肌萎缩侧索硬化伴锥体外系运动障碍和痴呆称Mariana型肌萎缩侧索硬化症。是西太平洋的马里亚钠群岛上的一种地方病,发生于Chamono族居民中,有明显的家族史。外族人中亦有散发出现,我们亦遇到2例报道如下: 例1:男,42岁,江苏籍。因进行性前臂肌萎缩伴震颤,智力减退2年,于1985年12月15日入院。患者2年前发现双上肢前臂及手部肌肉萎缩,相继出现双手静止性震颤,四肢僵直,运动缓慢,记忆力减退,计算不准。时感肩、背部肌肉跳动。病情逐渐加重。2个月来因四肢颤动、僵硬、乏力,丧失工作能力。无脑炎、高血压、中风、脑外伤及中毒史。无家族史。查体:颅神经检查阴性,视乳头边界清。四肢肌肉均有萎缩,以双上肢前臂及手部大小鱼际肌、骨间肌为著,肌力4°,肌张力呈齿轮样增高;双上肢静止性震颤,双下肢膝反射卅,病理反射(一),感觉无异 Amyotrophic lateral sclerosis with extrapyramidal movement disorders and dementia called Mariana-type amyotrophic lateral sclerosis. Is a endemic disease on the islands of the Marianas in the Western Pacific that occurs among Chamono residents and has a clear family history. There are also outbreaks of alien people, we also encountered two cases reported as follows: Example 1: Male, 42 years old, Jiangsu membership. Because of progressive forearm muscle atrophy with tremor, mental retardation for 2 years, on December 15, 1985 admission. Two years ago, the patient found muscle atrophy on his forearms and hands in his upper extremity, and his hands had static tremor one after another. His limbs were stiff, his movements slowed, their memory was diminished, and his calculations were not allowed. When the sense of shoulder, back muscles beating. The condition gradually aggravated. 2 months due to limbs trembling, stiff, weak, incapacitated. No encephalitis, high blood pressure, stroke, traumatic brain injury and poisoning history. No family history. Examination: Negative cranial nerve examination, clear nipple border. Both limbs muscle atrophy, both the upper limb forearm and hand size of the intermuscular muscle, interosseous muscle is located, muscle strength 4 °, muscle tension was gear-like; resting upper extremity tremor, knee lower extremity knee reflex, pathological reflex (A), no different feeling