Achalasia-Alacrima-Acth Insensitivity Syndrome (Triple a Syndrome) in a Saudi Family

Triple A Multisystem Disorder: Allgrove Syndrome

“…Thus, treatments for adrenal insufficiency are highly dependent on the adrenal gland function. Treatment for severe adrenal insufficiency is usually oral glucocorticoids such as prednisolone or hydrocortisone [10]. In our case, however, the patient had partial adrenal insufficiency with low but relatively normal cortisol and the discontinuation of prednisolone was chosen as the best route of treatment.…”

“…AS classically presents with the triad of adrenal insufficiency, alacrimia, and achalasia in 70% of patients; however, patients may have a different collection of presenting symptoms [8]. Symptoms and signs of AS include alacrimia, ACTH resistant adrenal insufficiency, achalasia, dysphagia, autonomic disturbances, weight loss, hyperpigmentation, extrapyramidal dysfunction symptoms, recurrent infections, recurrent hypoglycemic episodes, neurologic deficits, mental retardation, hypotension, and orthostatic hypotension [10,11]. Thus, diagnosis of AS can be challenging due to the numerous presentations.…”