Neurology Service, Hospital Clínic of Barcelona, Barcelona, Spain.
Mov Disord. 2011 Jun;26(7):1316-23. doi: 10.1002/mds.23506. Epub 2011 Apr 11.
Lewy body syndromes (mainly Parkinson's disease and dementia with Lewy bodies) share many clinical features and usually have a slowly progressive course. Some patients may show rapid symptoms progression.
To evaluate clinical and neuropathological features of patients with a rapidly progressive diffuse Lewy Body disease.
Review clinical records and pathological findings of 6 cases with diffuse Lewy Body disease and rapid disease progression (less than 18 months before death).
Mean age at disease onset was 72.5 years, and mean disease duration was 9 months. Onset consisted of delirium in 3 patients and rapidly progressive dementia in the other three. All cases presented visual hallucinations and delusions; cognitive symptoms were fluctuating in two, parkinsonism occurred in four, and myoclonus in three. Brain MRI did not show cortical or basal ganglia hyperintensities. Periodic sharp waves were absent on EEG. 14.3.3 protein in CSF was negative. Myocardial (123) I-metaiodo-benzyl-guanidine SPECT showed marked reduction in tracer uptake in the 2 patients tested. Neuropathological studies did not identify any particular feature that could differentiate rapidly progressive diffuse Lewy body disease from classical diffuse Lewy body disease.
Diffuse Lewy body disease is a possible cause of rapidly progressive dementia and should be included in the differential diagnosis of confusional states of undetermined cause. In patients with rapidly progressive dementia, the presence of fluctuating cognition, parkinsonism, hallucinations, delusions, or severe dysautonomia, should raise the suspicion of diffuse Lewy body disease. Neuroimaging studies such as (123) I-metaiodo-benzyl-guanidine myocardial scintigraphy may support the clinical diagnosis of diffuse Lewy body disease. © 2011 Movement Disorder Society.
路易体综合征（主要为帕金森病和路易体痴呆）具有许多临床特征，且通常呈缓慢进展过程。部分患者可能表现为症状迅速进展。
评估快速进展性弥漫性路易体病患者的临床和神经病理学特征。
回顾性分析 6 例快速进展性弥漫性路易体病（起病至死亡时间小于 18 个月）患者的临床记录和病理检查结果。
发病年龄平均为 72.5 岁，平均病程为 9 个月。3 例以谵妄起病，另 3 例以快速进展性痴呆起病。所有患者均有视幻觉和妄想；2 例认知症状波动，4 例有帕金森病，3 例有肌阵挛。脑 MRI 未见皮质或基底节高信号。脑电图未见周期性尖波。4 例脑脊液 14.3.3 蛋白阴性。2 例行心肌（123）I-间碘苄胍 SPECT 检查，均显示示踪剂摄取明显减少。神经病理学研究未发现任何可将快速进展性弥漫性路易体病与经典弥漫性路易体病区分开来的特定特征。
弥漫性路易体病可能是快速进展性痴呆的病因之一，应列入原因不明的意识障碍的鉴别诊断。对于快速进展性痴呆患者，如果存在认知波动、帕金森病、幻觉、妄想或严重自主神经功能障碍，应考虑弥漫性路易体病。神经影像学检查，如（123）I-间碘苄胍心肌闪烁显像，可能有助于弥漫性路易体病的临床诊断。 © 2011 运动障碍学会。
