Frontotemporal dementia mimicking dementia with Lewy bodies
Frontotemporal dementia mimicking dementia with Lewy bodies
复制标题
DOI：
10.1097/wnn.0b013e3181864a09
复制
发表时间：
2008-09-01
影响因子：
1.4
通讯作者：
Josephs, Keith A.
中科院分区：
文献类型：
--
作者：
Claassen, Daniel O.;Parisi, Joseph E.;Josephs, Keith A.
背景：一些额颞叶痴呆（FTD）患者伴有锥体外系症状和精神病，可能同时符合FTD和路易体痴呆（DLB）的共识标准。临床病理研究有助于了解这些病例中潜在的神经退行性过程。目的：描述6例首次临床表现同时具有FTD和DLB诊断体征和症状的患者的临床和病理特征，其中2例已进行尸检，并与尸检证实的FTD和路易体病患者进行比较。结果：所有6例患者均符合发表的FTD和DLB诊断共识标准（5例可能，1例可能）。FTD的临床症状包括人格和行为改变，而提示DLB的临床症状包括帕金森病、波动认知、睡眠异常和幻觉。5例患者行单光子发射计算机断层扫描（Tc-99m）成像，显示不同程度的额叶灌注不足。磁共振、脑电图、肌电图对诊断FTD和DLB没有帮助。2例尸检病例的组织学检查符合TDP-43蛋白病的病理诊断；特别是额颞叶变性伴泛素免疫反应性改变，1型。这6例患者与两组尸检证实的FTD和路易体病患者有显著差异。结论：我们发现了一组新的FTD患者，其临床特征与DLB重叠，但似乎不同于典型的FTD和典型的路易体病。
Background: Some patients with frontotemporal dementia (FTD) have concomitant extrapyramidal symptoms and psychosis and may simultaneously meet consensus criteria for FTD and for dementia with Lewy bodies (DLB). Clinicopathologic studies are helpful ill understanding the underlying neuro-degenerative process in such cases.Objective: To describe clinical and pathologic features of 6 patients with signs and symptoms Suggestive of both a diagnosis of FTD and DLB at first clinical presentation, of which 2 patients have now undergone autopsy, and to compare them with autopsy-confirmed FTD and Lewy body disease patients.Results: All 6 patients met published consensus criteria for a diagnosis of both FTD and DLB (5 probable and I possible). Clinical symptoms of FTD included personality and behavioral changes, whereas those suggestive of DLB included Parkinsonism, fluctuating cognition, parasomnia, and hallucinations. Five patients underwent single photon emission computed tomography (Tc-99m) imaging, which showed varying degrees of frontal lobe hypoperfusion. Magnetic resonance imaging, electroencephalogram, and electromyogram were not helpful in differentiating FTD from DLB. Histologic examination of the 2 autopsy cases was consistent with it pathologic diagnosis of TDP-43 proteinopathy; specifically frontotemporal lobar degeneration With ubiquitin-only immunoreactive changes, type 1. There were significant differences between these 6 patients and the 2 groups of autopsy confirmed FTD and Lewy body disease patients.Conclusions: We have identified a novel group of FTD patients with clinical features that overlap with DLB, yet seem to be different from both typical FTD and typical Lewy body disease.