Fig. 1

a: Sagittal view of a T1-weighted brain MR image showing mild atrophy of the upper vermis of the cerebellum. b,c: An axial MRI (fluid-attenuated inversion recovery image) showed mild atrophy in the upper vermis of the cerebellum and the right dominant anterior medial temporal lobe (b), and moderate atrophy of the frontotemporal cortex (c). d: Synaptophysin staining shows an eosinophilic halo-like structure (arrow) around a degenerated Purkinje cell in the right cerebellar hemisphere. e: Gallyas-positive, rod-like (arrowheads) or granular argyrophilic grains in the subiculum of the left hippocampus