A patient with a progressive, inherited disease of the CNS developed remarkable esophageal dilatation without the lower esophageal sphincter dysfunction characteristic of achalasia. Postmortem examination included neuropathologic study of the CNS and evaluation of esophageal myenteric plexus histology, but the pathogenesis of the megaesophagus remains unknown.
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Longstreth et al. (1994) studied this question.