Achalasia, alacrima, and glucocorticoid deficiency, with congenital absence of zona fasciculata and congenital defective tear formation. Associated symptoms include mild mental retardation, ataxia, sensory disturbances, and optic atrophy. Onset of Addison disease begins at 2 years of age, and onset of achalasia occurs during school years. Heredity is autosomal recessive.