Background: Iodine- 123- labeled metaiodobenzylguanidine ( 123I- MIBG) myocardial scintigraphy has been used to evaluate cardiac sympathetic denervation in Lewy body disease (LBD) including Parkinson’s disease (PD) a...Background: Iodine- 123- labeled metaiodobenzylguanidine ( 123I- MIBG) myocardial scintigraphy has been used to evaluate cardiac sympathetic denervation in Lewy body disease (LBD) including Parkinson’s disease (PD) and dementia with Lewy bodies (DLB). Patients with LBD had marked reductions in cardiac MIBG accumulation, indicative of severe impairment of the cardiac sympathetic nervous systems. However, the differences in scintigraphy between DLB and PD have not been determined. Objective: To compare cardiac sympathetic function in early disease stage measured with 123I- MIBG scintigraphy between DLB and PD. Methods: 123I- MIBG myocardial scintigraphy was performed in 22 patients with early-stage DLB, 41 patients with early idiopathic PD and 15 normal control subjects who were matched for age and disease duration. The heart-to-mediastinum (H/M) ratio was calculated. Results: 123I- MIBG uptake of the myocardium was significantly lower in patients with early DLB than in controls. The mean value of H/M ratio in patients with DLB was significantly lower than those in patients with PD, independent of the Hoehn and Yahr stage. Conclusions: Our findings suggest that cardiac sympathetic function in DLB is severely impaired even in the early disease stage.展开更多
1912年,德国病理学家Frederick Henry Lewy在帕金森病患者大脑神经元中检出一种异常蛋白小体;1919年,前苏联病理学家Konstantin Tretiakoff将其命名为"路易小体";1961年,日本学者Okazaki证实痴呆的发生发展与皮质型路易小体相...1912年,德国病理学家Frederick Henry Lewy在帕金森病患者大脑神经元中检出一种异常蛋白小体;1919年,前苏联病理学家Konstantin Tretiakoff将其命名为"路易小体";1961年,日本学者Okazaki证实痴呆的发生发展与皮质型路易小体相关;1980年,Kenji Kosaka提出"路易体病"的概念;至1995年,首届国际路易体痴呆研讨会将弥漫性路易体病、路易小体型老年痴呆、阿尔茨海默病路易小体变异型和大脑型路易体病统一命名为"路易体痴呆",并于2003年对其诊断标准进行重新修订,2005年发表于Neurology后应用于临床实践。展开更多
目的总结路易体痴呆(dementia with Lewy bodies,DLB)的临床特征及神经影像学特点。方法收集2012年2月-2018年11月于新乡医学院第一附属医院诊治的26例“可能的”或“很可能的”DLB患者的临床资料进行回顾性分析。结果本组患者男19例,女...目的总结路易体痴呆(dementia with Lewy bodies,DLB)的临床特征及神经影像学特点。方法收集2012年2月-2018年11月于新乡医学院第一附属医院诊治的26例“可能的”或“很可能的”DLB患者的临床资料进行回顾性分析。结果本组患者男19例,女7例,平均发病年龄(66.73±7.58)岁。以认知症状为首发者占65%,帕金森样症状首发者占19%,38%的患者认知障碍波动明显,77%的患者出现帕金森样症状,62%的患者出现幻觉,50%的患者出现快速眼球运动期睡眠行为障碍,65%的患者伴随自主神经功能障碍。59%(13/22)患者的头部核磁检查显示海马结构无萎缩或轻度萎缩,7例患者的18F-FDG PET检查均表现为以双侧顶枕叶为著的脑代谢减低模式,2例患者的11C-CFT PET检查均表现为双侧基底节转运体摄取减低。结论DLB多数以认知症状起病者,帕金森样症状、幻觉、快速眼动期行为障碍和精神症状是其主要表现,神经影像学检查有助于DLB的临床诊断。展开更多
Primary progressive freezing gait (PPFG) is characterized by early gait freezing and a stereotyped progression. Of nine patients followed up for 6 to 16 years, two were diagnosed pathologically: pallidonigroluysian de...Primary progressive freezing gait (PPFG) is characterized by early gait freezing and a stereotyped progression. Of nine patients followed up for 6 to 16 years, two were diagnosed pathologically: pallidonigroluysian degeneration (PNLD) and diffuse Lewy body disease. Four others evolved clinically into progressive supranuclear palsy and corticobasal degeneration. PPFG is not a distinct disorder but a syndrome with diverse causes. Long-term follow-up (≥10 years) and postmortem are required for accurate diagnosis. PNLD may be the primary form of disease.展开更多
文摘Background: Iodine- 123- labeled metaiodobenzylguanidine ( 123I- MIBG) myocardial scintigraphy has been used to evaluate cardiac sympathetic denervation in Lewy body disease (LBD) including Parkinson’s disease (PD) and dementia with Lewy bodies (DLB). Patients with LBD had marked reductions in cardiac MIBG accumulation, indicative of severe impairment of the cardiac sympathetic nervous systems. However, the differences in scintigraphy between DLB and PD have not been determined. Objective: To compare cardiac sympathetic function in early disease stage measured with 123I- MIBG scintigraphy between DLB and PD. Methods: 123I- MIBG myocardial scintigraphy was performed in 22 patients with early-stage DLB, 41 patients with early idiopathic PD and 15 normal control subjects who were matched for age and disease duration. The heart-to-mediastinum (H/M) ratio was calculated. Results: 123I- MIBG uptake of the myocardium was significantly lower in patients with early DLB than in controls. The mean value of H/M ratio in patients with DLB was significantly lower than those in patients with PD, independent of the Hoehn and Yahr stage. Conclusions: Our findings suggest that cardiac sympathetic function in DLB is severely impaired even in the early disease stage.
文摘1912年,德国病理学家Frederick Henry Lewy在帕金森病患者大脑神经元中检出一种异常蛋白小体;1919年,前苏联病理学家Konstantin Tretiakoff将其命名为"路易小体";1961年,日本学者Okazaki证实痴呆的发生发展与皮质型路易小体相关;1980年,Kenji Kosaka提出"路易体病"的概念;至1995年,首届国际路易体痴呆研讨会将弥漫性路易体病、路易小体型老年痴呆、阿尔茨海默病路易小体变异型和大脑型路易体病统一命名为"路易体痴呆",并于2003年对其诊断标准进行重新修订,2005年发表于Neurology后应用于临床实践。
文摘Primary progressive freezing gait (PPFG) is characterized by early gait freezing and a stereotyped progression. Of nine patients followed up for 6 to 16 years, two were diagnosed pathologically: pallidonigroluysian degeneration (PNLD) and diffuse Lewy body disease. Four others evolved clinically into progressive supranuclear palsy and corticobasal degeneration. PPFG is not a distinct disorder but a syndrome with diverse causes. Long-term follow-up (≥10 years) and postmortem are required for accurate diagnosis. PNLD may be the primary form of disease.
