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Table 1 Distribution of neuronal loss, tauopathy, and macrophage accumulation in SCA34

From: Neuropathology of SCA34 showing widespread oligodendroglial pathology with vacuolar white matter degeneration: a case study

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  ±   
  1.  − , 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