筋萎缩性侧索硬化症
...另外此组病人脑脊液中抗GMI抗体增高的阳性率更高。有时需长时间随访,才能做出鉴别。 3、下肢中枢性瘫痪:下肢中枢性瘫痪是先天性脑积水的临床表现。脑积水严重,进展较快时,亦可出现,其症状为反复呕吐。脑退行性变,脑发育障碍,四肢中枢性瘫痪,尤以下肢为重。 根据临床表现进行诊断。 根据受损最严重的神经系统部位而定,临床症状也根据病变部位不同而各异,具体分型如下: 1.肌萎缩性侧索硬化症(ALS):最常见。发病年龄在40-50岁,男性多于女性。
肌萎缩侧索硬化
...Spine杂志近期报道了ALS对症疗法的讨论及其循证医学基础。肌萎缩侧索硬化(ALS)是一种进展性、无法治愈且致命的神经退行性疾病。疾病原因仍未完全明了,以至于缺乏有效延缓疾病进程的治疗手段。
肌萎缩性脊髓侧索硬化症(Amyotrophic Lateral Sclerosis)
...神经退行性疾病的一个常见特征是永久性的不溶性蛋白缠结物在细胞中形成。在阿尔茨海默病患者中发现的β-淀粉样蛋白斑块和在肌萎缩性脊髓侧索硬化症(ALS)患者大脑的运动神经元中发现的包涵体(inclusion body)是其中的两个例子。
杂粮面包
渐冻人协会 ; 侧索硬化症协会 ; 美国肌萎缩性脊髓侧索硬化症协会
冰桶挑战 ; 冰水挑战 ; 冰桶挑战赛
Amyotrophic lateral sclerosis (ALS), also known as Lou Gehrig's disease and Charcot disease, is a specific disorder that involves the death of neurons. In the United Kingdom the term motor neurone disease (MND) is commonly used, while others use that term for a group of five conditions of which ALS is the most common. ALS is characterized by stiff muscles, muscle twitching, and gradually worsening weakness due to muscle wasting. This results in difficulty speaking, swallowing, and eventually breathing.The cause is not known in 90% to 95% of cases. About 5-10% of cases are inherited from a person's parents. About half of these genetic cases are due to one of two specific genes. It results in the death of the neurons that control voluntary muscles. The diagnosis is based on a person's signs and symptoms with testing done to rule out other potential causes.There is no cure for ALS. A medication called riluzole may extend life expectancy by about two to three months. Non-invasive ventilation may result in both improved quality and length of life. The disease usually starts around the age of 60 and in inherited cases around the age of 50. The average survival from onset to death is three to four years. About 10% survive longer than 10 years. Most die from respiratory failure. In much of the world, rates of ALS are unknown. In Europe and the United States, the disease affects about 2 people per 100,000 per year.Descriptions of the disease date back to at least 1824 by Charles Bell. In 1869, the connection between the symptoms and the underlying neurological problems were first described by Jean-Martin Charcot, who in 1874 began using the term amyotrophic lateral sclerosis. It became well known in the United States when it affected the famous baseball player Lou Gehrig, and later when the ice bucket challenge became popular in 2014.