title: Bulbar-onset amyotrophic lateral sclerosis in a patient with Chiari I malformation.
abstract: We report on a patient with bulbar-onset, clinically defined, sporadic amyotrophic lateral sclerosis (ALS) who also showed a Chiari I malformation. This malformation, otherwise asymptomatic, was detected during the diagnostic work-up for ALS. To our knowledge this is the first report of an association between these two relatively uncommon disorders. Since our patient worked 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 mechanical 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.
authors.affiliation: ALS Clinic, Institute of Neuropsychiatry, via G. La Loggia 1, 90129 Palermo, Italy.
authors.forename: D
authors.initials: D
authors.lastname: Lo Coco
authors.forename: A
authors.initials: A
authors.lastname: Militello
authors.forename: F
authors.initials: F
authors.lastname: Piccoli
authors.forename: V
authors.initials: V
authors.lastname: La Bella
mesh_terms: D000690:Amyotrophic Lateral Sclerosis / Q000473:pathology*; D001334:Automobile Driving; D001696:Biomechanical Phenomena; D001933:Brain Stem / Q000002:abnormalities*; D015897:Comorbidity; D018450:Disease Progression; D006801:Humans; D008279:Magnetic Resonance Imaging; D008297:Male; D008875:Middle Aged; D012249:Rhombencephalon / Q000002:abnormalities*