López-Vega J M, Calleja J, Combarros O, Polo J M, Berciano J
Department of Medicine (Section of Neurology), National Hospital Marqués de Valdecilla, Faculty of Medicine, Santander, Spain.
Acta Neurol Scand. 1988 Jan;77(1):1-5. doi: 10.1111/j.1600-0404.1988.tb06965.x.
Sixty-two patients with motor neuron disease (MND), encompassing amyotrophic lateral sclerosis (ALS), progressive bulbar palsy (PBP) and progressive muscular atrophy (PMA), were selected from within a defined area (Cantabria) in northern Spain, from 1974 to 1985. The annual incidence of MND was 1.01 per 100,000 inhabitants and the prevalence rate was 3.52 per 100,000. The male to female ratio was 1.78:1. Age-specific incidence rates increased with advanced age, with a maximum between 60 and 69 years for males and over 70 years for females. The median age at onset was 60.5 years. The average interval between the onset symptoms and diagnosis was 11 months. Fifty-three per cent of the patients had conventional or pseudopolyneuritic ALS, 36% had PBP and 11% had PMA. There were three familial cases. Two PMA patients had had acute poliomyelitis. The mean duration of the disease was 26.6 months and was significantly longer in males aged under 60 years. The survival rates in 50 patients with adequate follow-up were 18% after 5 years from onset and 6% after 10 years.
1974年至1985年期间，从西班牙北部一个特定地区（坎塔布里亚）选取了62例运动神经元病（MND）患者，其中包括肌萎缩侧索硬化症（ALS）、进行性延髓麻痹（PBP）和进行性肌肉萎缩（PMA）。MND的年发病率为每10万居民1.01例，患病率为每10万居民3.52例。男女比例为1.78:1。年龄别发病率随年龄增长而增加，男性在60至69岁之间最高，女性在70岁以上最高。发病的中位年龄为60.5岁。首发症状与诊断之间的平均间隔为11个月。53%的患者患有传统型或假多神经炎型ALS，36%患有PBP，11%患有PMA。有3例家族性病例。2例PMA患者曾患急性脊髓灰质炎。疾病的平均持续时间为26.6个月，60岁以下男性患者的病程明显更长。50例有充分随访的患者从发病起5年后的生存率为18%，10年后为6%。
