Fig. 1
frontotemporal atrophy was observed based on the macroscopic pathological examination. The clinical evolution involved the development of atypical Parkinsonism. Case 4 had large FUS-positive neuronal inclusions and FUS-positive threads (D: right frontal lobe), developed severe global atrophy at a clinical picture of becoming mutistic and bedridden in 4 years after diagnosis. Lastly, case 5 showed long dystrophic neurites characteristic for FTD-TDP-C (E: insular cortex) and developed bilateral temporal atrophy at the end stage of the disease, based on the pathological examination. This patient’s clinical features were relatively benign, presenting with verbal and non-verbal semantic impairment and without the development of any motor disturbances and a disease duration of 12 years. Scalebar is 100 μm, scalebar insert is 10 μm
Different pathological diagnoses in donors with rtvFTD. The cases with rtvFTD displayed pathology from different pathological molecular subclasses in FTD. Although all pathological accumulations started from the right temporal lobe, according to the initial MRI atrophy pattern, over time the patients exhibited heterogeneous progression patterns. Case 1 showed FTD-TDP-B pathology with predominant neuronal inclusions throughout the cortical layers in right temporal lobe (a). Clinically, motor neuron disease developed over the disease course. Case 2 showed FTD-TDP-E pathology characterised by granulofilamentous neuronal inclusions (insert) and grains in right temporal lobe (b). The pathology spread to bilateral fronto-temporal areas. Clinically, this was accompanied by severe behavioural and language problems. Case 3 had tau-pathology with threads and tangles and some plaques (Anterior cingulate cortex: C, adapted from Ulugut Erkoyun et al.,2021, JAD, CC BY-NC 4.0). At the end stage of the disease, right predominant