阿尔茨海默病与路易体痴呆的脑葡萄糖代谢比较

目的 比较阿尔茨海默病与路易体痴呆在脑葡萄糖代谢方面的特征.方法 本研究入选了28例阿尔茨海默病(AD)患者和25例路易体痴呆(DLB)患者.受试者静脉注射示踪刺18F脱氧葡萄糖(18F-FDG),然后接受头部正电子发射断层扫描(PET).应用迭代法重建的脑图像,分别取额叶皮层、顶叶皮层、颞叶皮层、枕叶皮层、小脑皮层、尾状核头、壳核为感兴趣区(ROI).以脑内各ROI与小脑半球ROI的FDG摄取强度的比值为指标进行分析和比较.结果 AD在颞叶前部、顶叶皮层FDG摄取下降,额、顶、颞、枕叶、壳核、尾状核皮层感兴趣区与小脑皮层的FDG代谢比值分别为(0.861±0.173)、(0.625±0.149)、(0.598 ±0.185)、(0.914±0.214)、(1.030 ±0.084)、(0.997±0.102);DLB在额叶前部皮层、颞叶前部皮层、顶叶后部皮层、纹状体的FDG摄取均下降,额、顶、颞、枕叶、壳核、尾状核皮层感兴趣区与小脑皮层的FDG代谢比值分别为(0.538±0.147)、(0.615±0.138)、(0.587 ±0.142)、(0.415 ±0.107)、(0.685 ±0.094)、(0.547 ±0.103).DLB在枕叶皮层、额叶皮层、纹状体的FDG摄取下降比AD显著,差异有统计学意义(P均＜0.01).结论 AD与DLB在皮层和基底节的葡萄糖代谢特征存在显著差异.这些特征有助于两者的鉴别诊断.     

认识路易体痴呆

路易体痴呆是神经交性痴呆中仅次于Alzheimer病(AD)居第二位的常见类型，约占痴呆的15％-25％，已被公认为一个独立的疾病实体。与一般的单一疾病实体所不同的是，路易体痴呆包含了多种疾病的表现，其临床特征为波动性认知损伤，以视幻觉为主的精神症状和锥体外系症状，组织学必须满足大脑皮层及皮层下核团弥散分布的路易小体。由于路易体痴呆具有不同于其他交性痴呆疾病的病程、预后和治疗反应，因此准确的临床诊断和适当的管理措施如避免使用神经安定剂、合理正确的使用抗帕金森病(PD)药物是临床医师的重要责任、     

帕金森病合并轻度认知障碍的危险因素和脑葡萄糖代谢模式

目的 研究帕金森病(PD)合并轻度认知障碍的危险因素和脑葡萄糖代谢模式.方法 对101例非痴呆PD患者应用蒙特利尔认知评测量表进行认知评测并分类为轻度认知障碍组(PD-MCI组)和非认知障碍组(PD-NC组),分别用统一PD评定量表(UPDRS)、Hoher-Yahr分期、汉密尔顿抑郁量表(HAMD)等对2组患者进行比较.对2组中Hohen-Yahr Ⅰ期患者应用18F-脱氧葡萄糖(18F-FDG)正电子发射断层扫描(PET)进行脑部代谢检测,以皮层各感兴趣区与小脑半球FDG摄取强度比值作为指标进行分析和比较.结果 受试者中有77例(76.2%)合并轻度认知障碍.PD-MCI组的UPDRS第一(精神、行为和情绪)、二(日常生活活动能力)、三(运动检查)部分评分[(2.48±1.51)分,(10.71±4.88)分,(22.31±12.70)分]均高于PD-NC组[(1.65±1.29)分,(8.15±2.20)分,(15.92±7.56)分](P均＜0.05).PD-MCI组的抑郁评分[(11.16±7.67)分与(6.50±4.02)分]均高于PD-NC组(P均＜0.05),但教育程度低于PD-NC组(P＜0.05).PD-MCI组在额叶、顶叶和枕叶的FDG摄取指数均低于PD-NC组(P均＜0.05).结论 PD合并轻度认知障碍与运动障碍和抑郁等因素相关.脑皮层广泛代谢障碍可能是其病理生理基础.     

