瞭解路易氏體失智症：症狀與檢查方法-神經內科

洪煒斌 醫師回覆-神經內科

您好：1.路易氏體失智症，首先必須符合失智症 (日常生活功能因認知障礙造成退化)，其他的可能症狀包括病人的意識可能每天會出現明顯的變動、動作遲緩步態不穩、能夠仔細描述的視幻覺，其他還有記憶障礙、妄想、視覺空間障礙、睡眠障礙、自主神經障礙等等。
有興趣的話可以參考以下說明，這是關於路易氏體失智症的診斷準則 (可以用Google翻譯英翻中，但是有時對照原文比較不會理解錯誤)。
取自McKeith IG, Boeve BF, Dickson DW, et al. Diagnosis and management of dementia with Lewy bodies: Fourth consensus report of the DLB Consortium. Neurology. 2017;89(1):88-100. doi:10.1212/WNL.0000000000004058Essential for a diagnosis of DLB is dementia, defined as a progressive cognitive decline of sufficient magnitude to interfere with normal social or occupational functions, or with usual daily activities. Prominent or persistent memory impairment may not necessarily occur in the early stages but is usually evident with progression. Deficits on tests of attention, executive function, and visuoperceptual ability may be especially prominent and occur early.Core clinical features (The first 3 typically occur early and may persist throughout the course.)Fluctuating cognition with pronounced variations in attention and alertness.Recurrent visual hallucinations that are typically well formed and detailed.REM sleep behavior disorder, which may precede cognitive decline.One or more spontaneous cardinal features of parkinsonism: these are bradykinesia (defined as slowness of movement and decrement in amplitude or speed), rest tremor, or rigidity.Supportive clinical featuresSevere sensitivity to antipsychotic agents; postural instability; repeated falls; syncope or other transient episodes of unresponsiveness; severe autonomic dysfunction, e.g., constipation, orthostatic hypotension, urinary incontinence; hypersomnia; hyposmia; hallucinations in other modalities; systematized delusions; apathy, anxiety, and depression.Indicative biomarkersReduced dopamine transporter uptake in basal ganglia demonstrated by SPECT or PET.Abnormal (low uptake) 123iodine-MIBG myocardial scintigraphy.Polysomnographic confirmation of REM sleep without atonia.Supportive biomarkersRelative preservation of medial temporal lobe structures on CT/MRI scan.Generalized low uptake on SPECT/PET perfusion/metabolism scan with reduced occipital activity 6 the cingulate island sign on FDG-PET imaging.Prominent posterior slow-wave activity on EEG with periodic fluctuations in the pre-alpha/theta range.Probable DLB can be diagnosed if:a. Two or more core clinical features of DLB are present, with or without the presence of indicative biomarkers, orb. Only one core clinical feature is present, but with one or more indicative biomarkers.Probable DLB should not be diagnosed on the basis of biomarkers alone.Possible DLB can be diagnosed if:a. Only one core clinical feature of DLB is present, with no indicative biomarker evidence, orb. One or more indicative biomarkers is present but there are no core clinical features.DLB is less likely:a. In the presence of any other physical illness or brain disorder including cerebrovascular disease, sufficient to account in part or in total for the clinical picture, although these do not exclude a DLB diagnosis and may serve to indicate mixed or multiple pathologies contributing to the clinical presentation, orb. If parkinsonian features are the only core clinical feature and appear for the first time at a stage of severe dementia.DLB should be diagnosed when dementia occurs before or concurrently with parkinsonism. The term Parkinson disease dementia (PDD) should be used to describe dementia that occurs in the context of well-established Parkinson disease. In a practice setting the term that is most appropriate to the clinical situation should be used and generic terms such as Lewy body disease are often helpful. In research studies in which distinction needs to be made between DLB and PDD, the existing 1-year rule between the onset of dementia and parkinsonism continues to be recommended.2.如上面的診斷準則，目前對於診斷路易氏體失智症有幫助的檢查包括多巴胺受體造影 (dopamine transporter scan SPECT or PET)、碘123心臟閃爍造影 (123iodine-MIBG myocardial scintigraphy)、多頻道睡眠生理檢查 (Polysomnographic) 用來判斷是否有 快速動眼期失去動作抑制 (REM sleep without atonia)、電腦斷層/核磁共振、腦波、腦血流正子造影等等。
但是這些檢查是用來輔助診斷，並非診斷的黃金標準。