Figure 3.
EWS pathology in FTLD-FUS. EWS immunohistochemistry performed on sections of post-mortem brain tissue from normal control (A), atypical FTLD-U (B–D, G), NIFID (E) and BIBD (F and H). Normal physiological staining pattern of nuclei and diffuse cytoplasmic labelling (A). In atypical FTLD-U, round cytoplasmic and intranuclear inclusions were observed in the dentate granule cells with variable labelling intensity (B). Higher magnification of cytoplasmic (C) and vermiform intranuclear inclusion (D) in atypical FTLD-U. Numerous neuronal cytoplasmic inclusions with variable morphology including round, crescentic, globular and tangle-like showed strong immunoreactivity in NIFID (E) and BIBD (F) as shown here in frontal cortex. Most cases with FTLD-FUS revealed at least rare inclusions in lower motor neurons (G) as well as variable numbers of glial cytoplasmic inclusions in the white matter of affected brain regions (H). Scale bar: A, B, E and F = 25 µm; C, D and H = 5 µm; G = 10 µm.