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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