ALS patients on TPPV - Totally locked-in state, neurologic findings and ethical implications

Seventy patients with ALS using tracheostomy positive pressure ventilation (TPPV) were evaluated for impaired communication. Severely impaired communication is divided into the totally locked-in state (TLS) and the minimal communication state (MCS). TLS occurred in 8 of 70 (11.4%). In the 33 patients on TPPV for more than 5 years, TLS developed in 6 (18.2%) and MCS in 11 (33.1%). Respiratory management for ALS allows long-term survival if mechanical ventilation is used.