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本网站的医疗相关内容仅供参考，并不能视作专业意见。如需医疗服务或可靠意见，请咨询专业人士。| 路易氏体失智症 | |
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| 分类和外部资源 | |
| ICD-10 | G31.8 |
| ICD-9-CM | 331.82 |
| DiseasesDB | 3800 |
| eMedicine | neuro/91 |
| MeSH | D020961 |
路易氏体失智症（英语：Dementia with Lewy bodies，缩写为 DLB）是一种伴随着行为、认知及活动功能退化的失智症[1]，患者的记忆力虽不见得在罹病初期就会衰退[2]，但失智的情形还是会随着时间逐渐恶化[3]，通常是当患者的认知功能退化到影响日常生活，之后接受检查而确诊[1][4]。。此症的主要特征之一是动眼期睡眠行为障碍（RBD），患者在动眼期应出现的肌失张并未出现，反而会出现反应梦境之行为表现[1]；此障碍可能较其他症状提早数年甚至数十年就发生[5]。其他常见的症状包括出现幻觉、注意力的起伏不定及行动迟缓、步态不稳或运动功能减退[1]。患者的自主神经紊乱自主神经系统通常也会受到影响，导致血压、心脏及消化道功能改变，并且常常便秘[6]；此外像是变的抑郁及对人冷漠等情绪转变也很常见[1]。
目前还无法确定路易氏体失智症的确切原因[7]，不过和神经中α-突触核蛋白异常团块的广泛沉积有关，这种沈积称为路易氏体或路易氏突起（Lewy neurites）[8][9]。一般而言，路易氏体失智症不是遗传的，不过也有少数家庭的路易氏体失智症和基因有关[7]。可能性的诊断会以其症状以及生物标记来判断，诊断会包括血液检查、神经心理学测试、医学影像及多项生理睡眠检查[1][10]。其他会有类似症状的疾病有阿兹海默症、帕金森氏症、谵妄，偶尔思觉失调也会有类似症状[2]。
目前没有治愈方法或药物会改变疾病的进展[7]。这种病现行的治疗旨在缓解一些症状[7]并减轻照顾者的负担[4][11][12]。乙酰胆碱酯酶抑制剂（AChEI）如donepezil和rivastigmine，可有效改善认知和整体功能，褪黑激素则可用于睡眠相关症状[1]。即使有幻觉，患有DLB的人也会避免使用抗精神病药物，因为患者对这类药物很敏感[1]，若使用可能导致死亡[13]。特定症状的药物也可能会使另一种症状恶化[8]。
路易氏体失智症是常见失智症中的一种，另外二种是阿兹海默症及血管性痴呆[10][14][a]。路易氏体失智症通常发生在50岁以后，且大约0.4%的人65岁后会罹患此疾病[15]，疾病晚期，患者将无法照顾自己[16]，确诊后预期寿命约8年[7]不正常的蛋白质沉积机制于1912年由Frederic Lewy发现，而首次路易氏体失智症则在1976年由小阪宪司记录[3]。
知名患者
脚注
- ↑ Kosaka (2017) writes: "Dementia with Lewy bodies (DLB) is now well known to be the second most frequent dementia following Alzheimer disease (AD). Of all types of dementia, AD is known to account for about 50%, DLB about 20% and vascular dementia (VD) about 15%. Thus, AD, DLB, and VD are now considered to be the three major dementias."[14]The NINDS (2017) says that Lewy body dementia "is one of the most common causes of dementia, after Alzheimer’s disease and vascular disease."[10]
参考文献
- ↑ 1.0 1.1 1.2 1.3 1.4 1.5 1.6 1.7 McKeith IG, Boeve BF, Dickson DW, et al. Diagnosis and management of dementia with Lewy bodies: Fourth consensus report of the DLB Consortium. Neurology (Review). 2017-07, 89 (1): 88–100. PMC 5496518
. PMID 28592453. doi:10.1212/WNL.0000000000004058.
- ↑ 2.0 2.1 Tousi B. Diagnosis and management of cognitive and behavioral changes in dementia with Lewy bodies. Curr Treat Options Neurol (Review). 2017-10, 19 (11): 42. PMID 28990131. doi:10.1007/s11940-017-0478-x.
- ↑ 3.0 3.1 Weil RS, Lashley TL, Bras J, Schrag AE, Schott JM. Current concepts and controversies in the pathogenesis of Parkinson's disease dementia and dementia with Lewy bodies. F1000Res (Review). 2017, 6: 1604. PMC 5580419 . PMID 28928962. doi:10.12688/f1000research.11725.1.
- ↑ 4.0 4.1 St Louis EK, Boeve BF. REM sleep behavior disorder: Diagnosis, clinical implications, and future directions. Mayo Clin Proc (Review). 2017-11, 92 (11): 1723–36. PMID 29101940. doi:10.1016/j.mayocp.2017.09.007.
- ↑ St Louis EK, Boeve AR, Boeve BF. REM sleep behavior disorder in Parkinson's disease and other synucleinopathies. Mov Disord (Review). 2017-05, 32 (5): 645–58. PMID 28513079. doi:10.1002/mds.27018.
- ↑ Palma JA, Kaufmann H. Treatment of autonomic dysfunction in Parkinson disease and other synucleinopathies. Mov Disord (Review). 2018-03, 33 (3): 372–90. PMID 29508455. doi:10.1002/mds.27344.
- ↑ 7.0 7.1 7.2 7.3 7.4 Dementia with Lewy bodies information page. National Institute of Neurological Disorders and Stroke. 2017-05-25 [2018-04-07].
- ↑ 8.0 8.1 Walker Z, Possin KL, Boeve BF, Aarsland D. Lewy body dementias. Lancet (Review). 2015-10, 386 (10004): 1683–97. PMC 5792067 . PMID 26595642. doi:10.1016/S0140-6736(15)00462-6.
- ↑ Velayudhan L, Ffytche D, Ballard C, Aarsland D. New therapeutic strategies for Lewy body dementias. Curr Neurol Neurosci Rep (Review). 2017-09, 17 (9): 68. PMID 28741230. doi:10.1007/s11910-017-0778-2.
- ↑ 10.0 10.1 10.2 Lewy body dementia: Hope through research. National Institute of Neurological Disorders and Stroke. US National Institutes of Health. 2017-12-08 [2018-04-06].
- ↑ Mueller C, Ballard C, Corbett A, Aarsland D. The prognosis of dementia with Lewy bodies. Lancet Neurol (Review). 2017-05, 16 (5): 390–98. PMID 28342649. doi:10.1016/S1474-4422(17)30074-1.
- ↑ Boot BP, McDade EM, McGinnis SM, Boeve BF. Treatment of dementia with Lewy bodies. Curr Treat Options Neurol (Review). 2013-12, 15 (6): 738–64. PMC 3913181 . PMID 24222315. doi:10.1007/s11940-013-0261-6.
- ↑ Boot BP. Comprehensive treatment of dementia with Lewy bodies. Alzheimers Res Ther (Review). 2015, 7 (1): 45. PMC 4448151 . PMID 26029267. doi:10.1186/s13195-015-0128-z.
- ↑ 14.0 14.1 Kosaka K, ed. (2017), p. v.
- ↑ Levin J, Kurz A, Arzberger T, Giese A, Höglinger GU. The differential diagnosis and treatment of atypical parkinsonism. Dtsch Arztebl Int (Review). 2016-02, 113 (5): 61–69. PMC 4782269 . PMID 26900156. doi:10.3238/arztebl.2016.0061.
- ↑ What is Lewy body dementia?. National Institute on Aging. US National Institutes of Health. 2017-05-17 [2018-04-07].