Blanc Frédéric, Verny Marc
Hôpital de jour de gériatrie, Centre mémoire de ressources et de recherche (CM2R), Pôle de gériatrie, Hôpital de la Robertsau, Hôpitaux universitaires de Strasbourg, Strasbourg, France, Équipe IMIS/Neurocrypto, ICube, UMR 7357, FMTS, Université de Strasbourg et CNRS, Strasbourg, France.
Centre de gériatrie et CM2R Île de France Sud, Hôpital Pitié-Salpêtrière, AP-HP, Paris, France, Université Pierre et Marie Curie et DHU FAST, UMR 8256 (CNRS), Paris, France.
Geriatr Psychol Neuropsychiatr Vieil. 2017 Jun 1;15(2):196-204. doi: 10.1684/pnv.2017.0675.
Disease with Lewy bodies or dementia with Lewy bodies (DLB), particularly at the prodromal stage, is a complex disease to diagnose because of different clinical beginnings and variable paths in terms of clinical expression. Thus DLB can be entcountered in different input modes: mild cognitive impairment, depression, acute behavioral disorders, confusion and delirium, or sleep disorders. In the aim to better diagnose the disease, should be sought obviously to search for the key symptoms: fluctuations, hallucinations, extra-pyramidal syndrome, and REM sleep behavior disorder. These symptoms are more subtle at the prodromal stage (mild neurocognitive disorder) than at the major stage. Thus fluctuations can be cognitive, simply as attentionnal fluctuations, or of alertness, such as sleepiness or more frequent nap; the visual phenomena can begin by sensation of passage, sensation of presence, or illusions; the extra-pyramidal syndrome can be really subtle such as isolated amimia or rigidity detected only with Froment's manœuvre. The frequent accompagnying symptoms are autonomic symptoms such as rhinorrhea or constipation, or sensorial symptoms such as olfactory impairment. The clinician has to be aware of the frequent presence of geriatric syndroms, also at the prodromal stage and including: falls, orthostatic hypotension, syncopa, urinary troubles, depression, delirium (after surgery, during infection...). On neuropsychological tests, sub-cortical frontal syndrome is frequent, visual memory impairment, visuospatial impairment and visuo-constructive difficulties are also characteristics. On brain MRI, isolated diminished insula is to look for. CSF analysis is usually normal but sometimes with low Abeta-42. Dat-Scan and MIBG scintigraphy were not enough explored in this context. Thus, with every patient presenting a compatible input mode, the search for symptoms of DLB has to be systematic, in the aim to have an etiological diagnosis of prodromal DLB, to avoid adverse drug events (neuroleptics) and to optimize care for patients.
路易体病或路易体痴呆(DLB),尤其是在前驱期,是一种难以诊断的复杂疾病,因为其临床起病不同,临床表现的路径也各异。因此,DLB 可通过不同的输入模式被发现:轻度认知障碍、抑郁、急性行为障碍、意识模糊和谵妄,或睡眠障碍。为了更好地诊断该疾病,显然应寻找关键症状:症状波动、幻觉、锥体外系综合征和快速眼动睡眠行为障碍。这些症状在前驱期(轻度神经认知障碍)比在主要阶段更为隐匿。因此,症状波动可以是认知性的,如单纯的注意力波动,或警觉性方面的,如嗜睡或更频繁的小睡;视觉现象可以从闪过感、存在感或幻觉开始;锥体外系综合征可能非常隐匿,如仅通过弗罗芒征检测到的孤立性表情缺乏或僵硬。常见的伴随症状是自主神经症状,如鼻溢或便秘,或感觉症状,如嗅觉障碍。临床医生必须意识到老年综合征也经常出现,在前驱期也包括:跌倒、体位性低血压、晕厥、泌尿系统问题、抑郁、谵妄(手术后、感染期间……)。在神经心理学测试中,皮质下额叶综合征很常见,视觉记忆损害、视觉空间损害和视觉构建困难也是其特征。在脑部 MRI 检查中,要寻找孤立的脑岛萎缩。脑脊液分析通常正常,但有时β-淀粉样蛋白 42 水平较低。在这种情况下,多巴胺转运体扫描(Dat-Scan)和间碘苄胍闪烁显像(MIBG 闪烁显像)的研究还不够充分。因此,对于每一位表现出符合症状的患者,都必须系统地寻找 DLB 的症状,以便对前驱期 DLB 进行病因诊断,避免不良药物事件(使用抗精神病药物)并优化患者的护理。