| | | | | |
Time from onset to clinical event (months)
| | | |
|---|
|
Patients
|
Sex
|
Age at onset(years)
|
Disease duration(months)
|
Disease duration(months)
|
Need for tracheostomy invasive ventilation
|
Progression to total quadriplegia
|
Development of overt oculomotor limitation
|
Resulting in communication Stage Va
|
Time from Stage V to death(months)
|
Accumulated protein
|
Reference
|
|---|
|
1
|
F
|
62
|
104
|
LE
|
12
|
34
|
33
|
37
|
67
|
TDP-43
|
[20]
|
|
2
|
F
|
52
|
161
|
UE
|
12
|
25
|
14
|
76
|
85
|
TDP-43
| |
|
3
|
F
|
53
|
120
|
B
|
56
|
120
|
120
|
120
|
0
|
TDP-43
| |
|
4
|
M
|
73
|
78
|
B
|
5
|
na
|
11
|
29
|
49
|
TDP-43
|
[27] patient 2
|
|
5
|
M
|
60
|
117
|
UE
|
15
|
31
|
18
|
81
|
36
|
TDP-43
|
[7] case 1
|
|
6
|
M
|
64
|
57
|
UE
|
12
|
33
|
33
|
56
|
1
|
TDP-43
|
[7] case 2
|
|
7
|
F
|
13
|
312
|
LE
|
18
|
18
|
36
|
120
|
192
|
FUS
|
[17] patient 1
|
|
8
|
M
|
39
|
102
|
LE
|
24
|
66
|
86
|
102
|
0
|
FUS
|
[16]
|
|
9
|
F
|
38
|
106
|
LE
|
8
|
9
|
11
|
36
|
70
|
SOD1
|
[24]
|
|
10
|
M
|
57
|
128
|
B
|
21
|
45
|
52
|
84
|
44
|
SOD1
|
[10]
|
|
11
|
M
|
61
|
29
|
UE
|
5
|
6
|
19
|
28
|
1
|
SOD1
|
[27] patient 1
|
|
mean
| |
52.0
|
119.5
| |
17.0
|
38.7
|
39.4
|
69.9
|
47.8
| | |
-
F female, M male, na not available, TDP-43 TAR DNA-binding protein 43, FUS fused in sarcoma, SOD1 copper/zinc superoxide dismutase 1, LE lower extremity, UE upper extremity, B bulbar
-
aCommunication Stage V is indicative of a “totally locked-in state” [7, 8], in which all voluntary movements are lost and communication is impossible by any means
