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 | − | − | ± | − |
− , negative; ± , trace; + , mild; ++ , severe. Tauopathy was evaluated by anti-phosphorylated tau (AT8) and anti-three-repeat and anti-four-repeat tau (3R and 4R, respectively) staining. Glial neurofibrillary tangles included those resembling tuft-shaped astrocytes, coiled bodies, threads, and other lesions which were difficult to classify