A Case of Isolated ACTH Deficiency

Keimyung Medical Journal 1997;16(3):397-404.

부신피질자극호르몬 단독 결핍 1례

박경식; 이승현; 최석영; 박태규; 이동화; 이찬우; 정성복; 이인규

Abstract

Isolated ACTH deficiency is a rare disorder which shows secondary adrenal insufficiency with normal states of other pituitary hormones. A 56-year-old postmenopausal woman complaining general weakness and intermittent mental confusion presented negligible serum cortisol and ACTH level with hyperprolactinemia and mild hyponatremia. Rapid ACTH stimulation test did not show significant increase of serum cortisol level. Combined pituitary stimulation test showed negligible response of cortisol and ACTH, and slightly decreased response of prolactin and gonadotropin. But the responses of TSH and GH were normal. There was no evidence of organic pituitary of hypothalamic lesion in brain MRI and antithyroid autoantibody was not detected in serum. After receiving 12 months of glucocorticoid replacement therapy, she skipped the medicine for 3 weeks and we performed follow-up endocrinologic tests. Serum ACTH and cortisol level was also negligible and prolonged ACTH simulation test showed normal response of serum cortisol. CRF stimulation test showed no response of serum ACTH. Basal prolactin level was decreased to near normal and combined pituitary stimulation test showed normal response of prolactin with slightly blunted response of TSH and GH. We concluded that the case was nonautoimmune origined isolated ACTH deficiency which was accompanied by hyperprolactinemia and resultant deficiency of gonadotropin, so we report this case with review of literatures. A 56~year-old postmenopausal woman complaining general weakness and intermittent mental confusion presented negligible serum Cortisol and ACTH level with hyperprolactinemia and mild hyponatremia. Rapid ACTH stimulation test did not show significant increase of serum Cortisol level. Combined pituitary stimuiation test showed negligible response of Cortisol and ACTH; and slightly decreased response of prolactin and gonadotropin. But the responses of TSH and Gil were normal. There was no evidence of organic pituitary or hypothalamic lesion in brain MRI and antithyroid autoantibody was not detected in serum. After receiving 12 months of gluc(>corticoid replacement therapy; she skipped the medicine for 3 weeks and we performed follow-up endocrinologic tests. Serum ACTH and Cortisol level was also negligible and prolonged ACTH stimulation test showed normal response of serum Cortisol. CRF stimulation test showed no response of serum ACTH. Basal prolactin level was decreased to near normal and combined pituitary stimulation test showed normal response of prolactin with slightly blunted response of TSH and GH. We concluded that the case was nonautoimmune origined isolated ACTH deficiency which was accompanied by hyperprolactinemia and resultant deficiency of gonadotropin; so we report this case with review of literatures.

Key Words: 부신피질자극호르몬단독결핍, 뇌하수체, ACTH deficiency, Hyperprolactinemia, Hyponatremia