瞭解路易氏體失智症：症狀與檢查方法-神經內科

路易氏體失智症症狀？

1.網路查詢有多篇每篇講的都有點不一樣到底哪些症狀是路易氏體失智症的正確症狀?

2.現在有哪些放射性檢查可以檢查出路易氏體失智症?

謝謝醫師解答

lov157，30~39歲男性，詢問日期：2020/09/26

洪煒斌醫師回覆-神經內科

醫事人員經歷：衛生福利部臺南醫院神經內科特約主治醫師

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.0000000000004058

Essential 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 features
Severe 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 biomarkers
Reduced 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 biomarkers
Relative 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, or
b. 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, or
b. 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, or
b. 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。

回覆日期：2020/09/26
資料來源：台灣e院 - 路易氏體失智症症狀

彙整補充說明

路易氏體失智症（Dementia with Lewy Bodies, DLB）是一種特殊類型的失智症，主要特徵是認知功能的變化、視覺幻覺及帕金森症狀。
以下是針對您提出的問題的詳細解答：

1. 路易氏體失智症的症狀
路易氏體失智症的症狀可以分為核心症狀和輔助症狀：
核心症狀：
- 波動性認知功能：患者的注意力和警覺性會有明顯的波動，可能在一天內有不同的表現。

- 視覺幻覺：通常是詳細且具體的幻覺，患者可能會看到不存在的物體或人。

- 快速眼動睡眠行為障礙（REM sleep behavior disorder）：患者在睡眠中可能會出現劇烈的動作，甚至夢中行為，這通常會在認知衰退之前出現。

- 帕金森症狀：如動作遲緩、肌肉僵硬、顫抖等。

輔助症狀：
- 對抗精神病藥物的高度敏感性。

- 自主神經系統功能障礙，如便秘、低血壓、尿失禁等。

- 情緒問題，如焦慮、抑鬱等。

這些症狀可能會隨著病情的進展而變化，並且不一定每位患者都會出現所有症狀。
根據McKeith等人的診斷準則，確診需要有兩個或以上的核心症狀，並且可以輔以生物標記的檢查結果。

2. 檢查方法
目前對於路易氏體失智症的診斷，除了臨床症狀的評估外，還有幾種放射性檢查可以輔助診斷：
- 多巴胺轉運體掃描（Dopamine transporter scan, SPECT或PET）：這種掃描可以評估基底核的多巴胺活性，通常在路易氏體失智症患者中會顯示出多巴胺轉運體的減少。

- 碘-123心臟閃爍造影（123I-MIBG myocardial scintigraphy）：這項檢查可以評估自主神經系統的功能，路易氏體失智症患者通常會顯示出心臟的攝取減少。

- 腦部影像檢查（CT或MRI）：雖然這些檢查無法直接診斷路易氏體失智症，但可以排除其他可能的病因，如腦腫瘤或中風等。

- 腦波檢查（EEG）：可能會顯示出特定的波形變化，這在某些情況下可以輔助診斷。

這些檢查的結果需要與臨床症狀結合來解讀，因為單靠檢查結果並不能確診路易氏體失智症。

總結
路易氏體失智症是一種複雜的疾病，其症狀多樣且可能與其他疾病重疊，因此正確的診斷需要專業的醫療團隊進行全面的評估。
若您或您的家人有相關症狀，建議儘早就醫，並與神經科醫師進行詳細的討論與檢查，以獲得正確的診斷與適當的治療。
希望這些資訊能對您有所幫助，祝您健康！