Table 1.
Code | Age of onset |
Disease duration (months) |
Gender |
PRNP genotype (mutation- 129codon) |
PrPSc subtype | Symptoms at disease onset |
Family history of prion disease |
---|---|---|---|---|---|---|---|
1 | 49 | 120 | M | F198S-129MV | GSS, 8 kDa | Cerebellar | Brother and niece |
2 | 51 | 72 | M | F198S-129VV | GSS, 8 kDa | Cerebellar | Brother and daughter |
3 | 60 | 89 | M | F198S-129MV | GSS, 8 kDa | Not reported | Father |
4 | 67 | 63 | M | F198S-129MV | GSS, 8 kDa | Not reported | Not reported |
5 | 22 | 23 | M | P102L-129MV | fCJD, Type 1 | Seizures | Cousin |
6 | 60 | 20 | M | 129MV | sCJD, Type intermediate+ 2 | Visuospatial | Not reported |
7 | 55 | 6 | F | 129VV | sCJD, Type 2 | Apraxia | Not reported |
8 | 55 | 24 | F | 129MV | sCJD, Type intermediate+ 2 | Behavior | Not reported |
9 | 69 | 6 | F | 129MV | sCJD, Type 1 | Behavior/Memory | Not reported |
10 | 60 | 1.5 | M | 129MM | sCJD, Type 1 | Cognitive/Visual | Not reported |
11 | 57 | 4 | F | 129MM | sCJD, Type 1 | Motor | Not reported |