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Table 1 Clinical characteristics

From: Clinicopathological characteristics of patients with amyotrophic lateral sclerosis resulting in a totally locked-in state (communication Stage V)

      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   
  1. 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
  2. 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