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 | |||
| Dementia | 10 (100%) | 63 (97%) | 0.1 |
| Akinetic mutism | 10 (100%) | 6 (9%) | 0.0001 |
| Typical EEG | 8 (80%) | 22 (34%) | 0.01 |
| Myoclonus | 8 (80 %) | 58 (89%) | 0.5 |
| Cerebellar signs | 7 (70%) | 30 (46%) | 0.3 |
| (Extra-)pyramidal signs | 3 (30%) | 19 (29%) | 0.8 |
| Visual signs | 2 (20%) | 12 (19%) | |
| Sex (female/male) | 5/5 | 34/31 | 0.5 |
| Age of onset (years) | 65 (8) | 62 (9) | 0.5 |
| Duration (months) | 2 (1) | 7 (5) | 0.001 |
| Biomarkers | |||
| 14-3-3 | 10 (100%) | 62 (97%) | 0.7 |
| Tau (pg/ml) | 15 384 | 7582 | 0.01 |
| Phospho tau (pM) | 1164 | 14.7 | 0.0001 |
| Aβ1-42 (pg/ml) | 303 | 324 | 0.3 |
| Neuropathology | (n=6) | (n=36) | |
| NFT | 2 (33%) | 7 (20%) | 0.5 |
| Amyloid β | 2 (33%) | 9 (26%) | 0.5 |
| CJD classification | |||
| 6 MM1 | 32 MM1, 1 MV1, 1MV2, 2 VV2 | 0.3 | |