Table 1 Distribution of neuronal loss, tauopathy, and macrophage accumulation in SCA34
Region | Neuronal loss | Neurofibrillary tangles and pretangles by AT8 | Glial fibrillary tangles by AT8 | Macrophage accumulation | Characteristic findings, mainly based on anti-three (3R) and four (4R) tau staining |
|---|---|---|---|---|---|
Frontal cortex | − |  ±  |  +  | − | Infrequent glial fibrillary tangles (GFTs) resembling tuft-shaped astrocytes (4R-tau positive). 4R-tau positive lesions (neuronal/glial) were more frequent than 3R (4R > 3R) as a whole |
Primary motor cortex | − |  ±  |  +  | − | 4R > 3R. Rare GFTs resembling tuft-shaped astrocytes (4R-tau positive) |
Temporal cortex (tip) | − |  +  |  +  | − | 3R- and 4R-tau positive NFTs were both comparably observed (4R = 3R). 4R-tau positive astrocytes in subependymal regions of the lateral ventricule |
Parietal cortex | − |  ±  |  ±  | − | 4R > 3R |
Posterior cortex (visual cortex) | − | − |  ±  | − |  |
Caudate | − | − |  ±  | − |  |
Putamen | − |  ±  |  +  | − | GFTs resembling tuft-shaped astrocytes. 4R-tau positivity was much greater than 3R (4R >  > 3R) |
Globus pallidus | − | − |  ±  | − | 4R >  > 3R |
Subthalamic nucleus |  ±  |  +  |  +  | − | 4R >  > 3R |
Amygdala | − |  +  |  +  | − | 4R = 3R |
Parahippocampal cortex | − |  +  | − | − | 4R = 3R |
Hippocampus CA1/subiculum | − |  +  | − | − | 4R = 3R |
Hippocampus CA2-4 | − |  +  | − | − | 4R = 3R |
Dentate gyrus | − |  ±  | − | − |  |
Hypothalamus | − |  ±  |  ±  | − |  |
Thalamus | − |  ±  |  ±  | − |  |
Substantia nigra PR | − |  ±  | − | − |  |
Substantia nigra PC |  ±  |  +  |  ±  | − | 4R >  > 3R |
Midbrain tegmentum | − |  ±  |  ±  | − | 4R >  > 3R |
Pontine nucleus |  ++  | − | − |  ++  | Marked macrophage accumulation in the pontine base |
Locus ceruleus |  ±  |  +  | − | − | 4R = 3R |
Cerebellar cortex |  +  | − | − | − | Purkinje cells were moderately decreased |
Cerebellar nucleus (dentate) | − |  ±  |  ±  | − | 4R > 3R |
Inferior olive | − | − |  ±  | − |  |
Dorsal column nuclei |  ±  | − | − | − |  |
Spinal cord anterior horn | − | − |  ±  | − |  |
Spinal cord dorsal horn | − | − |  ±  | − |  |