Molecular and Anatomical Imaging of Dementia With Lewy Bodies and Frontotemporal Lobar Degeneration

Abstract

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 ¹²³I-FP-CIT SPECT and ¹²³I-metaiodobenzylguanidine (¹²³I-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. ¹⁸F-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. ¹⁸F-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.