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我们几十年来完全忽视了另一种痴呆症

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Dementia is a neurodegenerative syndrome that affects memory, behavior, and cognitive function.

痴呆是一种影响记忆、行为和认知功能的神经退行性综合征。
Around 50 million people worldwide have a condition that causes it, such as Alzheimer's, and there are 10 million new diagnoses every year.
全世界约有五千万人患有引起这种疾病的病症,例如阿尔茨海默氏病,每年会诊断出一千万新病例。
Though we pour a whole lot of money into researching cures for conditions that cause dementia, so far we've not really turned up anything truly effective outside of mouse models.
尽管我们投入了大量资金来研究治疗引发痴呆症的疗法,但到目前为止,除了老鼠模型,我们还没有找到真正有效的方法。
Thankfully, after years of observations and studies, scientists have uncovered another piece of the puzzle.
谢天谢地,经过多年的观察和研究,科学家们发现了另一个谜团。
A previously unidentified type of dementia has been flying under the radar, and it's quietly been messing up our research efforts.
他们发现了一种以前未被确认的痴呆症,它正在悄悄地扰乱研究工作。
A paper published in 2019 in the journal Brain has formally defined a condition known as LATE.
2019年发表在《大脑》期刊上的一篇论文正式定义了一种称为LATE的疾病。
After years of studying dementia caused by the accumulation of a protein called TDP-43, it's only now that scientists have been able to formally say 'yes, this is its own condition'.
经过多年对一种名为TDP-43的蛋白质积累引起的痴呆症的研究,现在科学家才能正式地表明“是的,它本身是一种病症”。
When TDP-43 is working correctly, it hangs out in the nucleus of a cell, where it binds to DNA and regulates the production of proteins.
当TDP-43正常工作时,它会出现在细胞核中,在细胞核中与DNA结合并调节蛋白质的产生。
Normally, TDP-43 can also have a limited role outside of the cell's nucleus in protein production.
正常情况下,TDP-43在细胞核外蛋白质的生产中发挥的作用很有限。
But in cases of LATE, there are certain brain areas where TDP-43 starts to build up outside of the nucleus in tangles.
但在LATE的病例中,有些脑区的TDP-43开始在细胞核外聚集纠结。
These tangles are associated with damage to brain tissue.
这些纠结与脑组织损伤有关。
This can be observed in limbic brain regions - which are involved with memory - in three stages.
这可以在与记忆有关的边缘脑区分三个阶段观察到。
In the first stage, TDP-43 accumulates in the amygdala.
在第一阶段,TDP-43在杏仁核内积聚。
In stage two, it spreads to the hippocampus.
在第二阶段,它扩散到海马。
And in stage three, wandering TDP-43 can be found in the middle frontal gyrus.
在第三阶段,TDP-43在额叶中回游走。
We don't yet know what's causing this to happen.
我们还不知道这是什么原因造成的。
Genetic studies have so far indicated five different gene variants associated with a higher risk of LATE.
迄今为止,遗传研究表明,五种不同的基因变异与LATE的发病风险较高有关。
Many of these variants are also implicated in other types of neurodegenerative conditions, which indicates they're likely doing something similar in each case.
在这些变异中,有许多也与其他类型的神经退行性疾病有关,这表明它们可能在每种病例中都做了类似的事情。
But so far, there are no definitive answers.
但到目前为止,还没有明确的答案。
We do know that LATE produces symptoms that closely mimic Alzheimer's disease.
我们知道LATE会产生类似阿尔茨海默病的症状。
Things like forgetfulness, changes in behavior and personality, and other cognitive issues.
比如健忘、行为和个性的改变,以及其他认知问题。
They're so similar, that, like… we didn't know they were different for a long time.
它们如此相似,我们很久都不知道它们存在差异。
But unlike Alzheimer's disease, LATE occurs, well… late.
但与老年痴呆症不同的是,LATE会出现的较晚。
LATE appears predominantly in those aged over 80 years.
LATE主要发生在80岁以上的人群中。
Autopsy studies suggest evidence of LATE is present in anywhere from 20 to 50% of people in this age bracket.
尸检研究表明,在这个年龄段的人群中,20%到50%的人都有LATE的患病证据。
However, not everyone with TDP-43 accumulating in their brains will develop LATE symptoms, perhaps because they die of other causes before LATE symptoms emerge.
然而,并不是每个脑中积聚TDP-43的人都会出现LATE症状,也许是因为他们在LATE症状出现之前死于其他病因。
But this can happen with other forms of dementia, too.
但是,这也可能发生在其他形式的痴呆症上。
For whatever reason, just the presence of disease-associated proteins doesn't seem to be sufficient.
不管出于什么原因,仅仅存在与疾病相关的蛋白质似乎并不足够。

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Right now, it's next to impossible to tell the difference between Alzheimer's and LATE while someone is still alive.

