Table 2.
Demographics, CSF data, neuropathological classification, and clinical profiles of prospective cohort.
| Neuropathological Classification | |||||||||||
|---|---|---|---|---|---|---|---|---|---|---|---|
| Prion Disease Origin |
Sporadic | Genetic | |||||||||
| Classification | Unit | MM1 | MV1 | VV2 | MM2-C | MM1-2 | MV1-2 | VV1-2 | sFI | gCJD | Total |
| n (%) |
29 (44.6) |
5 (7.7) |
11 (16.9) |
2 (3.1) |
6 (9.2) |
8 (12.3) |
1 (1.5) |
1 (1.5) |
2 (3.1) |
65 | |
| Age at death | yrs (SD) |
69.3 (7.0) |
64.4 (9.3) |
64.5 (8.0) |
66 (4.2) |
74.7 (11.9) |
66.6 (5.2) |
52 (0.0) |
61 (0.0) |
62 (0.0) |
67.5 (8.1) |
| Duration | mo (SD) |
3.4 (2.9) |
3.6 (3.2) |
6.1 (3.2) |
8.5 (0.0) |
2.9 (0.9) |
11.3 (8.9) |
28.9 (0.0) |
7.5 (0.0) |
1.4 (1.1) |
5.5 (5.7) |
| Male | n (%) |
14 (48.3) |
4 (80.0) |
8 (72.7) |
2 (100.0) |
1 (16.7) |
4 (50.0) |
0 (0.0) |
1 (100.0) |
2 (100.0) |
36 (55.4) |
| 14-3-3 positive | n (%) |
25 (86.2) |
2 (40.0) |
11 (100.0) |
1 (50.0) |
6 (100.0) |
5 (62.5) |
1 (100.0) |
0 (0.0) |
2 (100.0) |
53 (81.5) |
|
tTau positive (>1150 pg/ml) |
n (%) |
28 (96.6) |
4 (80.0) |
11 (100.0) |
2 (100.0) |
6 (100.0) |
8 (100.0) |
1 (100.0) |
0 (0.0) |
2 (100.0) |
62 (95.4) |
| tTau | pg/ml (SD) |
6278 (4079) |
3048 (3009) |
8651 (2001) |
4681 (5991) |
5314 (2461) |
3908 (2088) |
6793 (0.0) |
1837 (0.0) |
7372 (462) |
6067 (3528) |
|
RT-QuIC positive |
n (%) |
28 (96.6) |
4 (80.0) |
11 (100.0) |
2 (100.0) |
6 (100.0) |
8 (100.0) |
1 (100.0) |
0 (0.0) |
2 (100.0) |
62 (95.4) |
|
PSWCs on EEG (% with EEG results) |
n (%) |
0 (0.0) |
1 (33.3) |
0 (0.0) |
- | 1 (33.3) |
0 (0.0) |
0 (0.0) |
0 (0.0) |
0 (0.0) |
2 (6.3) |
|
Positive MRI (% with MRI results) |
n (%) |
14 (87.5) |
3 (60.0) |
7 (77.8) |
2 (100) |
4 (80.0) |
4 (57.1) |
1 (100) |
0 (0.0) |
1 (100) |
36 (76.6) |
|
Dementia (% with clinical history) |
n (%) |
20 (95.2) |
5 (100) |
9 (100) |
2 (100) |
5 (100) |
8 (100) |
1 (100) |
1 (100) |
1 (100) |
52 (98.1) |
|
Visual (% with clinical history) |
n (%) |
6 (31.6) |
1 (25.0) |
4 (44.4) |
1 (50.0) |
2 (40.0) |
1 (14.3) |
1 (100) |
0 (0.0) |
0 (0.0) |
16 (32.7) |
|
Ataxia (% with clinical history) |
n (%) |
12 (66.7) |
1 (33.3) |
9 (100) |
1 (50.0) |
2 (40.0) |
6 (85.7) |
1 (100) |
0 (0.0) |
0 (0.0) |
32 (68.1) |
|
Myoclonus (% with clinical history) |
n (%) |
10 (55.6) |
2 (66.7) |
3 (37.5) |
1 (50.0) |
1 (20.0) |
2 (28.6) |
1 (100) |
1 (100) |
0 (0.0) |
21 (45.7) |
|
Pyramidal Symptoms (% with clinical history) |
n (%) |
9 (47.4) |
0 (0.0) |
2 (25.0) |
1 (50.0) |
2 (40.0) |
3 (42.9) |
0 (0.0) |
1 (100) |
0 (0.0) |
18 (38.3) |
|
Extrapyramidal Symptoms (% with clinical history) |
n (%) |
3 (16.7) |
1 (33.3) |
1 (12.5) |
0 (0.0) |
1 (20.0) |
3 (42.9) |
0 (0.0) |
0 (0.0) |
0 (0.0) |
9 (19.6) |