De Seze J, Mackowiak A, Stojkovic T, Ferriby D, Hautecoeur P, Vermersch P
Au nom du Groupe Septentrional d'étude et de recherche sur la Sclérose en plaques (G-SEP).
Rev Neurol (Paris). 2002 Mar;158(3):341-5.
Approximately 15 p.100 of all cases of multiple sclerosis (MS) are primary progressive multiple sclerosis (PPMS). Diagnosis is however frequently delayed due to the lack of relapse. The commonly used Poser criteria established in 1983 are not directly applicable to this subgroup of MS patients. In 2000, Thompson et al proposed new diagnostic criteria for PPMS. The aim of our study was to apply retrospectively these new criteria to a cohort of patients from northern France (G-SEP cohort). The cohort included 184 patients (94 women and 90 men, sex ratio=1.04). Mean age at disease onset was 40.3 years (18-67 years) and mean follow-up was 9.9 (1-39 years). Only one patient was older than 65 years but 13 patients (7.1 p.100) were younger than 25 years. Patients were classed as having definite, probable or possible PPMS on the basis of clinical, MRI, cerebrospinal fluid and visual evoked potential (VEP) data. Most of the patients (61.4 p.100) had one symptom at onset; spastic paraparesis was frequent (79 p.100). Mean delay to diagnosis was 4.4 years. Ten patients (5.4 p.100) were surgically treated for cervic arthrosic myelopathy. Mean EDSS score at the end of follow-up was 5.8 (3-10). MRI was positive in 87.3 p.100 of the patients. Oligoclonal bands were found in 78.2 p.100. VEPs ware abnormal in 79.9 p.100 of the patients. Applying the Thompson criteria, 57.7 p.100 of the patients had definite PPMS, 38.7 p.100 probable PPMS. Our results are very similar to previous studies and confirm the usefulness of the new proposed criteria, which however should be prospectively tested to determine sensitivity and specificity in a new cohort of patients.
所有多发性硬化症(MS)病例中约15%为原发性进行性多发性硬化症(PPMS)。然而,由于缺乏复发情况,诊断常常延迟。1983年制定的常用波泽标准并不直接适用于这一亚组的MS患者。2000年,汤普森等人提出了PPMS的新诊断标准。我们研究的目的是将这些新标准回顾性地应用于来自法国北部的一组患者(G - SEP队列)。该队列包括184名患者(94名女性和90名男性,性别比 = 1.04)。疾病发病的平均年龄为40.3岁(18 - 67岁),平均随访时间为9.9年(1 - 39年)。只有1名患者年龄超过65岁,但有13名患者(7.1%)年龄小于25岁。根据临床、MRI、脑脊液和视觉诱发电位(VEP)数据,患者被分类为确诊、可能或疑似PPMS。大多数患者(61.4%)发病时有1种症状;痉挛性截瘫很常见(79%)。诊断的平均延迟时间为4.4年。10名患者(5.4%)因颈椎关节病性脊髓病接受了手术治疗。随访结束时的平均扩展残疾状态量表(EDSS)评分为5.8(3 - 10)。87.3%的患者MRI呈阳性。78.2%的患者发现寡克隆带。79.9%的患者VEP异常。应用汤普森标准,57.7%的患者为确诊PPMS,38.7%为可能PPMS。我们的结果与先前的研究非常相似,并证实了新提出标准的有用性,然而,应该对其进行前瞻性测试,以确定在新的患者队列中的敏感性和特异性。