Table 1 Demographic and clinical characteristics of the patients with PrDs and non-PrDs
Characteristics | PrDs | non-PrDs | |||||
|---|---|---|---|---|---|---|---|
sCJD | T188K-gCJD | E200K-gCJD | P102L-GSS | D178N-FFI | AD + PD | VE + AE | |
No | 86 | 58 | 32 | 14 | 33 | 17 | 74 |
Gender (M/F) | 46/40 | 34/24 | 11/21 | 6/8 | 18/15 | 7/10 | 46/28 |
Median age at onset (y, IQR) | 64 (55–69) | 62 (56–68) | 57 (49–66) | 49 (43–59) | 55 (43–63) | 64 (56–70) | 58 (47–69) |
CSF 14-3-3 Positive/Total no. (%) | 56 (65.1) | 40 (69.0) | 20/31 (64.5) | 7 (50.0) | 11 (33.3) | 8 (47.1) | 35/71 (49.3) |
129 MM of PRNP/Total no. (%) | 82 (95.3) | 58 (100.0) | 31 (96.9) | 14 (100.0) | 33 (100.0) | 16 (94.1) | 72 (97.3) |
129 MV of PRNP/Total no. (%) | 3 (3.5) | 0 (0.0) | 1 (3.1) | 0 (0.0) | 0 (0.0) | 1 (5.9) | 2 (2.7) |
129 VV of PRNP/Total no. (%) | 1 (1.2) | 0 (0.0) | 0 (0.0) | 0 (0.0) | 0 (0.0) | 0 (0.0) | 0 (0.0) |
PSWC in EEG (%) | 17/24 (70.8) | 17/44 (38.6) | 13/21 (61.9) | 1/8 (12.5) | 0 (0.0) | 1/17 (5.9) | 1/47 (2.1) |
MRI abnormal change/Total no. (%) | 70/84 (83.3) | 46/57 (80.7) | 29 (90.6) | 13 (92.9) | 7/30 (23.3) | 4/16 (25.0) | 24/69 (34.8) |
Progressive dementia/Total no. (%) | 85 (98.8) | 58 (100.0) | 32 (100.0) | 13 (92.9) | 33 (100.0) | 16 (94.1) | 74 (100.0) |
Myoclonus no. (%) | 53 (61.6) | 34 (58.6) | 22 (68.8) | 5 (35.7) | 17 (51.5) | 2 (11.8) | 27 (36.5) |
Visual or cerebellar disturbance no. (%) | 67 (77.9) | 42 (72.4) | 28 (87.5) | 9 (64.3) | 21 (63.6) | 5 (29.4) | 12 (16.2) |
Pyramidal or extrapyramidal dysfunction no. (%) | 76 (88.4) | 44 (75.9) | 29 (90.6) | 12 (85.7) | 25 (75.8) | 8 (47.1) | 26 (35.1) |
Akinetic Mutism no. (%) | 34 (39.5) | 30 (51.7) | 16 (50.0) | 4 (28.6) | 5 (15.2) | 4 (23.5) | 10 (13.5) |
Individuals with survival time no. (%) | 37 (43.0) | 25 (43.1) | 4 (12.5) | 2 (14.3) | 6 (18.2) | 1 (5.9) | 2 (2.7) |
Survival time (month, IQR) | 3 (2.0–3.5) | 5 (4.0–6.0) | 4.5 (3.3–9.5) | NA | 10 (6.3 ~ 15.0) | 14 | NA |