Hawkins S A, McDonnell G V
Northern Ireland Regional Neurology Service, Royal Victoria Hospital, Belfast, Northern Ireland, UK.
J Neurol Neurosurg Psychiatry. 1999 Aug;67(2):148-52. doi: 10.1136/jnnp.67.2.148.
To establish the characteristics of patients following a benign course of multiple sclerosis and evaluate the importance of potential prognostic factors. Also, an assessment of the value of the Kurtzke EDSS as a prognostic indicator has been undertaken in patients previously determined to have benign multiple sclerosis, after 10 years of follow up.
A prevalence study in the Coleraine, Ballymena, Ballymoney, and Moyle districts of Northern Ireland used the Kurtzke expanded disability scale score (EDSS) in 259 patients with multiple sclerosis. Of these, 181 had had multiple sclerosis for>/=10 years, 36 having benign disease (EDSS</=3.0) >/=10 years after onset. Clinical and demographic details of the various patient groups, including the minimal record of disability, were compared. The 1987 study in Northern Ireland identified 33 patients with benign multiple sclerosis. Twenty eight were available for follow up in 1996 along with 42 contemporary non-benign patients.
Patients with benign multiple sclerosis were predominantly women (ratio 4.1:1 v 2.1:1) and younger at onset (25.8 v 31.2 years). Commonest symptoms at onset were sensory and optic neuritis (33.3% each). Patients with late onset (older than 40 years) were less likely to have a benign course, more likely to have a progressive course from onset, significantly more likely to have motor disturbance at presentation, and had a lesser female predominance. Optic neuritis was significantly more common in those with a younger age at onset. In the follow up study, patients with benign multiple sclerosis continued to have a more favourable course than non-benign counterparts but progression of disability and to the secondary progressive phase remained significant.
The association of female sex, early onset, and presentation with optic neuritis and sensory symptoms with a favourable course is confirmed. However, although the EDSS does provide a useful indicator of prognosis, the label "benign multiple sclerosis" is often temporary as apparently benign disease often becomes disabling.
确定多发性硬化症良性病程患者的特征，并评估潜在预后因素的重要性。此外，在先前被判定为良性多发性硬化症的患者中，经过10年随访后，对Kurtzke扩展残疾状态量表（EDSS）作为预后指标的价值进行了评估。
在北爱尔兰的科尔雷恩、巴利米纳、巴利莫尼和莫伊尔地区进行了一项患病率研究，对259例多发性硬化症患者使用了Kurtzke扩展残疾量表评分（EDSS）。其中，181例患有多发性硬化症≥10年，36例在发病≥10年后患有良性疾病（EDSS≤3.0）。比较了不同患者组的临床和人口统计学细节，包括最小残疾记录。1987年在北爱尔兰进行的研究确定了33例良性多发性硬化症患者。1996年，28例可供随访，同时还有42例同期非良性患者。
良性多发性硬化症患者以女性为主（比例为4.1:1对2.1:1），发病年龄较轻（25.8岁对31.2岁）。发病时最常见的症状是感觉和视神经炎（各占33.3%）。发病较晚（40岁以上）的患者良性病程的可能性较小，从发病开始更可能有进展性病程，就诊时出现运动障碍的可能性显著更高，女性优势也较小。视神经炎在发病年龄较轻的患者中明显更常见。在随访研究中，良性多发性硬化症患者的病程仍比非良性患者更有利，但残疾进展和进入继发进展期仍然显著。
女性、早发、以视神经炎和感觉症状就诊与良好病程之间的关联得到证实。然而，尽管EDSS确实提供了一个有用的预后指标，但“良性多发性硬化症”这一标签往往是暂时的，因为看似良性的疾病往往会导致残疾。
