Nilsson Petra, Larsson Elna-Marie, Maly-Sundgren Pia, Perfekt Roland, Sandberg-Wollheim Magnhild
Department of Neurology, University Hospital, 22185 Lund, Sweden.
J Neurol. 2005 Apr;252(4):396-402. doi: 10.1007/s00415-005-0655-9. Epub 2005 Mar 22.
Multiple sclerosis (MS) is a common disease with considerable risk for disability. Optic neuritis (ON) is a common first symptom of MS but it can also remain an isolated episode. Therefore, predicting the outcome of ON has gained in importance, particularly in light of current discussions of early disease modifying treatments in individuals at risk of developing MS. We reported previously on our cohort of 86 patients with acute monosymptomatic unilateral ON of whom 33 had progressed to MS after up to 18 years. Three patients had died. The present study extends the observation period to 31 years.
Patients were followed for up to 31 years or until a diagnosis of MS was made. Cerebrospinal fluid (CSF) was examined at onset. HLA class I and II antigens were determined. Magnetic Resonance Imaging (MRI) was performed during follow up.
Only one of 50 patients at risk developed clinical manifestations of MS during the extended follow up period. The estimated 15-year-risk of MS was 40 % (confidence interval [CI] 31%-52%). Most cases, 20 of 34 or 60%, occurred within three years. Among factors present at onset, CSF with mononuclear pleocytosis and/or oligoclonal Ig increased the risk for subsequent MS significantly, 49% (CI 38%-65%) compared with 23 % (CI 12%-44%) for those with normal CSF, p=0.02. Younger patients and those with winter onset also had greater risk. Recurrence of ON similarly elevated the risk significantly, p<0.001. After 19-31 years MRI lesions suggestive of demyelinating disease were detected in 20 of 30 individuals although no clinical manifestations of MS had occurred.
The risk of MS in this large population-based prospective ON patient series was 40% and significantly higher in those with inflammatory CSF abnormalities at onset. Clinically silent MRI lesions suggestive of MS were detected in a majority of those with "ON-only". This finding should be taken into account when discussing prognosis and early intervention in patients with clinically isolated ON.
多发性硬化症(MS)是一种常见疾病,致残风险较高。视神经炎(ON)是MS常见的首发症状,但也可能仅为孤立发作。因此,预测ON的预后变得愈发重要,尤其是鉴于当前对有发展为MS风险个体的早期疾病修正治疗的讨论。我们之前报告了86例急性单症状性单侧ON患者的队列研究,其中33例在长达18年后进展为MS。3例患者死亡。本研究将观察期延长至31年。
对患者进行长达31年的随访,或直至确诊为MS。发病时检查脑脊液(CSF)。测定HLA I类和II类抗原。随访期间进行磁共振成像(MRI)检查。
在延长的随访期内,50例有风险的患者中仅1例出现MS临床表现。估计MS的15年风险为40%(置信区间[CI] 31%-52%)。大多数病例,即34例中的20例或60%,发生在3年内。在发病时存在的因素中,脑脊液单核细胞增多和/或寡克隆Ig增加了后续发生MS的风险,与脑脊液正常者的23%(CI 12%-44%)相比,显著增加至49%(CI 38%-65%),p = 0.02。年轻患者和冬季发病者风险也更高。ON复发同样显著增加风险,p<0.001。19 - 31年后,30例中有20例检测到提示脱髓鞘疾病的MRI病变,尽管未出现MS的临床表现。
在这个基于人群的大型前瞻性ON患者系列中,MS风险为40%,发病时脑脊液有炎症异常者风险显著更高。在大多数“仅ON”患者中检测到提示MS的临床无症状MRI病变。在讨论临床孤立性ON患者的预后和早期干预时应考虑这一发现。