Lance J W, Enis J, Duckworth J W
Clin Exp Neurol. 1977;14:42-50.
The case histories of 70 patients with typical motor neuron disease (MND) have been analysed to ascertain the natural history of the disease in Australia. The place of birth and residence during life of each patient has been sought to determine whether there is any 'clustering' of patients in the State of New South Wales. The mean age of onset was 55 years and mean duration of life from onset was 3 years 5 months. These figures are comparable with those from the USA and UK and Japan although the onset in Australia was later and the duration longer. The case history of 1 patient who is still living 38 years after the onset of the disease is described as there is no longer survival time recorded in the literature to our knowledge. Attention is drawn to muscle aches and cramps mentioned spontaneously by 27 of the 70 patients. These symptoms occurred at the onset of MND and often correlated with the site of onset of weakness. Weight loss averaged 1.6lb (0.7kg) per month in those patients in whom weight was recorded. The CSF protein was greater than 45mg/100ml, ranging up to 110mg/100ml, in 9 of 37 patients. The place of residence of patients bore no relation to known areas of increased manganese in the soil, and there was no evidence of 'clustering' of cases.
对70例典型运动神经元病（MND）患者的病历进行了分析，以确定澳大利亚该疾病的自然病程。已查明每位患者的出生地和一生中的居住地，以确定新南威尔士州是否存在患者“聚集”现象。发病的平均年龄为55岁，从发病起的平均寿命为3年5个月。这些数字与美国、英国和日本的数字相当，尽管澳大利亚的发病时间较晚，病程较长。描述了1例发病38年后仍在世患者的病历，据我们所知，文献中尚无更长的存活时间记录。值得注意的是，70例患者中有27例自发提到肌肉疼痛和痉挛。这些症状在运动神经元病发病时出现，且常与肌无力的发病部位相关。记录体重的患者体重平均每月下降1.6磅（0.7千克）。37例患者中有9例脑脊液蛋白大于45mg/100ml，最高可达110mg/100ml。患者的居住地与土壤中已知锰含量增加的地区无关，且没有病例“聚集”的证据。
