Endocrine Abstracts

Introduction: Glucocorticoid resistance syndrome is a rare disease, sporadic or familial, of autosomal dominant or recessive inheritance. It is a partial or complete inability of glucocorticoids to exert their effects on target tissues. Associated with compensatory increases in corticotropin and circulating cortisol with excessive secretion of adrenal androgens and mineralocorticoids.

Observation: A 67-year-old patient, having recently discovered diabetes started on insulin then on metformin 1g /d, hypertensive for 1 year on amlodipine 10 mg/d, with no personal or family endocrinopathy. Having presented a month before his admission tremors of the extremities, with muscular weakness, and walking disorders. All in a context of weight loss and asthenia. On examination, the patient did not present with clinical cushing syndrome The results showed a persistent hypokalaemia at 1.9mmol/l despite potassium supplementation, a hypernatremia at 147mmol / l. On the overnight 1mg-Dexamethasone suppression test, cortisol levels were high to 25.7 μg / dl. A high urinary free cortisol 1610 mg / 24 h (20-50), with ACTH at 408ng / l (5-60), the pituitary MRI did not reveal any abnormality. The bone densitometry was normal. The diagnosis of glucocorticoid resistance syndrome was retained and the patient was placed on dexamethasone 2 mg / day with a good clinical course, in particular a notable improvement in paresthesia and asthenia, and biological improvement with normalization of serum potassium. Furthermore, the patient presented with hypercalcemia due to primary hyperparathyroidism having undergone resection of the parathyroid adenoma with good progress. The anatomo-pathology did not objectify signs of malignancy

Conclusion: Glucocorticoid resistance syndrome is a rare and often unrecognized condition. This should be considered in case of chronic asthenia associated with hypokalaemia in the context of excess cortisol without clinical cushing syndrome.