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. 2020 Mar 23;6(3):e417. doi: 10.1212/NXG.0000000000000417

Figure 2. Histopathologic features of affected cases III-13 (A–H) and III-10 (I and J).

Figure 2

(A and B) Nearly complete loss of Purkinje cells, loss of neurons, and spongiosis in the cerebellar granular layer of the patient (A) compared with the control brain (B) in H/E staining; (C and D) NII in the cerebellar granular layer with ubiquitin (C) and p62 staining (D); (E) NII in the occipital cortex with p62 staining; (F) DNS in the occipital cortex with 1C2 staining; (G) DNS in the inferior olive with 1C2 staining; (H) granular CI in the hypoglossal nucleus with 1C2 staining; (I) AT8-positive neurons, neurites, and glial staining in the thalamic nuclei; (J) AT8-positive tufted astrocyte in the putamen. The arrowheads indicate NII, which are magnified in figure 2C-F. CI = cytoplasmic inclusion; DNS = diffuse nuclear staining; H/E = hematoxylin and eosin staining; NII = neuronal intranuclear inclusion.