[Amyotrophic lateral sclerosis].

J. Delattre
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Abstract

There are many different types of ALS; these types are distinguished by their signs and symptoms and their genetic cause or lack of clear genetic association. Most people with ALS have a form of the condition that is described as sporadic, which means it occurs in people with no apparent history of the disorder in their family. People with sporadic ALS usually first develop features of the condition in their late fifties or early sixties. A small proportion of people with ALS, estimated at 5 to 10 percent, have a family history of ALS or a related condition called frontotemporal dementia (FTD), which is a progressive brain disorder that affects personality, behavior, and language. The signs and symptoms of familial ALS typically first appear in one's late forties or early fifties. Rarely, people with familial ALS develop symptoms in childhood or their teenage years. These individuals have a rare form of the disorder known as juvenile ALS.
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肌萎缩性侧索硬化症。
ALS有很多不同的类型;这些类型的区别在于它们的体征和症状以及它们的遗传原因或缺乏明确的遗传关联。大多数ALS患者都有一种被描述为散发性的症状,这意味着它发生在没有明显家族病史的人身上。散发性肌萎缩侧索硬化症患者通常在50多岁或60岁出头时首次出现症状。一小部分ALS患者,估计有5%到10%,有ALS家族史或相关疾病额颞叶痴呆(FTD),这是一种影响人格、行为和语言的进行性大脑疾病。家族性肌萎缩侧索硬化症的症状和体征通常在一个人四十多岁或五十出头时首次出现。家族性ALS患者很少在童年或青少年时期出现症状。这些人患有一种罕见的疾病,称为青少年肌萎缩性侧索硬化症。
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