Mortality and complications of the locked-in syndrome

The locked-in syndrome is a severe disability consisting of quadriplegia and anarthria with preserved consciousness. No large series of cases have been reported and very few cases of long-term survival have been described. We present a follow-up of 27 patients "locked-in" for more than one year. Twenty-four were still alive up to 12.5 years after onset, with a mean survival of 4.9 years. Significant recovery was noted in only a few patients. Seventeen patients lived at home at the time of study. Eight were never hospitalized after the initial event. Gastrostomy and tracheostomy tubes and indwelling catheters were eventually removed from many patients. Electronic devices were used by ten patients to facilitate communication. We conclude that rehabilitation and medical care must be planned carefully, given the length of survival shown in this group.