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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
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Acta Neuropathologica Communications

ISSN: 2051-5960

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