Fig. 2

Representative images of TDP-43 pathology subtypes in FTLD-TDP brains. (A) Immunohistochemistry with an anti-phosphorylated-TDP-43 antibody (pSer409/pSer410) shows numerous neuronal cytoplasmic inclusions, short dystrophic neurites, and neuronal intranuclear inclusion (inset) in Type A; diffuse granular neuronal cytoplasmic inclusions in Type B; and numerous thick and long dystrophic neurites in Type C, in the superficial layer of the midfrontal gyrus (upper panel). In the dentate gyrus (lower panel), Type A shows compact neuronal cytoplasmic inclusions; Type B shows diffuse granular neuronal cytoplasmic inclusions; and Type C shows Pick body-like neuronal cytoplasmic inclusion. Scale bar = 50 μm. (B) A summary of clinical, pathological, and genetic features of TDP-43 pathology subtypes. NCI: Neuronal cytoplasmic inclusion; NII: Neuronal intranuclear inclusion; DN: Dystrophic neurite; GCI: Glia cytoplasmic inclusion; DG: Dentate gyrus; FTD: Frontotemporal dementia; bvFTD: Behavioral variant frontotemporal dementia; PNFA: Progressive non-fluent aphasia; MND: Motor neuron disease; and SD: Semantic dementia