以譫妄為表現之空蝶鞍症併發泛腦垂腺低能症：二例報告

Empty Sella Syndrome with Panhypopituitarism Presented as Delirium: Report of Two Cases

空蝴鞍症患者一般腦垂腺機能正常而無症狀。在此報告一48歲男性，表現膽妄之症狀，生化及血液檢查發現低血鈉及貧血，內分泌功能檢查顯示泛腦垂腺低能症，腦部核磁共振影像顯示為空蝶鞍症狀。病患之意識情況及精神症狀，在荷爾蒙補充及低血鈉矯正之後，即慢慢恢復正常。本文回顧過去文獻並分析此疾病病因及臨床表現，使醫師增加對此一疾病罕見表現之認識。

Patients with empty sella syndrome usually have normal pituitary functions and show no symptom. We report a man with panhypopituitarism who presented as acute psychotic symptoms. This 48-year-old man had three traffic accidents without loss of consciousness 24 years ago. After recovery, he led an uneventful life. He noted pale appearance, cold intolerance and decreased beard and eyebrow gradually in these ten years. Hypotension was found in health exams. Absence of axillary and pubic hair was also noticed. There was no impairment of libido. Two weeks prior to this admission, he suffered from productive cough, sore throat and vomiting, which was treated by herb drugs. Acute-onset conscious change with queer behavior developed when he was working in the office. He was then taken to the emergency department. On arrival, he was irritable and disoriented. The BP was 114/64 mmHg, temperature 36.5°C and pulse rate 120/min. Physically, he looked pale. Eyebrow was thin and scanty. The chest, heart nnd abdomen were normal except diminished pigmentation of areola. There was no axillary hair and pubic hair. The testes were soft. Delirium with acute psychotic state was suspected. Hyponatremia (Na 122 mM) and anemia (Hb 9.7 mg/dl) were disclosed. Hypertonic saline was administered. Brain MRI revealed empty sella. His basal hormone levels were: T3 67.5 ng/dl, T4 8.69 μg/dl, hsTSH 0.383 μIU/ml, ACTH 17.6 (8am), 10.8 (4pm) pg/ml, cortisol 1.73 (8am), 6.50 (4pm) μg/dl, hGH 0.44 μg/1, testosterone 0.12 ng/ml, estradiol <20 pg/ml. After IV 100 μg LHRH, the serum FSH and LH levels at 0, 15, 30, 60 and 120 minutes were 0.3, 0.3, 0.6, 0.9, 1.2 and <0.7, 1.8, 3.8, 3.6, 2.7 mIU/ml, respectively. The serum prolactin and hsTSH levels in response to IV 400 μg TRH at 0, 15, 30, 60 and 120 minutes were 4.7, 7.0, 7.4, 5.7, 5.2 ng/ml and 0.316, 1.320, 2.920, 3.200, 2.100 μIU/ml, respectively. Insulin hypoglycemic test revealed the hGH response at 0, 15, 30, 60 and 120 minutes were 0.25, 0.16, 0.12, 0.16, 0.35 μg/1; ACTH: 3.5, 10.9, 12.1, 10.1, 2.1 pg/ml; cortisol: 2.82, 1.82, 2.38, 1.93, 3.88 μg/dl; prolactin: 5.1, 4.9, 4.8, 4.8, 5.4 ng/ml. Under the impression of empty sella with panhypopituitarism, hormone supplement with IV hydrocortisone lOOmg q8h and oral Levothyroxine 100μg qd was administered. His consciousness improved gradually and he was discharged smoothly with oral prednisolone and Levothyroxine. Literatures were reviewed for this rare condition.