E-Table 6: Summary information on age at onset and duration of disease reported in a total of 140 studies of untreated human prion disease. Study ‘average’ is taken as the median value wherever presented. If the median was not available then the mean value was used. All figures rounded to nearest year or month. *Including studies not reporting mutation and those reporting a mix of mutations. Further details on individual studies can be found in E-Tables 1-5, presented in the Supplemental Data.

Age at onset (years) / Disease duration (months)
Median of study ‘average’ / Range / No pts / No studies / Median of study ‘average’ / Range / Nopts / No studies
SporadicCreutzfeldt Jakob Disease
sCJD / 63 / 15 - 94 / 3741 / 38 / 6 / 1- 130 / 5217 / 53
Iatrogenic Creutzfeldt Jakob Disease
hGH/hGN transmission / 26 / 10 - 45 / 172 / 4 / 9 / 2 - 30 / 162 / 4
DM transmission / 46 / 15 – 80 / 119 / 4 / 7 / 3 – 33 / 21 / 3
Variant Creutzfeldt Jakob Disease
vCJD / 28 / 12 – 74 / 165 / 2 / 14 / 6 – 39 / 159 / 2
Inherited prion disease
A117V / 41 / 20 - 64 / 29 / 4 / 38 / - / 4 / 2
D178N 129M / 47 / 7 - 76 / 114 / 19 / 12 / 2 - 276 / 106 / 19
D178N 129V / 48 / 26 – 56 / 28 / 4 / 15 / 5 – 51 / 28 / 4
E196K / 70 / - / 1 / 1 / 13 / - / 1 / 1
E200K / 59 / 35 – 84 / 141 / 6 / 6 / 1 – 96 / 233 / 7
E211Q / 71 / 42 – 81 / 6 / 2 / 12 / 3 – >18 / 6 / 2
F198S / 46 / 42 - 59 / 3 / 1 / - / - / - / -
H187R / 42 / 33 – 50 / 4 / 1 / 156 / 84–228 / 4 / 1
M232R / 64 / 55 – 70 / 3 / 1 / 20 / 4 – 24 / 3 / 1
P102L / 44 / 22 – 71 / 38 / 7 / 48 / 2 – 84 / 21 / 6
P102L& E219K (same allele) / 42 / 31 - 60 / 4 / 1 / 48 / - / 1 / 1
P105L / 45 / 38 - 48 / 5 / 1 / 84 / 84 -144 / 4 / 1
Q217R / 56 / 47 - 66 / 2 / 1 / 156 / 72 -240 / 2 / 1
R208H / 61 / - / 1 / 1 / 4 / - / 1 / 1
T183A / 44 / 37 – 49 / 12 / 1 / 48 / 24–108 / 10 / 1
V180I / 72 / 58 – 81 / 10 / 2 / 46 / - / 1 / 1
V203I / 77 / - / 3 / 1 / 3 / - / 3 / 1
V210I / 64 / - / 6 / 1 / 5 / - / 6 / 1
24bp 1 repeat (1 OPRI) / 69 / - / 2 / 1 / 3 / - / 2 / 1
48bp 2 repeats(2 OPRI) / 61 / - / 1 / 1 / 3 / - / 1 / 1
96bp 4 repeats (4 OPRI) / 82 / - / 1 / 1 / 4 / - / 1 / 1
120bp 5 repeats(5 OPRI) / 47 / 26 – 61 / 6 / 2 / 54 / 4 – 96 / 4 / 2
144bp 6 repeats(6 OPRI) / 36 / 22 – 53 / 67 / 4 / 91 / 12–252 / 46 / 4
168bp 7 repeats(7 OPRI) / 47 / 23 – 75 / 5 / 2 / 64 / 3 – 132 / 3 / 2
192bp 8 repeats(8 OPRI) / 40 / 21 - 54 / 22 / 4 / 33 / 3 - 156 / 12 / 4
240bp 10 repeats(10 OPRI) / 38 / 31 – 45 / 2 / 1 / 120 / 60–180 / 2 / 1
All inherited prion disease (iPD)* / 48 / 7 - 84 / 635 / 70 / 14 / 1 – 276 / 703 / 71