Duignan John A, Haughey Aoife, Kinsella Justin A, Killeen Ronan P
Department of Radiology, St Vincent's University Hospital, Dublin 4, Ireland; UCD - SVUH PET CT Research Centre, St Vincent's University Hospital, Dublin 4, Ireland.
Department of Neurology, St Vincent's University Hospital, UCD, Dublin 4, Ireland.
Semin Nucl Med. 2021 May;51(3):264-274. doi: 10.1053/j.semnuclmed.2020.12.002. Epub 2021 Jan 2.
Dementia with Lewy bodies (DLB) and frontotemporal lobar degeneration (FTLD) are common causes of dementia. Early diagnosis of both conditions is challenging due to clinical and radiological overlap with other forms of dementia, particularly Alzheimer's disease (AD). Structural and functional imaging combined can aid differential diagnosis and help to discriminate DLB or FTLD from other forms of dementia. Imaging of DLB involves the use of I-FP-CIT SPECT and I-metaiodobenzylguanidine (I-MIBG), both of which have an established role distinguishing DLB from AD. AD is also characterised by more pronounced atrophy of the medial temporal lobe structures when compared to DLB and these can be assessed at MR using the Medial Temporal Atrophy Scale. F-FDG-PET is used as a supportive biomarker for the diagnoses of DLB and can distinguish DLB from AD with high accuracy. Polysomnography and electroencephalography also have established roles in the diagnoses of DLB. FTLD is a heterogenous group of neurodegenerative disorders characterised pathologically by abnormally aggregated proteins. Clinical subtypes include behavioral variant FTD (bvFTD), primary progressive aphasia (PPA), which can be subdivided into semantic variant PPA (svPPA) or nonfluent agrammatic PPA (nfaPPA) and FTD associated with motor neuron disease (FTD-MND). Structural imaging is often the first step in making an image supported diagnoses of FTLD. Regional patterns of atrophy can be assessed on MR and graded according to the global cortical atrophy scale. FTLD is typically associated with atrophy of the frontal and temporal lobes. The patterns of atrophy are associated with the specific clinical subtypes, underlying neuropathology and genetic mutations although there is significant overlap. F-FDG-PET is useful for distinguishing FTLD from other forms of dementia and focal areas of hypometabolism can often precede atrophy identified on structural MR imaging. There are currently no biomarkers with which to unambiguously diagnose DLB or FTLD and both conditions demonstrate a wide range of heterogeneity. A combined approach of structural and functional imaging improves diagnostic accuracy in both conditions.
路易体痴呆(DLB)和额颞叶变性(FTLD)是痴呆的常见病因。由于这两种疾病在临床和影像学上与其他形式的痴呆,尤其是阿尔茨海默病(AD)存在重叠,早期诊断具有挑战性。结构和功能成像相结合有助于鉴别诊断,并有助于将DLB或FTLD与其他形式的痴呆区分开来。DLB的成像检查包括使用碘氟培司汀单光子发射计算机断层扫描(I-FP-CIT SPECT)和碘间位苄胍(I-MIBG),这两种检查在区分DLB和AD方面都有既定作用。与DLB相比,AD的特征还在于内侧颞叶结构萎缩更为明显,可使用内侧颞叶萎缩量表在磁共振成像(MR)上进行评估。氟代脱氧葡萄糖正电子发射断层显像(F-FDG-PET)用作DLB诊断的辅助生物标志物,能够高精度地区分DLB和AD。多导睡眠图和脑电图在DLB诊断中也有既定作用。FTLD是一组异质性神经退行性疾病,其病理特征为蛋白质异常聚集。临床亚型包括行为变异型额颞叶痴呆(bvFTD)、原发性进行性失语(PPA),后者可再细分为语义变异型PPA(svPPA)或非流利性语法缺失型PPA(nfaPPA)以及与运动神经元病相关的额颞叶痴呆(FTD-MND)。结构成像通常是做出影像学支持的FTLD诊断的第一步。可在MR上评估萎缩的区域模式,并根据整体皮质萎缩量表进行分级。FTLD通常与额叶和颞叶萎缩相关。尽管存在显著重叠,但萎缩模式与特定临床亚型、潜在神经病理学和基因突变有关。F-FDG-PET有助于将FTLD与其他形式的痴呆区分开来,代谢减低的局灶区域通常先于结构MR成像发现的萎缩出现。目前尚无能够明确诊断DLB或FTLD的生物标志物,且这两种疾病均表现出广泛的异质性。结构和功能成像相结合的方法可提高这两种疾病的诊断准确性。