Table 2.
Quantitative neuropathological analysis of spongiosis, gliosis, and PrPSc deposits in all the examined nuclei according to the different genotypes. The main pattern of PrPSc deposits is also reported. Group 2 is the second part of the table.
| Case number | sCJD molecular type | DMNV | HN | PHN | IOC | ||||||||
|---|---|---|---|---|---|---|---|---|---|---|---|---|---|
| S | G | PrPSc | S | G | PrPSc | S | G | PrPSc | S | G | PrPSc | ||
| 1 | MM1 | ± | ± | − | ± | ± | − | − | ± | − | − | ± | − |
| 2 | MM1 | − | ± | − | − | − | − | − | − | − | − | ± | − |
| 3 | MM1 | + | + | + (syn.) | ± | + | + (syn.) | − | ± | + (syn.) | − | ± | + (syn.) |
| 4 | MM1 | ± | ± | − | + | + | − | + | + | − | − | − | − |
| 5 | MM1 | ± | − | − | − | − | − | − | − | + (syn.) | − | − | + (syn.) |
| 6 | MM1 | − | − | − | − | − | − | − | − | − | − | − | − |
| 7 | MM1 | ± | ± | − | ± | ± | − | ± | ± | − | − | ± | + (syn.) |
| 8 | MM1 | + | + | − | ± | ± | − | − | − | − | − | − | − |
| 9 | MM1 | + | ++ | − | + | ± | − | − | − | − | − | − | − |
| 10 | MM1 | ± | − | − | + | ± | − | − | ± | − | − | − | − |
| 11 | MM2 | + | ± | − | − | − | − | − | − | − | − | − | − |
| 12 | MM2 | ± | ± | − | ± | ± | − | − | − | − | − | − | − |
|
| |||||||||||||
| 13 | MV1 | ++ | ++ | ± | + | + | − | ± | ± | ± (syn.) | − | ± | + (syn.) |
| 14 | MV2 | + | + | + (syn.) | ± | + | + (syn.) | ± | + | + (syn.) | − | ± | + (syn.) |
| 15 | MV2 | ± | ± | + (syn.) | ± | ± | + (syn.) | ± | ± | + (syn.) | − | + | + (syn.) |
| 16 | MV2 | − | − | + (syn.) | + | ± | + (gran., pl.-like) | + | + | + (syn.) | − | ± | + (syn.) |
| 17 | VV2 | ± | ± | + (syn., periaxo.) | ± | ± | + (syn., periaxo.) | ± | ± | + (syn., periaxo.) | − | ± | + (syn., periaxo.) |
| 18 | VV2 | ± | ++ | ± | ± | + | ± | + | + | + | − | + | + |
| 19 | VV2 | ± | ± | + | − | − | ± | − | − | + | − | ± | + |
| 20 | VV2 | ± | ± | + (syn.) | − | ± | − | − | ± | + (syn.) | − | ± | + (syn.) |
| 21 | VV2 | ± | + | + (syn., gran., periaxo.) | ± | + | − | ± | + | + (syn., gran., periaxo.) | − | ± | + (syn.) |
DMNV: dorsal motor nucleus of the vagus; HN: hypoglossal nucleus; PHN: perihypoglossal nuclei; IOC: inferior olivary complex; S: spongiosis; G: gliosis; PrPSc: prion protein;syn.: synaptic; gran.: granular; periaxo.: periaxonal; pl.-like: plaque-like; −: negative; ±: mild; +: moderate; ++: severe.