Table 2 Selected clinical features of sCJDVV1-2, sCJDVV1 and sCJDVV2.

From: Co-existence of PrPD types 1 and 2 in sporadic Creutzfeldt-Jakob disease of the VV subgroup: phenotypic and prion protein characteristics

Presentation

sCJDVV2

sCJDVV1-2

sCJDVV1

Case number (Table 1)

1-8

1-8

9-14

15-18

1-5

% T2

100

≥91

37-77

≤9

0

Age at onset (years) (mean ± SD; median) [range]a

68 ± 8; 72

[56–78]

62 ± 12; 68

[44–74]

68 ± 11; 64

[57–84]

64 ± 9; 67

[52–71]

32 ± 5; 34

[25–38]

Duration (months) (mean ± SD; median) [range]b

6 ± 2; 5

[3–9]

6.5 ± 4; 5

[3–17]

6 ± 2; 5

[4–8]

13.5 ± 10; 12

[3–26]

11 ± 2; 12

[8–14]

Male gender

38c (3/8)d

50 (4/8)

67 (4/6)

25 (1/4)

60 (3/5)

Cognitive declinee

33 (2/6)

37 (3/8)

67 (4/6)

100 (3/3)

100 (4/4)

Cerebellar signsf

100 (6/6)

88 (7/8)

50 (3/6)

0 (0/3)

0 (0/4)

PSWC on EEG

0 (0/6)

20 (1/5)

33 (1/3)

0 (0/4)

0 (0/4)

Positive 14-3-3

100 (6/6)

100 (8/8)

100 (4/4)

100 (2/2)

100 (3/3)

  1. aAll sCJDVV1-2 cases combined and each of the three groups indicated were significantly different (P < 0.0004-0.0001) from -VV1 but not from -VV2 cases. bVV1-2 cases 1-8 and 9-14, combined were significantly different from VV1 (P < 0.007); cases 15-18 were not significantly different from VV1. cExpressed in percent; dcases with the feature listed/total cases examined. e,fCognitive decline and cerebellar signs were respectively inversely and directly correlated to % T2 (r = −0.99 and r = 0.99). Statistical significance was calculated using Student’s t-test for age and duration, and Fisher’s exact test to determine non-random associations in the other clinical data; Pearson’s test was used to assess correlations; SD: standard deviation.
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