Esophageal achalasia is an idiopathic disorder characterized by degeneration of neurons in the myenteric plexus of the esophagus [1]. Enteric neuropathies, primary and secondary, have been proposed as causes of enteric motility disorders, including achalasia. Secondary causes of enteric neuropathies have been attributed to diabetes mellitus, autoimmune diseases, paraneoplastic syndromes, infectious disease, and states of systemic inflammation [1,2]. Case reports of pediatric patients with Triple-A syndrome (adrenocorticotropic hormone insensitivity, achalasia, and alacrima) and concomitant autonomic neuropathy, as well as a case of a patient with Sjogren's syndrome, achalasia, and sensory neuropathy suggest that systemic disease leading to metabolic or inflammatory derangements may play a role in the pathogenesis of achalasia [3,4].