路易体痴呆临床研究进展

路易体痴呆(dementia with Lewy bodies,DLB)是一种神经变性性痴呆,以细胞内路易体(Lewy bodies,LB)沉积为病理特点,临床表现为波动性认知功能障碍、视幻觉和帕金森综合征.这一疾病由Lewy等[1]于1912年首次报道.由于帕金森病(Parkinson disease,PD)患者晚期...     

路易氏体痴呆的神经电生理和临床特点

目的探讨路易氏体痴呆(DLB)的临床和神经电生理特点。方法综合分析3例DLB患者的脑电图(EEG)、P300、视觉诱发电位(VEP)和临床表现。结果DLB的EEG特点为:早期出现优势节律慢化;慢波活动增加;阵发性慢波活动。P300潜伏期延长。VEP无特征性改变。波动性认知功能减退,生动的视幻觉,僵直性帕金森症状为DLB的主要临床特点。结论早期出现的EEG优势节律慢化和阵发性慢波活动有助于DLB的临床诊断。EEG动态观察,可能会获得更多有价值的EEG信息。     

2005年路易体痴呆诊断标准与2017年新版诊断标准的临床比较分析

目的：研究2017年新版路易体痴呆（dementia with Lewy bodies,DLB）指南的诊断要点、治疗、预后等,以期提高医师对DLB的关注和认识,使更多的DLB患者得到早期诊断、全程管理及综合治疗。方法：选取30例符合2005年DLB诊断标准的很可能的DLB患者,回顾性分析其临床特点、治疗等,并用2017年新版DLB诊断标准对其进行诊断。结果：根据2017年新版DLB诊断标准,30例患者中有27例被诊断为很可能的DLB,3例被诊断为可能的DLB;其中伴有帕金森症患者29例（96.7%）,波动性认知障碍的患者有16例（53.3%）;视幻觉患者25例（83.3%）;伴快速眼动期睡眠行为障碍（rapid eye movement sleep behavior disorder,RBD）症状患者11例（36.7%）。21例患者（70%）以帕金森症起病,有9例患者（30%）以认知障碍伴视幻觉起病。结论：2017新版诊断标准增加了快速眼动期睡眠行为障碍（多导睡眠图异常）及¹²³I-间位碘代苄胍心肌闪烁扫描成像异常对于诊断路易体的权重,为早期识别和诊断路易体痴呆提供客观依据,有利于患者得到及时的管理及综合治疗,从而改善其预后。     

路易氏体痴呆的神经电生理和临床特点

目的 探讨路易氏体痴呆（DLB）的临床和神经电生理特点。方法 综合分析3例DLB患的脑电图（EEG），P300，视觉诱发电位（VEP）和临床表现。结果 DLB的EEG特点为：早期出现优势节律慢化；慢波活动增加；阵发性慢波活动。P300潜伏期延长VEP无特征性改变。波动性认知功能减退，生动的视幻觉，僵直性帕金森症状为DLB的主要临床特点。结论 早期出现的EEG优势节律慢化和阵发性慢波活动有助于DLB的临床诊断。EEG动态观察，可能会获得更多有价值的EEG信息。     

帕金森病痴呆患者的脑电活动研究

目的：通过比较帕金森病（PD）患痴呆组与非痴呆组的脑电图特点，探讨PD患痴呆与脑电活动的关系。方法：将经临床确诊的PD患分为痴呆组和非痴呆组，分别作脑电图（EEG）描记，结果：痴呆组EEG异常率明显高于非痴呆组（P＜0．01）；痴呆程度与EEG异常程度有关，痴呆组EEGα波低波幅，低波率，低指数及有泛化均显高于对照组（P＜0．05或P＜0．01），痴呆组异常EEG快波（β）与慢波（θ，δ）出现率均高于对照组，但以θ，δ波为（P＜0．01或P＜0．05）。结论：EEG可用以帮助判断PD痴呆的严重程度，并可作为评价PD智能状况的一个参考指标。     

