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Table 4 Regional distribution of filamentous PrP in a brain with A117V mutation (Case #17): semiquantitative scoring of white matter PrP filaments

From: Filamentous white matter prion protein deposition is a distinctive feature of multiple inherited prion diseases

Case #17: areas analysed Density of filamentous PrP in white matter tracts in the region
Anterior frontal F1-F2 +++
Posterior frontal Superior frontal gyrus and pre motor cortex ++
Temporal T1-T2 +++
Temporal T2-T3 +++
Tip of temporal lobe +++
Parietal ++
Occipital ++
Anterior cingulate +++
Hippocampus +++
Amygdala +++
Basal ganglia, ant. +++
Basal ganglia, mid. +++
Basal ganglia, post ++
Thalamus with subthalamic nucleus +
Upper midbrain +/++
Lower midbrain +
Pons +
Lower medulla n.a.
Cerebellum Vermis +
Cerebellum with dentate -
Cerebellum hemisphere (+)
  1. The graphical representation is shown in Figure 7.