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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.