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失智症

失智症. 失智症的分類. 漸進性中樞神經退化 阿茲海默症 (AD) 、額顳葉形失智症 (FTD) 血管性失智症 腦中風、慢性腦血管病變 其他因素導致之失智症 路易氏體失智症、巴金森氏症、亨汀頓氏症、營養失調、顱內病灶、新陳代謝及電解質失調的內分泌疾病、中樞神經系統感染、藥物、酗酒、化學毒素中毒或重金屬中毒後遺症等. Synergistic interaction between causative factors in AD & VaD. Iadecola: Stroke, February 2003.

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失智症

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  1. 失智症

  2. 失智症的分類 • 漸進性中樞神經退化 • 阿茲海默症(AD)、額顳葉形失智症(FTD) • 血管性失智症 • 腦中風、慢性腦血管病變 • 其他因素導致之失智症 • 路易氏體失智症、巴金森氏症、亨汀頓氏症、營養失調、顱內病灶、新陳代謝及電解質失調的內分泌疾病、中樞神經系統感染、藥物、酗酒、化學毒素中毒或重金屬中毒後遺症等

  3. Synergistic interaction between causative factors in AD & VaD Iadecola: Stroke, February 2003

  4. Silver stained Alzheimer neuritic plaques (arrows) viewed at low power

  5. neurofibrillary tangle and amyloid plaque (silver stain  ) http://jswood.wordpress.com/ http://feany-lab.bwh.harvard.edu/link2/

  6. 台灣地區社區中老年人（≧65歲）失智症之盛行率台灣地區社區中老年人（≧65歲）失智症之盛行率 ＊≧60歲 △：只調查沒有罹患過腦中風的老人 劉景寬等：應用心理研究, 2000

  7. 4/4 Cupples AL et al. Genet Med 6: 192-196, 2004. Figure 1: Risk Curves of AD for Control Men

  8. TICS is a test of cognitive function Nature Reviews Genetics 6, April 2005

  9. OK>80 exp<45 OK at 80 OK at 100 胃癌 5 exp=50 記性差 OK at 80 Onset 48 exp=56 Onset 42 Onset 49 Onset 47 Onset 45 沒來 沒來 沒來 OK 56 53 50 36 沒來 OK ? OK OK OK OK ? OK ? ? 22 20 17 12 41 39 37 34 28 27 26 24 23 24 19 15 20 18 拒 有智能退化的症狀 臨床診斷為AD 分子診斷有PS1 mutation 分子診斷沒有PS1 mutation 沒有症狀的可疑帶因者 ? ? 31 23 20 15 OK 在PsyLab找到基因突變的第三個AD家族 Hong CJ, 2003/02/11

  10. AD052王OO48歲發病 (資料來源堂哥、太太) *3 *4 *5 *6 *7 50+y 40+y 50+y 50+y 50+y 63y 67y 65y 56y ?y 20+y 79y 77y 59y 52y OK OK OK 50+y 60+y 50+y ? ? 60y 53y 54y 48y 51y ? ? ? ?y ?y 25y 23y 37y ?y ?y ?y ?y 30y 23y 29y 25y 27y 22y ?y ?y ?y ?y ?y 1y 21y 30y 28y 26y 32y 46y 38y 48y 44y 40y 42y 67y 73y 35y ? 64y 59y ? 13y 19y 12y 17y 14y 13y 30y 46y 44y 42y ?y ?y 30y 35y 32y 30y 28y 73y 50+y 50+y 81y 69y ?y ?y ?y ?y ?y ?y ?y ?y ?y ?y ?y ? ?y ?y ?y ?y 46y 45y 43y ?y ?y 42y 基因檢測中 40y 38y 36y 智能不足 ? ? 有智能退化症狀 確認有PS1突變 確認無PS1突變 確診為AD ?y 5y ?y OK 1y

  11. Early onset(55y or 60y以下)且affected member≧1.5人, 找到突變的機會為50% (11/22)

  12. 早發的家族性失智症 • 阿茲海默氏症，約55% => 1-2%早發+家族性 • 血管性失智症，約20-25% • 混合性失智症，約5-10% • 額顳葉失智症，約5% => 20-40%早發+家族性

  13. AD & FTD Mutation Database 2008/11/30

  14. AD & FTD • AD的主要病理是腦組織有senile plaques + neurofibrilary tangles, FTD的主要病理是ubiquitin-positive的腦組織染色染色。如果沒有病理切片就只能靠臨床症狀做診斷。額顳葉型失智症臨床症狀除了記憶力退化之外最常出現的臨床症狀為人格改變(personality changes)，病人常出現不適當的行為，如社交退縮、易怒、無法克制自己的衝動、暴食、酗酒、情感冷漠、飲食喜好改變等與發病前大相逕庭的行為。另外也常可發現病患有語言表達困難的現象，如說話不流利、用詞沒有變化、語句怪異不自然等症狀。因此常被誤診為情感性精神病、精神分裂症或酒精濫用，有時候也很容易跟阿茲海默氏症搞混(Magnus Sjoeren 2006)。

  15. Neurology 2008;71:1191–1197

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