现在,在一个人还活着的时候,几乎无法区分老年痴呆症和LATE痴呆症。
You'd have to take samples from limbic regions of the brain and stick them under a microscope, and… well, that's way too invasive, and could cause further damage.
你必须从大脑边缘区域采集样本,然后把它们贴在显微镜下,这样做也太具侵略性了,可能会造成进一步的伤害。
This has lit extra fire under researchers to develop less invasive tests to determine exactly what patients have, so that they can get the most suitable treatment as soon as possible.
这为研究人员点亮了另一个方向,以开发出具有较少侵入性的检测方法,来准确确定患者的病情,从而使他们能够尽快得到最合适的治疗。
In the future, clinicians hope to be able to identify specific markers in blood to make more definitive dementia diagnoses.
未来,临床医生希望能够识别血液中的特定标记物,从而做出更明确的痴呆诊断。
But we're not there yet.
但我们目前还没走到那一步。
And it turns out it's hard to research clear answers about a disease when you don't actually know for sure that people have it.
事实证明,当你不确定人们是否患有某种疾病时,很难找到明确的答案。
Since the presentations between LATE and Alzheimer's are so similar, researchers are concerned that we may have been accidentally labelling cases of LATE as Alzheimer's.
由于LATE和老年痴呆症的表现极其相似,研究人员担心,我们可能不小心将LATE病例标记为老年痴呆症。
In fact, they estimate that 15 to 20 % of all Alzheimer's diagnoses could actually be due to LATE.
事实上,他们估计15%到20%的阿尔茨海默病诊断实际上可能是源于LATE。
And as if it isn't complicated enough, many people with dementia will have multiple underlying conditions that cause it.
而且似乎还不够复杂,许多痴呆患者会有多种潜在的疾病会导致痴呆。
It's likely that many even have a combination of Alzheimer's and LATE.
很可能很多人甚至同时患有老年痴呆症和LATE。
This is important, because it means LATE has been diluting possible effects in our research for an Alzheimer's cure.
这一点很重要,因为这意味着在我们治疗老年痴呆症的研究中,LATE已经降低了其可能的影响。
In order to conduct a clinical trial properly, scientists need to be able to minimize interference from confounding variables - things that influence final measurements, without being the variable of interest.
为了正确地进行临床试验,科学家们需要能够最大限度地减少来自混杂变量的干扰——这些变量影响最终测量,而不是兴趣变量。
These are usually things like age, sex, or the presence of another health related condition.
这些变量通常是像年龄、性别,或存在的另一种与健康相关的疾病。
But when you're recruiting participants for a study on Alzheimer's, trying out a drug designed to treat Alzheimers, and it turns out that actually, some participants had this other thing that kinda looked like Alzheimer's…
但当你招募参与者进行老年痴呆症的研究时,尝试一种治疗老年痴呆症的药物,结果发现,实际上,有些参与患有其它类似老年痴呆症的病症。
Well, you're studying the wrong thing, and you don't even know it.
好吧,研究错了,而且甚至都没意识到。
While your shiny new drug might have helped out some of the sample with Alzheimer's, the ones with the Alzheimer's look-a-like with a completely different cause may not have been helped at all.
虽然新奇的药品可能帮助了一些阿尔茨海默氏症患者,但对于那些看起来像阿尔茨海默氏症,但病因完全不同的患者可能根本没有得到帮助。
And that drives down the measures of how effective your drug was, leading people to conclude that it was no good.
这就降低了衡量药物有效性的标准,导致人们认为它效果不好。
It might have been great!
可能药物本来很棒的!
It's just that you had such a massive confounding variable that it totally threw off your analysis.
只是混淆变量过大,以至于使分析完全偏离。
To combat this issue, the next step is to find ways to detect LATE in living patients.
为了解决这个问题,下一步是找到检测LATE患者的方法。
If they can do that, researchers will be able to effectively control for it in studies, begin working on a cure, and re-examine some of their old findings on Alzheimer's.
如果他们能做到这一点,研究人员将能够在研究中有效地控制它,开始研究疗法,并重新检查他们对老年痴呆症的一些旧有发现。
Which should be good for people with both conditions.
这对患有两种病症的人有益。
It's not likely to happen overnight.
这不可能在突然之间发生。
But with LATE identified, we've now got more pieces to dementia's puzzle, and we can continue to progress towards a cure.
但随着研究人员发现LATE,我们现在了解到更多关于痴呆症的谜团,可以继续寻找治疗方法。
Thanks for watching this episode of SciShow Psych.
感谢收看本集《心理科学秀》。
We love making free, awesome educational videos for everyone, and one amazing way you can help us do that is to become a patron.
我们喜欢为每个人制作免费的、很棒的教育视频,而你能帮助我们做到这一点的好方法就是成为赞助人。
If you'd like to lend a hand, check out patreon.com/scishow.
如果你想帮忙,请访问patreon.com/scishow。

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