Neuropathological alterations in SCA37. Pathological lesions in cerebellar sections derived from two SCA37 cases: Patients IV:9 and IV:10 from the AT-901 pedigree. (A and B) Macroscopic imaging of cerebellum sections showed decreased volume with increased cerebellar grooves. (C) Neuronal loss and gliosis were identified confined to the inferior olive. (D) Conserved cerebellar foliage, extensive loss of Purkinje cells, and Bergmann’ gliosis in the Purkinje cell layer shown by haematoxylin and eosin staining. No changes in the granular layer were identified. (E) Nuclear lobulation is shown in two Purkinje cells. (F and G) Immunostaining of phosphorylated neurofilaments showed abundant empty baskets in the Purkinje cell layer. (H) Haematoxylin and eosin staining showed extensive neuronal loss in lobule VII of the vermis and (I) intense gliosis in the dentate nucleus. (J–L) Ubiquitin immunostaining revealed distinctive perisomatic granules in Purkinje cell bodies (arrows) and (M) leptomeningeal vessels. (N) Haematoxylin and eosin staining showed specific severe gliosis in the flocculus. Scale bars = 100 µm.