ALS预后生存因素

渐冻人关爱在线北京大学第三医院神经内科王丽平

来源：http://alsn.mda.org/article/fa

Factors in Survival:
生存因素：

Age at diagnosis, diagnostic delay, fronto-temporal dementia, gender, genetic factors and modifiers, type of onset and rate of progression all play a role in length of survival.

诊断时的年龄，诊断延迟的时间，额颞叶痴呆，性别，遗传因素和基因修饰，发病类型和发展速率，这些都与生存时间有关系。

Factor
因素

Good or Bad?
好还是不好？

Notes
注释

Age at Diagnosis
诊断时的年龄
Generally, the younger the age of the individual at time of diagnosis, the longer the duration of the disease.
Survival time is longer for those who receive a diagnosis under age 45; diagnosis at old age (more than 65 years) is consistent with shorter survival time.
一般来说，诊断时患者的年龄越小，病程相对越长。45岁以下诊断的患者，生存时间较长；诊断时年龄较大（65岁以上），相对较短。Age is likely a better predictor of prognosis than any other “unalterable” factor.
Evidence from a number of studies shows a strong correlation between age at diagnosis and length of survival.
年龄作为预后预测指标，可能比其他任何“不可改变”的因素都好。多项研究的证据表明：诊断时的年龄与生存时间长短之间有很强的相关性。
Diagnostic Delay
诊断延迟Typically, the longer it takes to receive a definitive diagnosis from time of onset, the better the odds for longer survival; a short interval from onset to diagnosis is associated with shorter survival.
通常情况下，从发病到确诊的时间越长，长期生存的几率越大；反之则意味着较短的生存期。This may reflect the fact that more aggressive cases of ALS are more easily diagnosed; also, those who experience a slower course of disease may put off visiting a clinic.
这也许反映了一个事实：越是凶险的ALS病例，越容易早期诊断；同时，发展较慢的病例，则需要更长时间的临床观察（患者要经历更长时间才会去就医）。Fronto-Temporal Dementia (Ftd)
额颞叶痴呆Studies have shown shorter survival time in patients with ALS-FTD than in those who have onlyALS. FTD involves cognitive decline, including personality changes and language-related deficits.
研究显示：额颞叶痴呆型ALS比一般ALS生存时间短。额颞叶痴呆症状涉及认知能力下降，包括人格改变和语言障碍。May be attributable to the tendency of those with ALS-FTD to refuse or ignore treatment recommendations, such as assisted ventilation and feeding tubes.
可能是因为额颞叶痴呆性ALS患者有拒绝或忽略治疗措施（比如通气支持和饲管进食）的倾向。Gender
性别Female gender is often associated with shorter length of survival; males, longer.
女性生存时间较短，男性相对较长。Evidence for this is inconsistent and may be attributable to a greater incidence in women of bulbar-onset ALS, as well as more advanced age at onset of symptoms.
这方面的证据并不完全一致，有可能是因为女性延髓型ALS发病率较高，出现症状（发病）的年龄较大Genetic Factors

遗传因素Some familial forms of ALS have proven more aggressive than others.
一些家族型ALS被证明比其他类型的凶险Disease duration may be longer or shorter depending on the gene involved and the exact mutation, and may differ even among family members.
病程的长短依赖于所涉及的基因和明确的突变，即使是同一个家族的成员，也可能存在差异。
Genetic/Epigenetic Modifiers
基因/遗传修饰It’s suspected that factors that cause genes to behave differently without actually changing the DNA may positively or negatively affect length of survival.
有猜想认为，改变基因行为但不改变DNA本身，可能对生存时间产生积极或消极的影响。
These factors may apply in both familial and sporadic cases of ALS.
这些因素可能在家族和散发型ALS病例中都存在。Rate of Progression
疾病发展速率A slow rate of decline after disease onset tends to correlate with longer survival; the opposite is true for cases in which progression is rapid.
More severe symptoms at the time of diagnosis generally predict shorter survival time.
发病后较慢的衰退速度，意味着较长的生存时间，反之则较短。诊断时的症状比较严重，则预期生存期较短。Studies show strong evidence to support a relationship between rate of progression and survival length.
研究结果强烈支持疾病发展速率与生存时间之间的关系。Type Onset
发病类型Limb-onset (symptoms starting in an arm or leg) is consistent with an overall better prognosis.
Bulbar-onset (symptoms starting in the speaking, swallowing or facial muscles), typically more aggressive, is associated with shorter survival time.
Rates of progression in primary lateral sclerosis (PLS) and progressive muscular atrophy (PMA) are significantly slower.
肢体发病（症状从手臂或腿部开始）整体预后较好。延髓发病（症状从语言、吞咽、面部肌肉开始），通常病情更为凶险，生存时间相对较短。原发性侧索硬化（PLS）和进行性（脊）肌萎缩（PMA）发展速度明显较慢。
Evidence for these associations varies with different studies, possibly as a result of differing use of terminology among physicians.
关于这些联系的证据会随着不同的研究而改变，也可能由于医生使用的术语不同而不同。

原文链接：http://alsn.mda.org/article/fact ... wYPapipOH2uOTH1RjDw