Table 1

Comparison of the two Creutzfeldt-Jakob disease subgroups, CJDhigh and CJDlow

CJDhigh (n=10)CJDlow (n=65)p Value
Values are n (%) or mean (SD).
In the upper part a comparison is made between the clinical features and biomarkers. In the lower part the neuropathological features and CJD classification is compared in patients from whom frozen tissue was available (6 CJDhigh, 35 CJDlow). The p values were calculated using Fisher's exact test except for duration and age of onset (Mann–Whitney U test). Significant values are indicated in bold.
Clinical features
Dementia10 (100%)63 (97%)0.1
Akinetic mutism 10 (100%) 6 (9%) 0.0001
Typical EEG 8 (80%) 22 (34%) 0.01
Myoclonus8 (80 %)58 (89%)0.5
Cerebellar signs7 (70%)30 (46%)0.3
(Extra-)pyramidal signs3 (30%)19 (29%)0.8
Visual signs2 (20%)12 (19%)
Sex (female/male)5/534/310.5
Age of onset (years)65 (8)62 (9)0.5
Duration (months) 2 (1) 7 (5) 0.001
Biomarkers
14-3-310 (100%)62 (97%)0.7
Tau (pg/ml) 15 384 7582 0.01
Phospho tau (pM) 1164 14.7 0.0001
1-42 (pg/ml)3033240.3
Neuropathology (n=6)(n=36)
NFT2 (33%)7 (20%)0.5
Amyloid β2 (33%)9 (26%)0.5
CJD classification
6 MM132 MM1, 1 MV1, 1MV2, 2 VV20.3