by evoked potential techniques and persistent structural abnormalities can be detected by magnetic resonance imaging (MRI). These techniques, together with cerebrospinal fluid examination for oligoclonal IgG, provide supporting evidence for the diagnosis which, in the absence of a specific test, nevertheless remains primarily clinical. The course of the disease is very variable, but after a number of years neurological deficit begins to accumulate after each relapse. In most patients, the relapsing and remitting phase of the disease is followed by a phase of continuous progression of disability. Cognitive disturbances can be detected in many patients even quite early in the course of the illness. Deficits in attention, memory and executive skills may be prominent and tend to become increasingly prominent as neurological deficit increases, although this is not always the case. There is some correlation between the extent of MRI abnormalities in the cerebral white matter and the severity of cognitive deficit. Depression and anxiety are commonly experienced but are poorly correlated to the lesion load seen on MRI. In contrast, the much rarer psychotic symptoms, euphoria and emotional lability are closely linked to the severity of white matter disease.
多发性硬化症是一种中枢神经系统的免疫介导性炎症性脱髓鞘疾病,临床特征为神经功能障碍的复发和缓解。以视神经炎为例的典型复发,在一两周内严重程度增加,大约一个月后开始缓解。恢复过程需要两到三个月。在早期阶段,临床恢复几乎是完全的,尽管通过诱发电位技术通常可以检测到持续的传导异常,通过磁共振成像(MRI)可以检测到持续的结构异常。这些技术,连同对寡克隆IgG的脑脊液检查,为诊断提供了支持性证据,尽管缺乏特异性检测,但诊断仍主要基于临床。疾病的病程变化很大,但数年后每次复发后神经功能缺损开始累积。在大多数患者中,疾病的复发缓解期之后是残疾持续进展的阶段。即使在疾病过程的相当早期,也可以在许多患者中检测到认知障碍。注意力、记忆力和执行技能的缺陷可能很突出,并且往往随着神经功能缺损的增加而变得越来越突出,尽管并非总是如此。脑白质MRI异常的程度与认知缺陷的严重程度之间存在一定相关性。抑郁和焦虑很常见,但与MRI上所见的病变负荷相关性较差。相比之下,罕见得多的精神病性症状、欣快和情绪不稳定与白质疾病的严重程度密切相关。