Kristensen O, Melgaard B
Acta Neurol Scand. 1977 Oct;56(4):299-308.
In order to evaluate the prognosis and possible prognostic factors associated with a bening course, 118 cases of motor neuron disease diagnosed 1948-1975 were followed up. Survival curves, based on the actuarial method showed a survival rate of 18.7 per cent (95 per cent confidence limits: 11.4-26.0) and 7.6 per cent (95 per cent confidence limits: 2.7-12.5) after 5 and 10 years respectively. Comparing the survival curves of males/females, cases with bulbar/spinal- and upper/lower extremity onset, cases with/without upper motor neuron signs, only bulbar onset was associated with a significantly poorer prognosis. However, cases with bulbar onset had a significantly higher mean age of onset. Based on cases from Funen county with a 1970-population of 432,699 we found an average annual incidence rate, mortality rate and period prevalence rate of 0.85, 0.86 and 2.5 per 100,000 respectively. Comparing the incidence per 5-year period through 1948-1972, no significant deviations indicating a changing environmental factor were found.
为了评估与良性病程相关的预后及可能的预后因素，对1948年至1975年间确诊的118例运动神经元病患者进行了随访。基于精算方法的生存曲线显示，5年和10年后的生存率分别为18.7%（95%置信区间：11.4 - 26.0）和7.6%（95%置信区间：2.7 - 12.5）。比较男性/女性、延髓/脊髓及上肢/下肢起病、有/无上运动神经元体征患者的生存曲线，仅延髓起病与显著较差的预后相关。然而，延髓起病的患者发病平均年龄显著更高。基于菲英岛县1970年人口432,699的病例，我们发现平均年发病率、死亡率和期间患病率分别为每10万人0.85、0.86和2.5。比较1948年至1972年每5年期间的发病率，未发现表明环境因素变化的显著偏差。
