Fig. 1

Type and spreading of HR23B pathology found in C9FTD cases. Different types of HR23B pathology in C9FTD cases: a) neuropils and puncta in frontal cortex layer 2. b) intranuclear (cat eye) inclusion in hippocampus dentate gyrus. c) perinuclear inclusion in hippocampus dentate gyrus. d) round intranuclear inclusion in hippocampus dentate gyrus. e) round or oval inclusion with a hole in frontal cortex f) dystrophic neuron in cerebellum molecular layer. g) Spreading of HR23B compared to known p62 and pTDP-43 pathology. Depicted are semi-quantitive measures of neurodegeneration and pathological score in C9FTD. Neuronal loss score was based on hematoxylin and eosin (HE) staining and pathological report and scored as absent (0), mild (1), moderate (2) or severe (3). Pathological scores were based on the degree of pathology as absent (0), rare (1), occasional (2), moderate (3), or numerous (4). See also Additional file 9: Table S2 for details of pathological quantifications. All scale bars are 20μm