Table 2.
Histopathological findings in the brains and amplification with cell-PMCA of patients with FFI and sFI
| Case number | PRNP genotype | Histopathology |
Amplification with cell-PMCA b | Reference | |
|---|---|---|---|---|---|
| OD [number of neurons in the olivary nucleus/mm2 (mean ± SEM)] a | Spongiform changes | ||||
| FFI-Mother | 129M/M 219E/E D178N | Negative (55.1 ± 15.9) | +++ | Negative | Saitoh et al. (2010) |
| FFI-Child | Positive (11.8 ± 2.8) | − | Positive | Saitoh et al. (2010) | |
| FFI-I37 | Positive (4.5 ± 6.5) | + | Positive | ||
| FFI-I75 | Positive (0.3 ± 0.8) | + | Positive | ||
| sFI-H91 | Positive (2.2 ± 1.2) | + | Positive | Kawasaki et al. (1997) | |
| sFI-I110 | 129M/M 219E/E | Positive (1.2 ± 1.1) | + | Positive | |
| sFI-T00/06 | Positive (7.0 ± 7.3) | ++ | Positive | Yamashita et al. (2001) | |
| sFI-I48 | Positive (0.6 ± 0.8) | +++ | Positive | Hirose et al. (2006) | |
| sFI-J86 | Positive (5.0 ± 3.9) | − | Positive | ||
| sFI-H10 | Positive (0) | + | Positive | ||
| sFI-H94 | Positive (1.2 ± 1.2) | + | Positive | ||
| sFI-J93 | Positive (2.7 ± 3.1) | + | Positive | ||
| sFI-I43 | Positive (1.8 ± 2.0) | +++ | Positive | ||
| sFI-J99 | Positive (6.8 ± 3.1) | − | Positive | ||
−, absent; +, spotty (lesion is usually within 1 mm2); ++, partial (lesion spreads beyond gyrus or involves all cortical layers); +++, widespread (lesion spreads beyond one lobe).
a
The average and standard deviation of the number of neurons at each of 5 places/mm2 in the inferior olive.
b
The cases were described as positive if the brain samples with 10−3-fold dilution could be amplified using this PMCA method.