Cyclical Cushing's disease. A case report.
A 41-year-old man with clinical Cushing's syndrome and intermittent central ACTH hypersecretion for a period of 9 1/2 years follow-up is described. Episodes of biochemical and clinical remission alternated with periods of florid Cushing's disease, characterized by circadian hyperpulsatile ACTH and cortisol secretion. Responses to metyrapone and inhibition of ACTH and cortisol hypersecretion after high dose dexamethasone during active phases of the disease favored a central origin of ACTH hypersecretion, confirmed by simultaneous bilateral venous sampling of the sinus petrosus inferior. Prolonged clinical remission followed near total anterior hypophysectomy. However, on anatomopathological examination of the pituitary neither corticotroph cell hyperplasia nor a microadenoma could be documented. The possibility of a functional ACTH hypersecretion is discussed.
Velkeniers
B
B
Department of Endocrinology, University Hospital of Brussels, Belgium.
Beckers
A
A
Stevenaert
A
A
Smits
J
J
Finné
E
E
Vanhaelst
L
L
D000311:Adrenal Glands / Q000378:metabolism; D000324:Adrenocorticotropic Hormone / Q000378:metabolism / Q000502:physiology; D000328:Adult; D003480:Cushing Syndrome / Q000503:physiopathology / Q000601:surgery; D006801:Humans; D006854:Hydrocortisone / Q000378:metabolism; D007016:Hypophysectomy; D008297:Male; D010507:Periodicity; D010903:Pituitary Gland, Anterior / Q000601:surgery
D000324:Adrenocorticotropic Hormone; D006854:Hydrocortisone