Intractable Nausea Attributable to Isolated Deficiency of Adrenocorticotropic Hormone: Prompt Resolution after Administration of Glucocorticoid

Abstract

To highlight the vague symptoms of isolated adrenocorticotropic hormone (ACTH) deficiency and suggest helpful diagnostic tests and appropriate management. We present a detailed case report, including clinical and laboratory findings, and discuss the diagnostic approach to this rare disorder of isolated ACTH deficiency. A 74-year-old woman had persistent severe nausea and other vague symptoms. Elaborate testing during a period of several weeks failed to determine the cause. Ultimately, isolated ACTH deficiency was diagnosed after documentation of subnormal random serum cortisol and ACTH levels as well as urinary free cortisol concentrations. Moreover, the nausea and other symptoms abated after administration of glucocorticoid. The patient was then maintained on orally administered dexamethasone, 0.5 mg twice daily, for 4 to 6 weeks, at which time the diagnosis was confirmed by a subnormal cortisol response on cosyntropin stimulation testing and an unmeasurable ACTH level after overnight oral administration of metyrapone. Although the cause of this patient's isolated ACTH deficiency remained undetermined, it was thought to be autoimmune in nature because of the presence of Graves' disease. This report emphasizes the need for recognition of symptoms, appropriate testing to confirm the diagnosis, and prompt treatment with glucocorticoids in patients with isolated ACTH deficiency.