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

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           
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 ubiquitina 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 1b
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 2b
M 44 27 6 15 15 26 1 na 1320 +++ + + + +++ + [26]
  1. M male, F female, na not available, N/A not applicable, OPTN the optineurin gene
  2. aubiquitin-immunoreactive basophilic inclusions
  3. bClinical report