路易尸体的痴呆症是什么？
Dementia with Lewy bodies (DLB) is a progressive neurodegenerative disorder that typically results in changes in attention, speed of information processing, visuospatial/visuoperceptual capacities, planning and memory.
认知功能通常会波动，偶发性混乱和警惕性降低。经常性视觉幻觉和帕金森主义是other defining clinical featuresDLB，除此之外，患者还可以在睡觉时“露出自己的梦想”（快速眼动睡眠行为障碍，RBD）。
在法国超过6000万的居民中，有100万人患有痴呆症，其中20万人将拥有DLB。DLB在认知模式下与阿尔茨海默氏病（AD）不同 - AD患者通常具有主要的记忆障碍 - 也是由于AD中没有DLB核心临床特征。
诊断疾病
患者可能不会向临床医生提及讲述的幻觉，这尤其是因为临床医生并不总是问他们。
但是，两种疾病之间的鉴别诊断在临床实践中并不那么容易。患者可能不会向临床医生提及讲述的幻觉，这尤其是因为临床医生并不总是问他们。
Similarly, patients and families are not easily able to describe the cognitive fluctuations to their doctor, and even if they do, the diagnostic significance of these isn’t always recognized. Parkinsonism is not always present in DLB, and even when it is, it can be so mild that it is hardly distinguishable from normal aging.
患者也可能没有意识到自己的RBD症状，尤其是当他们独自睡觉并且没有证人时。睡眠问题可能已经存在了多年，才在认知能力下降之前，患者及其家人没有连接这两个现象，因为临床医生可能没有，除非他们对DLB的表现有专业知识。
These diagnostic difficulties are even more problematic在轻度认知障碍的阶段（MCI）/前驱DLB。
A case example
为了更好地解释，我们可以使用83岁患者的情况
一开始，患者独自一人来，抱怨记忆力障碍，但没有其他问题。神经心理学测试显示出明显的言语和视觉记忆障碍以及细微的注意缺陷，而没有任何其他认知异常。
全球认知功能正常，MMSE（迷你心理状态检查）得分为29/30。身体检查没有帕金森氏症。脑MRI仅显示出微妙的海马萎缩。
由于AD而导致的诊断是轻度的认知障碍。没有开始治疗，但我们每年两次跟随患者。我们每年都问他有关波动，幻觉，RBD的信息，答案总是没有。
The only surprising thing we detected during the follow-up wasclear variation of the MMSE (from 29 to 18, and then to 26, etc…), without any overall decline in functional capacity, the patient remaining independent at home.
Thanks to his partner who attended the clinic and explained to us that he has for two years had visual hallucinations of isolated animals, clear fluctuations with episodes of confusion and was one time agitated during sleep and kicking her in bed.
最后我们现在有答案,谢谢to his partner who attended the clinic and explained to us that he has for two years had visual hallucinations of isolated animals, clear fluctuations with episodes of confusion and was one time agitated during sleep and kicking her in bed.
几个月前，我们决定进行腰椎穿刺进行脑脊液分析，并且AD的所有生物标志物（Abeta42，Tau和Phosho-tau）已正常返回。在反复的脑部MRI上，海马萎缩与七年前相同，但是当我们更准确地说岛时，我们看到了最初未检测到的更大的双侧萎缩，但是在那里进行了综述。
The reason for this is that previously we hadn’t been looking for atrophy of the insula. We only started doing it in Newcastle-upon-Tyne when we began a collaboration between Strasbourg and Newcastle to investigate the newly emerging concept of prodromal DLB.
What did we do?
首先，我们证明，将患者与临床定义的前驱动物DLB和AD进行比较，在皮质厚度方面显示了两件事：首先是前驱AD患者的颞叶内侧部分的众所周知的稀疏，其次，前叶的意外稀疏。岛的一部分in prodromal DLB patients。At the same time, colleagues from Chinadescribed该绝缘是在七个DLB患者的荟萃分析中双侧萎缩的。
我们的下一步使用来自Strasbourg和Newcastle的同一患者，是使用基于Voxel的形态计量学（VBM）研究前驱DLB患者的局灶性萎缩，找到了我们在这里报告的岛状和前扣带回萎缩。
This is consistent with what we had demonstrated previously using the ADNI cohort when we compared a group of patients with hallucinations (which is probably the best clinical biomarker of DLB) and another with the same MMSE but without hallucinations. Here we could demonstrate右前绝缘的萎缩在幻觉者中。
We recently found also that patients with prodromal DLB have a diminished perfusion of the insula.Thus, all these studies are consistent and in favour of early involvement of the insula in DLB.
The insula is a key region for cognition, emotions and neurovegetative aspects which has been implicated in the genesis of bothhallucinationsand认知波动。我们根据迄今为止的发现提出，该岛可能是DLB中局灶性萎缩的特定标记，这将是一个有用的诊断标记，将其与AD区分开来，并且适用于最困难的临床诊断的前驱阶段。
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