Bulbar-onset amyotrophic lateral sclerosis in a patient with Chiari I malformation

We report on a patient with bulbar-onset, clinically defined, sporadic amyo trophic lateral sclerosis (ALS) who also showed a Chiari I malformation. Th is malformation, otherwise asymptomatic, was detected during the diagnostic work-up for ALS. To our knowledge this is the first report of ail associat ion between these two relatively uncommon disorders. Since our patient work ed for many years as a bus-driver and because the ALS symptoms began in the brainstem region, we suggest that his hindbrain anomaly, along with the me chanical stress on the spine and cranio-vertebral junction due to the daily driving, might have played a role in the region-specific onset of ALS.