Triple A (Allgrove) syndrome, an autosomal recessive disease is characterized by 
achalasia, alacrimia and ACTH-resistant adrenal failure with progressive 
neurological syndrome including central, peripheral and autonomic nervous system 
impairment, and mild mental retardation. The triple A syndrome gene, designated 
AAAS, localized on chromosome 12q 13 encodes for a 546 amino acid protein called 
ALADIN (Alacrimia-Achlasia-Adrenal Insufficiency and Neurologic disorder). This 
report relates to two sisters, aged 8 and 12 years, who had vomiting, muscle 
weakness, alacrimia, excessive fatigue and dysphagia. Abdominal sonography, 
esophago-gastroduodenoscopy, barium swallow, esophageal manometry, CT scan 
abdomen and brain, biochemical profiles, as well as neurologic and ophthalmic 
evaluations were consistent with Allgrove's syndrome. Management consisted of 
pneumatic balloon dilatation for achalasia and initiation of cortisone therapy 
with successful resolution of dysphagia and other symptoms.