MOTOR NEURON DISEASE IN CANTABRIA
MOTOR NEURON DISEASE IN CANTABRIA
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DOI：
10.1111/j.1600-0404.1988.tb06965.x
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发表时间：
1988-01-01
影响因子：
3.5
通讯作者：
BERCIANO, J
中科院分区：
文献类型：
--
作者：
LOPEZVEGA, JM;CALLEJA, J;BERCIANO, J
从1974年至1985年，在西班牙北部的坎塔布里亚选定了62例运动神经元疾病（MND）患者，包括肌萎缩性侧索硬化症（ALS）、进行性球性麻痹（PBP）和进行性肌萎缩症（PMA）。MND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。特定年龄的发病率随着年龄的增长而增加，男性在60至69岁之间最高，女性在70岁以上最高。中位发病年龄为60.5岁。从出现症状到诊断的平均时间间隔为11个月。53%的患者为常规或假性多神经炎性ALS， 36%为PBP， 11%为PMA。有三个家族病例。2例PMA患者有急性脊髓灰质炎。平均病程为26.6个月，60岁以下男性病程明显更长。经充分随访的50例患者，5年生存率为18%，10年生存率为6%。
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.