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. Author manuscript; available in PMC: 2022 Dec 29.
Published in final edited form as: Curr Opin Neurobiol. 2022 Jul 2;76:102599. doi: 10.1016/j.conb.2022.102599

Figure 1. Neuropathology and autoimmune dysfunction in FTLD patients.

Figure 1

(a) Gross neuropathology of FTLD caused by GRN mutations (upper panel). Microscopic features of TDP-43 proteinopathy in FTLD that define type A, B and C. Although TDP-43 is located inside of nuclei in normal neurons, aggregates of TDP-43 are detected in the frontal cortex of FTLD-TDP patients. Based on the morphology of TDP-43 proteinopathy, FTLD-TDP can be classified into type A, which shows compact aggregation of TDP-43 in neuronal cytoplasm and TDP-43 thread-like inclusions in the neuropils or neurites in layers 2/3, type B, which shows diffuse cytoplasmic granular TDP-43 aggregates in layers 2–5, and type C, which contains long tortuous TDP-43-containing neurites and cytoplasmic TDP-43 aggregates in layer 2/3 in the frontal cortex (b) Case-control studies show a higher propensity for FTLD-TDP patients to have co-occurrence of systemic autoimmune diseases. Pie charts are modified based on the data previously published [12,13].