早发型与晚发型帕金森病多巴胺能神经元和脑葡萄糖代谢变化差异

目的 观察早发型帕金森病(EOPD)与晚发型帕金森病(LOPD)多巴胺神经元和脑葡萄糖代谢变化差异。方法 前瞻性纳入45例EOPD患者(EOPD组)、55例LOPD患者(LOPD组)及93名健康对照(HC)者[HC1组(n=47),HC2组(n=46)],行¹¹C-β-CFT 和¹⁸F-FDG PET显像;观察EOPD组与HC1组、LOPD组与HC2组脑¹¹C-β-CFT标准摄取值比值(SUVR)和¹⁸F-FDG葡萄糖代谢网络模式,以回归分析评估脑区¹¹C-β-CFT SUVR与¹⁸F-FDG葡萄糖代谢的关系。结果 EOPD组与HC1组之间,尾状核、壳核和苍白球¹¹C-β-CFT SUVR差异均有统计学意义(P均＜0.05);LOPD组与HC2组之间,伏隔核、尾状核、壳核和苍白球¹¹C-β-CFT SUVR差异均有统计学意义(P均＜0.05)。与HC1组相比,EOPD组双侧尾状核、壳核、苍白球、岛叶、小脑、颞叶、丘脑及额叶葡萄糖代谢明显增高(P均＜0.05);相比HC2组,LOPD组双侧壳核、苍白球、中央旁回、小脑、颞叶及丘脑葡萄糖代谢明显增高(P均<0.05),双侧尾状核和额叶葡萄糖代谢明显减低(P均＜0.05)。尾状核代谢为EOPD组伏隔核¹¹C-β-CFT SUVR减低的影响因素(P＜0.001);尾状核、前额叶、小脑及丘脑代谢均为LOPD组伏隔核¹¹C-β-CFT SUVR减低的影响因素(P均＜0.001)。结论 LOPD患者伏隔核多巴胺神经元受损、尾状核和额叶葡萄糖代谢明显减低,而EOPD患者未见上述表现。     

Lewy小体型痴呆研究进展

Lewy小体型痴呆是一种具有临床和病理特征的神经精神疾病。现就本病的研究进展作一综述。     

The First Confirmed Case of Down Syndrome with Dementia with Lewy Bodies

Dementia with Lewy bodies (DLB) is the second commonest cause of dementia in the general population. Several researches have established an association between Down syndrome (DS) and Alzheimer’s disease. Very few studies have however showed such an association between dementia with Lewy bodies and Down syndrome. The occurrence of DLB in persons with DS is widely unrecognized. We report the first case of a person who fulfils the operational criteria for DLB and was also found to have Lewy bodies on neuropathological examination. It is important to make an early and accurate diagnosis as patients with DLB may respond differently than Alzheimer’s dementia patients to certain behavioural and medical treatments.     

Dementia with Lewy bodies

DLB is a complex disorder with important associations with PD and AD. As clinicians, it is important for us to identify these patients because of their unique responses to medical interventions and to help patients and caregivers more fully understand this disease process and its implications. Further research is needed to improve our understanding of the pathophysiology of this important dementing disorder, with the ultimate goal of improving clinical management of this disease.     

