In some patients with severe autonomic manifestations, beyond typical ALS neuropathological features, alpha-synuclein-positive intracytoplasmic inclusions have been found in ganglion cells of the esophageal nerve plexus [287], suggesting that in selected cases, alpha-synucleinopathy, and therefore an added early/presymptomatic parkinsonism, may be related, at least in part, with the autonomic dysfunction. This evidence concerns the gene SNCA and Parkinson disease.