Mild cognitive impairment associated with limbic and neocortical lewy body disease: a clinicopathological study

Mild cognitive impairment associated with limbic and neocortical lewy body disease: a clinicopathological study
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DOI:
10.1093/brain/awp280
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发表时间:
2010-02-01
期刊:
影响因子:
14.5
通讯作者:
Petersen, Ronald
Petersen, Ronald
中科院分区:
医学1区
文献类型:
--
作者:
Molano, Jennifer;Boeve, Bradley;Petersen, Ronald

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关于路易体病和轻度认知障碍综合征之间的关系的数据很少。在明尼苏达州罗切斯特和佛罗里达杰克逊维尔的马约诊所衰老和痴呆数据库中查询1996年1月1日至2008年4月30日期间诊断为轻度认知障碍的病例,前瞻性随访,随后发现尸检证实患有路易体病。快速眼动睡眠行为障碍的存在进行了专门评估。根据前瞻性操作标准,通过临床印象和神经心理学特征确定轻度认知功能障碍亚型。根据2005年McKeith标准,诊断为临床可能的路易体痴呆。海马体积、海马萎缩率和质子磁共振波谱通过可用的磁共振成像和波谱扫描进行评估。确定了8名受试者; 6名为男性。7人在死亡前患上了路易体痴呆症; 1人死亡的特征是轻度认知障碍。特定特征的病例数量和中位发病年龄(范围)为:7名患有快速眼动睡眠行为障碍的患者-60岁(27-91岁),8例有认知症状-69岁(62-89岁),8例轻度认知障碍-70.5岁(66-91岁),8例有帕金森症状-71岁(66-92岁),6人有幻视-72岁(64-90岁),7例痴呆症患者-75岁(67-92岁),6例认知和/或觉醒波动-76岁(68-92岁),8例死亡-76岁(71-94岁)。在6例病例中,快速眼动睡眠行为障碍先于认知症状发作,中位时间为10年(2-47年),轻度认知障碍诊断为12年(3-48年)。所代表的轻度认知功能障碍亚型包括:2例单域非遗忘型轻度认知功能障碍,3例多域非遗忘型轻度认知功能障碍,3例多域遗忘型轻度认知功能障碍。最常受影响的认知领域是注意力和执行功能以及视觉空间功能。在接受磁共振成像的三例患者中,海马体积和海马萎缩率平均在正常范围内,而在接受质子磁共振波谱检查的两例患者中,当他们被诊断为轻度时,胆碱/肌酸比率升高认知障碍。在尸检中,6人患有新皮质主导型路易体病,2人患有边缘主导型路易体病;只有1人同时患有高可能性阿尔茨海默病。这些发现表明,在经过轻度认知障碍阶段的路易体病病例中,任何认知模式或轻度认知亚型都是可能的,其中注意力/执行和视觉空间领域最常受损。海马体积和质子磁共振波谱数据与路易体痴呆的最新数据一致。所有患有快速眼动睡眠行为障碍和轻度认知障碍的病例最终都被证明患有尸检证实的路易体病,这表明快速眼动睡眠行为障碍加上轻度认知障碍可能反映了脑干和大脑路易体病。
There are little data on the relationship between Lewy body disease and mild cognitive impairment syndromes. The Mayo Clinic aging and dementia databases in Rochester, Minnesota, and Jacksonville, Florida were queried for cases who were diagnosed with mild cognitive impairment between 1 January 1996 and 30 April 2008, were prospectively followed and were subsequently found to have autopsy-proven Lewy body disease. The presence of rapid eye movement sleep behaviour disorder was specifically assessed. Mild cognitive impairment subtypes were determined by clinical impression and neuropsychological profiles, based on prospective operational criteria. The diagnosis of clinically probable dementia with Lewy bodies was based on the 2005 McKeith criteria. Hippocampal volumes, rate of hippocampal atrophy, and proton magnetic resonance spectroscopy were assessed on available magnetic resonance imaging and spectroscopy scans. Eight subjects were identified; six were male. Seven developed dementia with Lewy bodies prior to death; one died characterized as mild cognitive impairment. The number of cases and median age of onset (range) for specific features were: seven with rapid eye movement sleep behaviour disorder-60 years (27-91 years), eight with cognitive symptoms-69 years (62-89 years), eight with mild cognitive impairment-70.5 years (66-91 years), eight with parkinsonism symptoms-71 years (66-92 years), six with visual hallucinations-72 years (64-90 years), seven with dementia-75 years (67-92 years), six with fluctuations in cognition and/or arousal-76 years (68-92 years) and eight dead-76 years (71-94 years). Rapid eye movement sleep behaviour disorder preceded cognitive symptom onset in six cases by a median of 10 years (2-47 years) and mild cognitive impairment diagnosis by a median of 12 years (3-48 years). The mild cognitive impairment subtypes represented include: two with single domain non-amnestic mild cognitive impairment, three with multi-domain non-amnestic mild cognitive impairment, and three with multi-domain amnestic mild cognitive impairment. The cognitive domains most frequently affected were attention and executive functioning, and visuospatial functioning. Hippocampal volumes and the rate of hippocampal atrophy were, on average, within the normal range in the three cases who underwent magnetic resonance imaging, and the choline/creatine ratio was elevated in the two cases who underwent proton magnetic resonance spectroscopy when they were diagnosed as mild cognitive impairment. On autopsy, six had neocortical-predominant Lewy body disease and two had limbic-predominant Lewy body disease; only one had coexisting high-likelihood Alzheimer's disease. These findings indicate that among Lewy body disease cases that pass through a mild cognitive impairment stage, any cognitive pattern or mild cognitive subtype is possible, with the attention/executive and visuospatial domains most frequently impaired. Hippocampal volume and proton magnetic resonance spectroscopy data were consistent with recent data in dementia with Lewy bodies. All cases with rapid eye movement sleep behaviour disorder and mild cognitive impairment were eventually shown to have autopsy-proven Lewy body disease, indicating that rapid eye movement sleep behaviour disorder plus mild cognitive impairment probably reflects brainstem and cerebral Lewy body disease.