Case report: Safety and usefulnes of Metyrapone for the control of hypercortisolism in a complex patient with Cushing´s disease

P. Remon-Ruiz*^a (Mr), I. De Lara^a (Ms), JM. Canelo^a (Mr), E. Venegas-Moreno^a (Mrs), E. Dios-Fuentes^a (Mrs), A. Kaen^a (Mr), E. Cardenas Ruiz-Valdepeñas^a (Mr), A. Soto-Moreno^a (Dr)

^a Unidad de Gestión Clínica de Endocrinología y Nutrición. Instituto de Biomedicina de Sevilla (IBiS), Hospital Universitario Virgen del Rocío/CSIC/Universidad de Sevilla, Seville, SPAIN

* pjremonruiz@gmail.com

INTRODUCTION

Medical treatment for hypercortisolism control is necessary as pretreatment for patients with Cushing's disease and for patients with persistent or recurrent Cushing's disease.

These treatments must be effective in the biochemical control of hypercortisolism as well as in achieving recovery of the circadian cycle of cortisol in order to improve Cushing´s symptoms.

MATHERIAL AND METHODS

We present a case report of a patient with Cushing´s disease with ketoconazole intolerance but good response to metyrapone.

RESULTS

A 48-year-old patient with bipolar disorder, obesity and HTN with symptons of Cushing disease. Persistently elevated 24 hours urinary free cortisol (1302 and 1245 µg/24 hours [35-135]), nocturnal salivary cortisol (4.06 and 1.98 ug/dL [0.03-0.33]) confirmed hypercortisolism. A diagnosis of ACTH-dependent Cushing's syndrome was made based on elevated ACTH (25.8 pg/mL). In pituitary MR no adenoma was found, but a temporomesial glioma was observed. Multidisciplinary team decided prioritize glioma treatment and treat hypercortisolism by medical through steroidogenesis inhibitor.

Ketoconazol 200 mg twice a day was iniciated. 5 days after, patient began with arterial hypotension, dysarthria and absence seizures. He was referred to emergency room but symptons related with glioma were rouled out and she was diagnosed with lithium poisoning. Due to temporal association with initiation of ketoconazol, metyrapone 250 mg twice a day was iniciated reaching 500 mg twice a day. With this dose adjustement, hypercortisolism control was achieved with normal 24-hours urinary free cortisol (85,5 and ug/24 hours) and almost normal nocturnal salivary cortisol (0,38 µg/dL). There weren´t new episodes of lithium poisoning and glial lesion was treated with surgery and adyuvant radiotherapy.

CONCLUSIONS

Metyrapone is an useful and secure treatment for hypercortisolims control and can be used in complex cases as the one presented.

The author has declared no conflict of interest.