Table 1 The relevant characteristics of the patients with non-CJD and sCJD, gCJD and FFI

Number (n)

145

136

25

14

22

Gender (M/F)

71/74

72/64

16/9

7/7

9/13

Median age at onset (range) (y)

61 (45–89)

65 (47–81)

50 (40–85)

56 (44–68)

47.5 (19–63) ^a

Age at onset <50 years (%)

7(4.8)

3 (2.2)

2 (8)

4 (28.6)

12 (54.5)

Age at onset 50–70 years (%)

114 (78.6)

96 (70.6)

17 (68)

10 (71.4)

10 (45.5)

Age at onset >70 years (%)

24 (16.6)

37 (27.2)

6 (24)

0 (0)

0 (0)

Patients with PRNP gene sequenced (%)

145 (100)

136 (100)

25 (100)

14 (100)

22 (100)

Codon 129 genotype

Met-Met/Total (%)

143/145 (98.6)

133/136 (97.8)

13/25 (100)

14/14 (100)

22/22 (100)

Met-Val/Total (%)

2/145 (1.4)

3/136 (2.2)

0/25 (0)

0/14 (0)

0/22 (0)

Val-Val/Total (%)

0/145 (0)

0/136 (0)

0/25 (0)

0/14 (0)

0/22 (0)

EEG Typical/Total (%)

9/145 (6.2)

92/136 (67.6) ^b

3/25 (12)

8/14 (57.1) ^b

0/22 (0)

14–3–3 Positive/Total (%)

29/145 (20)

99/136 (72.8) ^b

18/25 (72) ^b

14/14 (100) ^b

6/22 (27.2)

Progressive dementia/Total (%)

121/145 (83.4)

128/136 (94.1) ^b

20/25 (80)

13/14 (92.9)

17/22 (77.3)

Myoclonus (%)

33/145 (22.8)

106/136 (77.9) ^b

15/25 (60) ^b

10/14 (71.4) ^b

12/22 (54.5) ^b

Visual or cerebellar disturbance (%)

22/145 (15.2)

88/136 (64.7) ^b

18/25 (72) ^b

11/14 (78.6) ^b

11/22 (50) ^b

Pyramidal or extramidal dysfunction (%)

70/145 (48.3)

104/136 (76.5) ^b

19/25 (76)^c

12/14 (85.7) ^c

13/22 (59.1)

Akinetic mutism (%)

24/145 (16.6)

66/136 (48.5) ^b

10/25 (40)^c

8/14 (57.1) ^b

3/22 (13.6)