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

Table 1 Clinical characteristics

					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 V^a	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

ISSN: 2051-5960