Table 2 Neuropathologic findings in the six atypical sCJD cases
Case | ||||||
|---|---|---|---|---|---|---|
#1 | #2 | #3 | #4 | #5 | #6 | |
Frontal | ||||||
SP | ++ | ++/+++ | +++ | +++/++++ | ++/+++ | ++ |
PrP | Faint syn | Faint syn | Faint syn | Faint syn | Faint syn | Faint syn |
Temporal | ||||||
SP | ++/+++ | ++/+++ | +++ | +++ | ++/+++ | ++/+++ |
PrP | Faint syn | Faint syn | Faint syn | Faint syn | Faint syn | Negative |
Parietal | ||||||
SP | ++ | ++ | ++/+++ | +++ | ++ | ++/+++ |
PrP | Faint syn | Faint syn | Faint syn | Faint syn | Faint syn | Faint syn |
Occipital | ||||||
SP | +++/++++ | +++ | +++/++++ | +++ | ++ | ++/+++ |
PrP | Faint syn, patchy* | Faint syn | Faint syn | Faint syn, patchy* | Faint syn | Faint syn |
Striatum | ||||||
SP | ++/+++ | ++ | ++ | +++ | + | + |
PrP | Faint syn | Faint syn | Faint syn | Faint syn | Faint syn | Faint syn |
Thalamus | ||||||
SP | + | + | + | + | + | + |
PrP | Negative | Faint syn | Faint syn | Faint syn | Negative | Negative |
Hippocampus | ||||||
SP | + | − | − | −/+ | − | − |
PrP | Negative | Negative | Negative | Negative | Negative | Negative |
Parahippocampus | ||||||
SP | +++/++++ | ++/+++ | +++/++++ | +++/++++ | ++/+++ | ++/+++ |
PrP | Faint syn | Faint syn | Faint syn | Faint syn | Faint syn | Faint syn |
Brainstem | ||||||
SP | −/+ | −/+ | −/+ | −/+ | +/++ | − |
PrP | Negative | Negative | Negative | Negative | Plaque-like | Negative |
Cerebellum | ||||||
SP | ++ | + | ++ | ++ | + | +/++ |
PrP | Syn, patchy* | Syn, patchy* | Syn, patchy* | Syn, patchy* | Syn, patchy* | Syn, patchy* |
Balloon neurons | Yes | Yes | Isolated | Yes | Isolated | No |
Copathologies | AgD III, ARP: A1,B1,C1 (“possible” PART) | AgD III, ARP: A2,B1,C2 | ARP: A1,B1,C1 (“possible” PART), mild CAA | LB limbic, mod-prominent CAA, “definite” PART (Braak II) | ARP: A2,B1,C0, mild CAA | ARP: A2,B1,C0 |