Objective: To determine the frequency, timing, causes, and risk factors of death in Guillain-Barré syndrome (GBS).
Methods: Prospectively collected data were reviewed from a cohort of 527 patients with GBS previously included in 1 observational and 3 therapeutic studies. Risk factors were identified by comparing deceased and surviving patients with GBS.
Results: Fifteen (2.8%) of 527 patients with GBS died within 6 months of follow-up at highly variable time points during the disease course, with a median time from onset of weakness to death of 76 days (interquartile range 23-152 days). In 356 patients with an extended follow-up of 12 months, the mortality rate was 3.9%. Only 3 patients (20%) died during the acute progressive phase and 2 patients (13%) died during the plateau phase. Ten patients (67%) died during the recovery phase after neurologic improvement, most frequently from respiratory or cardiovascular complications. Eleven patients (73%) were admitted to an intensive care unit during the course of disease, but only 7 patients (47%) died in the intensive care unit. Risk factors for death were age (p < 0.001), severity of weakness at entry (p = 0.02), mechanical ventilation (p < 0.001), delay from onset of weakness to entry (p = 0.035), and time to peak disability (p = 0.039).
Conclusions: Death after GBS predominantly occurs in the elderly and severely affected patients, especially during the recovery phase. Future research is required to determine whether mortality of GBS can be reduced by intensified monitoring in patients with an increased risk profile.