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 |
Scores: −, absent; +, mild; ++, moderate; +++, severe; ++++, status spongiosus. ARP defined according to Montine et al. 2012 [17] (A for amyloid-phase 0–3; B for Braak neurofibrillary stage 0–3; C for CERAD neuritic plaque score 0–3)
SP spongiform change, PrP pattern of prion protein deposition, syn synaptic, AgD argyrophilic grain disease (according to Saito et al. [28], ARP Alzheimer-related pathology, CAA cerebral amyloid angiopathy, LB Lewy body, staging according to Attems et al. [2], PART primary age-related tauopathy (according to Crary et al. [9])
*Focal