Table 2. Comparison of four 8 OPRI families.
| A family | Che family | M-E family | Swedish family | All the families | |
|---|---|---|---|---|---|
| n (females; males) | 6 (2;4) | 6 (1;5) | 11 (7; 4) | 7 (4;3) | 30 (14; 16) |
| PRNP polymorphic codon 129 in mutated allele (n) | 129V6 | 129V6 | 129M11 | 129M7 | 129V,12 129M18 |
| PRNP polymorphic codon 129 in both alleles (nr of homozygous and heterozygous) | 2 pol 129VV 1 pol 129 VM | NA | 4 pol 129MM | 4 pol 129 MM | 1 pol 129 VM 2 pol 129VV 8 pol 129 MM |
|
Age of onset, mean in years; SD (range; missing values) |
43.3; 12.4 (21–54; 0) | 43.3; 6.8 (35–54; 0) |
31.8; 7.0 (21–43; 1) | 29.7; 5.4 (23–41; 0) |
36.1; 6.6 (21-54; 1) |
|
Disease duration, mean in years; SD (range; number for which information is available; missing values; still alive at the time of publication) |
3.6; 2.6 (0.4–6a; 4; 0; 2) |
3.3; 4.9 (0.3–13; 6; 0; 0) |
3.4; 2.3 (1.3–7b; 6; 1; 4) |
15.1; 4.3 (11–23; 7; 0; 0) |
6.9; 6.6 (0.3–23; 23; 1; 6) |
|
Age of death, mean in years; SD (range; n) |
53.6; 3.9 (50.4–59; 4) |
46.6; 8.7 (35.3–56; 6) |
40.6; 4.8 (35-47;7) |
44.9; 4.0 (41–52; 7) |
45.5; 6.9 (35-59; 24) |
a Two subjects were still alive at the time of report publication more than 1 and two years after disease onset. bOne subject was still alive more than 12 y after symptom onset. Disease duration is the longest of all the reported IPD families with 8-OPRI (P < 0.001).