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

Table 3 Clinicopathological characteristics of previously reported patients in communication Stage V

			Time from onset to clinical event, months
Sex	Age at onset (years)	Disease duration (months)	Need for tracheostomy invasive ventilation	Progression to total quadriplegia	Development of overt oculomotor limitation	resulting in communication Stage V	Communication Stage V to death(months)	Accumulated protein	Neuropathology Brain weight	Upper and lower motor neurons	Substantia nigra	Globus pallidus	Subthalamic nucleus	Brainstem reticular formation	Cerebellar efferent system	Reference
M	38	60	11	20	20	32	28	ubiquitin	1300	+++	+++	+++	na	+++	++	[14] Patient 1
M	38	156	60	132	96	144	12	ubiquitin^a	1230	+++	+++	na	na	++	+++	[14] Patient 2
M	63	144	36	na	36	na	na	TDP-43	830	+++	+++	+	na	+++	+	[11]
F	33	288	24	na	na	156	84	a mutation in OPTN		N/A	N/A	N/A	N/A	N/A	N/A	[28] Case 1^b
M	35	240	36 ≥	na	na	36	204	a mutation in OPTN		N/A	N/A	N/A	N/A	N/A	N/A	[28] Case 2^b
M	44	27	6	15	15	26	1	na	1320	+++	+	+	+	+++	+	[26]

M male, F female, na not available, N/A not applicable, OPTN the optineurin gene
^aubiquitin-immunoreactive basophilic inclusions
^bClinical report

ISSN: 2051-5960