Clinical study of primary progressive multiple sclerosis in Northern Ireland, UK
Clinical study of primary progressive multiple sclerosis in Northern Ireland, UK
复制标题
英国北爱尔兰原发性进行性多发性硬化症的临床研究
DOI：
10.1136/jnnp.64.4.451
复制
发表时间：
1998
期刊：
影响因子：
--
通讯作者：
S. Hawkins
中科院分区：
文献类型：
--
作者：
G. McDonnell;S. Hawkins
目的 调查北爱尔兰原发性进行性多发性硬化症 (PPMS) 的临床和人口特征，并建立此类患者的数据库，用于遗传和免疫学研究以及未来的治疗试验。方法 由两名神经科医生进行诊断和分类，潜在病例从以下来源确定：神经内科门诊；神经内科住院患者；出院审查；以及正在进行的北爱尔兰多发性硬化症流行病学研究。只有那些从发病开始就呈进展性且既往无复发病史的患者才会被接受。邀请潜在病例进行访谈和评估，建立最低残疾记录（MRD）。结果 已发现 111 例 PPMS，其中 63 名女性和 48 名男性（比例 1.3:1），平均发病年龄为 39.5（SD 11.0）（范围 17-66）岁，平均病程为 13.6（SD 9.3）年。发病和诊断之间的平均间隔为 4.7 (SD 4.2) 年。 19 名患者 (17.1%) 不满足 Poser 标准中任何类别的要求。运动障碍是最常见的发病方式（67.6%），发病时视力丧失很少发生（3.6%）。 Kurtzke EDSS 分数集中在量表的高端，中位数为 6.0，失业率和经济依赖程度很高。结论 与多发性硬化症的其他亚组相比，北爱尔兰的 PPMS 发病年龄普遍较晚，女性发病率较低，并且主要以运动性发病为主。诊断延迟反映了起病往往隐匿，而且临床病程的性质使得 Poser 标准的应用变得困难。 MRD 测量的神经损伤、残疾和残障水平很高。
OBJECTIVE To investigate the clinical and demographic characteristics of primary progressive multiple sclerosis (PPMS) in Northern Ireland and to establish a database of such patients for genetic and immunological studies and future therapeutic trials. METHODS Diagnosis and categorisation were performed by two neurologists, potential cases being identified from the following sources: neurology outpatient clinics; neurology inpatients; a review of hospital discharges; and an ongoing epidemiological study of multiple sclerosis in Northern Ireland. Only those with a progressive course from onset and a clear history of no prior relapses were accepted. Potential cases were invited for interview and assessment, the minimal record of disability (MRD) being established. RESULTS One hundred and eleven cases of PPMS have been identified, 63 women and 48 men (ratio 1.3:1), with a mean age at onset of 39.5 (SD 11.0) (range 17–66)years, and mean disease duration of 13.6 (SD 9.3)years. The mean interval between onset and diagnosis was 4.7 (SD 4.2) years. Nineteen patients (17.1%) did not satisfy the requirements for any category in the Poser criteria. Motor disturbance was the commonest mode of onset (67.6%) with visual loss occurring only rarely at onset (3.6%). Kurtzke EDSS scores were concentrated at the upper end of the scale with a median of 6.0 and levels of unemployment and financial dependence were high. CONCLUSIONS PPMS in Northern Ireland has a generally later age at onset, lower female preponderance, and predominantly motor onset compared with other subgroups of multiple sclerosis. The delay to diagnosis reflects the often insidious onset and the nature of the clinical course makes application of the Poser criteria difficult. Levels of neurological impairment, disability, and handicap as measured by the MRD are high.