脑性瘫痪患儿脑细胞葡萄糖代谢与发育商关系的研究

目的：探讨脑瘫患儿脑细胞葡萄糖代谢与发育商（DQ）的关系。方法：符合脑瘫诊断标准的脑瘫患儿33例（脑瘫组），均采用Gesell发育量表（GDS）进行测试，同时进行18F-FDG脑显像检查（简称PET），并与健康自愿体检正常儿童（正常组）进行比较。结果：与正常组比较，脑瘫组中PET脑显像异常30例，正常3例；PET脑显像异常与动作能、应物能、应人能和言语能均有明显相关性；单灶低代谢灶其4个能区表现为轻度异常，多灶和弥漫低代谢灶则分别为中度和重度异常，且脑细胞葡萄糖代谢异常程度越重，DQ分值越低（P〈0．05或0．01）。结论：脑细胞葡萄糖代谢异常可影响小儿的DQ状况。GDS结合PET脑显像检查可了解患者脑葡萄糖代谢功能和细胞活动功能，客观反映脑细胞损伤部位及程度，对于脑瘫患儿DQ状况的评估以及指导临床治疗具有重要意义。     

糖代谢异常与阿尔茨海默病的关系

阿尔茨海默病(Alzheimer′s disease,AD)是一种中枢神经退行性疾病,是老年人最常见的痴呆类型。本文就AD与糖代谢之间的关系做一简单的综述。1 AD概述及其流行病学调查AD以进行性认知行为全面减退为临床特征,AD患者在早期出现渐进性的记忆障碍,尤其是近     

Functional Imaging of Cerebral Blood Flow and Glucose Metabolism in Parkinson’s Disease and Huntington’s Disease

Brain imaging of cerebral blood flow and glucose metabolism has been playing key roles in describing pathophysiology of Parkinson’s disease (PD) and Huntington’s disease (HD), respectively. Many biomarkers have been developed in recent years to investigate the abnormality in molecular substrate, track the time course of disease progression, and evaluate the efficacy of novel experimental therapeutics. A growing body of literature has emerged on neurobiology of these two movement disorders in resting states and in response to brain activation tasks. In this paper, we review the latest applications of these approaches in patients and normal volunteers at rest conditions. The discussions focus on brain mapping studies with univariate and multivariate statistical analyses on a voxel basis. In particular, we present data to validate the reproducibility and reliability of unique spatial covariance patterns related with PD and HD.     

Differentiating Alzheimer Disease,Lewy Body,and Parkinson Dementia Using DSM-5

Dementia is a growing problem with global, social, and financial repercussions. Early screening, diagnosis, education, planning, and treatment of dementia in primary care may reduce this burden. Presently, dementia is often diagnosed relatively late in the disease process, resulting in greater financial and caregiver burden. Primary care providers are often most likely to encounter patients with cognitive impairment early. The new guidelines outlined in the Diagnostic and Statistical Manual of Mental Disorders, Fifth Edition are presented to promote annual screening and early diagnosis of mild cognitive deficits by primary care providers.     

Glucose and Alanine Metabolism in Children with Maple Syrup Urine Disease

In vitro studies have suggested that catabolism of branched chain amino acids is linked with alanine and glutamine formed in, and released from, muscle. To explore this possibility in vivo, static and kinetic studies were performed in three patients with classical, and one patient with partial, branched chain α-ketoacid decarboxylase deficiency (maple syrup urine disease, MSUD) and compared to similar studies in eight age-matched controls. The subjects underwent a 24-30-h fast, and a glucose-alanine flux study using stable isotopes. Basal plasma leucine concentrations were elevated (P <0.001) in patients with MSUD (1,140±125 μM vs. 155±18 μM in controls); and in contrast to the controls, branched chain amino acid concentrations in plasma increased during the fast in the MSUD patients. Basal plasma alanine concentrations were lower (P <0.01) in patients with classical MSUD (153±8 μM vs. 495±27 μM in controls). This discrepancy was maintained throughout the fast despite a decrease in alanine concentrations in both groups. Plasma alanine and leucine concentrations in the patient with partial MSUD were intermediate between those of the controls and the subjects with the classical form of the disease. Circulating ketone bodies and glucoregulatory hormones concentrations were similar in the MSUD and normal subjects during the fast.     

Dementia with Lewy bodies: diagnosis and clinical approach

Dementia with Lewy bodies (DLB) is a common form of dementia that is being increasingly recognized. This paper reviews this disorder, emphasizing how it is different from other causes of dementia.