Gubbay S S, Kahana E, Zilber N, Cooper G, Pintov S, Leibowitz Y
J Neurol. 1985;232(5):295-300. doi: 10.1007/BF00313868.
All identified Israeli patients with amyotrophic lateral sclerosis (ALS) with onset of the disease from 1959 through 1975 (n = 318) were evaluated clinically. Most of our patients (63%) presented with weakness; only 10% presented with atrophy and 3% with fasciculations. In 31% of the cases, the onset of the disease was focal and 22% of the patients presented with bulbar signs, but only 6 patients presented with emotional lability (pseudo-bulbar). Twelve per cent of the patients presented with muscle cramps, pain or paraesthesia. Atypical signs such as motor cranial nerve lesion, dementia, sphincter disturbance and deep sensation loss are discussed. A relatively high proportion of our patients suffered from malignant tumour, but with no association with any specific tumour. The median survival time was 3 years. Patients with onset of their disease with bulbar signs had a shorter life expectancy (2.2 years): Twenty nine per cent of our patients survived for more than 5 years and 16% for more than 10 years.
对所有确诊的1959年至1975年发病的以色列肌萎缩侧索硬化症（ALS）患者（n = 318）进行了临床评估。我们的大多数患者（63%）表现为肌无力；仅有10%表现为萎缩，3%表现为肌束震颤。在31%的病例中，疾病起病为局灶性，22%的患者出现延髓体征，但仅有6例患者表现为情感不稳定（假性延髓麻痹）。12%的患者出现肌肉痉挛、疼痛或感觉异常。文中讨论了诸如运动性颅神经病变、痴呆、括约肌功能障碍和深感觉丧失等非典型体征。我们的患者中有相对较高比例患有恶性肿瘤，但与任何特定肿瘤均无关联。中位生存时间为3年。起病时有延髓体征的患者预期寿命较短（2.2年）：我们的患者中有29%存活超过5年，16%存活超过10年。
