Table 1 The main clinical and laboratory features of Chinese E200K gCJD patients.
Items | Total (n = 30) | Gender | Age of onset | ||||
|---|---|---|---|---|---|---|---|
M (n = 11) | F (n = 19) | P value | <60 y (n = 18) | ≥60 y (n = 12) | P value | ||
Age of onset (median) | 57.5 y | 61 y | 54 y | — | — | ||
Foremost symptoms | |||||||
Progressive dementia | 22 (73.3%) | 8 (72.7%) | 14 (73.7%) | 1.0000 | 12 (66.7%) | 10 (83.3%) | 0.4192 |
Mental problems | 16 (53.3%) | 5 (45.5%) | 11 (57.9%) | 0.7065 | 10 (55.6%) | 6 (50.0%) | 1.0000 |
Cerebellum symptoms | 9 (30.0%) | 5 (45.5%) | 1 (5.3%) | 0.0156 | 2 (11.1%) | 4 (33.3%) | 0.1844 |
Pyramidal & extrapyramidal symptoms | 6 (20.0%) | 4 (36.4%) | 5 (26.3%) | 0.6871 | 4 (22.2%) | 5 (41.7%) | 0.4181 |
Sleeping disturbances | 3 (10.0%) | 1 (9.1%) | 2 (10.5%) | 1.0000 | 1 (5.6%) | 2 (16.7%) | 0.5478 |
Major symptoms | |||||||
Dementia | 29 (96.7%) | 10 (90.9%) | 19 (100.0%) | 0.3667 | 17 (97.4%) | 12 (100.0%) | 1.0000 |
Myoclonus | 22 (73.3%) | 9 (81.8%) | 13 (68.4%) | 0.6722 | 13 (72.2%) | 9 (75.0%) | 1.0000 |
Visual or cerebellar disturbance | 21 (70.0%) | 9 (81.8%) | 12 (63.2%) | 0.4189 | 12 (66.7%) | 9 (75.0%) | 0.7036 |
Pyramidal and extrapyramidal symptoms | 25 (83.3%) | 9 (81.8%) | 16 (84.2%) | 1.0000 | 13 (72.2%) | 12 (100.0%) | 0.0657 |
Mutism | 16 (53.3%) | 5 (45.5%) | 11 (57.9%) | 0.7065 | 8 (44.4%) | 8 (66.7%) | 0.2839 |
Examinations | |||||||
14-3-3a | 20 (74.0%) | 8 (80.0%) | 12 (70.6%) | 0.6784 | 12 (75.0%) | 8 (72.7%) | 1.0000 |
EEGb | 13 (50.0%)b | 4 (40.0%) | 9 (56.3%) | 0.6882 | 8 (50.0%) | 5 (50.0%) | 1.0000 |
MRI | 26 (86.9%) | 9 (81.8%) | 17 (89.5%) | 0.6111 | 15 (83.3%) | 11 (91.7%) | 1.0000 |
