Table 3.

Autopsy findings from SCA psychosis study cohort (n=4)

Subject	SCA type	CAG repeat size	Age at death (years)	Cerebellar findings	Extra-cerebellar: cortical, brainstem, basal ganglia, and nigral findings
1	SCA 7	48	49	Cerebellum: grossly atrophic, marked patchy loss of Purkinje cells posterior-superior greater than anterior-inferior. Mild gliosis of deep cerebellar white matter tracts. Ubiquitin-positive intranuclear inclusions. Neuronal loss in dentate nuclei.	Substantia nigra: moderate pallor grossly and loss of pigmented neurons microscopically, without Lewy bodies. Normal locus ceruleus Note of grossly atrophy brainstem as well as gliosis and neuronal loss in thalamus, pons, and medulla. Normal striatum Cortex: moderate frontal, parietal and temporal lobe atrophy. Braak stage 1
2	SCA 7	50	48	Cerebellum: Moderate vermian atrophy grossly with marked atrophy and astrocytic gliosis of white matter tracts of cerebellar peduncles and basis pontis crossing fibers. Dentate nuclei showed severe neuronal loss and pronounced gliosis. Moderate Purkinje cell loss with anterior vermis most affected. Numerous empty basket cells.	Substantia nigra: moderate pallor grossly and neuronal loss microscopically, without Lewy bodies. Normal locus ceruleus. Inferior olivary nuclei: small grossly with severe neuronal loss. Normal striatum
7	SCA 17	53	68	Cerebellum: grossly small medially more so than laterally. Severe Purkinje cell loss.	Cortex: Moderate atrophy involving frontal, temporal, and parietal lobes. Basal ganglia: Severe atrophy of caudate nuclei and less severe putamen atrophy Inferior olivary nuclei: normal appearing
8	SCA 2	37	58	Cerebellum: atrophy grossly and neuronal loss microscopically, diffuse Purkinje cell loss with empty baskets and reactive proliferation of Bergmann astrocytes. Mild granule cell loss. Dentate nucleus with areas of gliosis and ischemic neurons without obvious cell loss.	Substantia nigra: normal appearing Substantia nigra: neuronal loss, gliosis, and macrophage infiltration without Lewy bodies. Normal striatum and thalamus Inferior olivary nuclei: atrophy grossly and marked neuronal loss Cortex: Some Alzheimer changes with Braak stage 3