脳波異常を呈した若年発症のACTH単独欠損症の1例

Abstract

A case of isolated adrenocorticotropic hormone (ACTH) deficiency is reported. A 25-year-old unconscious female was referred to Hirai Hospital. On admission, her blood glucose level was 25 mg/dl, and her systolic blood pressure was 110 mmHg. She became alert just after intravenous administration of glucose. Plasma ACTH, plasma cortisol, urinary 17-hydroxycorticosteroid (17-OHCS) and urinary 17-ketosteroid (17-KS) levels were low. The circadian periodicity of ACTH and cortisol was disordered. The ACTH-Z test indicated a delayed response in the urinary 17-OHCS and 17-KS levels. Following the rapid ACTH test and lysine vasopressin (LVP) test, plasma ACTH and cortisol were disordered. The levels of other pituitary hormones were within normal limits, and the various stimulation tests also showed normal responses. Hence, a diagnosis of isolated ACTH deficiency was made. On the 3rd hospital day, a diffuse slow-wave pattern was observed on the electroencephalogram (EEG). The clinical symptoms improved notably after oral administration of hydrocortisone 20 mg/day. On the 50th hospital day, the EEG did not show a diffuse slow-wave pattern. We therefore sugest that hydrocor- tisone is effective -for treatment of diffuse slow-wave pattern on the EEG. In this case, it is believed that the hypoglycemic symptoms and the diffuse slow-wave pattern were induced by isolated ACTH deficiency.