Fang V S, Jaspan J B
Department of Medicine, University of Chicago, Pritzker School of Medicine, Illinois 60637.
J Clin Endocrinol Metab. 1989 Nov;69(5):1073-7. doi: 10.1210/jcem-69-5-1073.
A 68-yr-old man had developed intractable vomiting soon after recovering from a flu-like illness. The use of Compazine as an antiemetic produced classic dystonic manifestations which resolved rapidly after discontinuation and treatment with Artane. However, he later developed a variety of neurobehavioral disturbances which led to his admission to the hospital. Extensive diagnostic procedures failed to identify any gastrointestinal or neurological causes. His condition unceasingly worsened until hypocortisolemia was serendipitously discovered, and all of his symptoms disappeared rapidly and completely with glucocorticoid replacement. Over the course of hospitalization, other than a single episode of orthostatic hypotension, the patient did not manifest any signs of adrenal insufficiency or endocrinopathy. Although detectable, his plasma ACTH level was markedly low in the presence of hypocortisolemia. His adrenal function was subnormal in the cortisol response to ACTH stimulation. His renin-angiotensin-aldosterone system and catecholamine levels were normal. He had normal pituitary responses to GnRH, TRH, and insulin, with rises in plasma levels of LH, FSH, TSH, PRL, and GH, but no stimulation of ACTH. Repeated CRH tests revealed no stimulation of ACTH and cortisol. No circulating anti-ACTH, antiadrenal, or antipituitary antibody was detected. We conclude that this elderly patient had a rare syndrome of selective corticotroph dysfunction which resulted in secondary adrenal failure and exacerbated his mental and neuromuscular abnormalities. To our knowledge, these symptoms, which clearly relate to hypocortisolism, have not been previously reported.
一名68岁男性在从类似流感的疾病康复后不久出现顽固性呕吐。使用丙氯拉嗪作为止吐药产生了典型的肌张力障碍表现,停药并用安坦治疗后迅速缓解。然而,他后来出现了多种神经行为障碍,导致他入院。广泛的诊断程序未能发现任何胃肠道或神经方面的病因。他的病情不断恶化,直到偶然发现低皮质醇血症,用糖皮质激素替代治疗后所有症状迅速完全消失。在住院期间,除了一次体位性低血压发作外,患者未表现出任何肾上腺功能不全或内分泌病的迹象。尽管可检测到,但在低皮质醇血症的情况下,他的血浆促肾上腺皮质激素(ACTH)水平明显偏低。他对ACTH刺激的皮质醇反应中肾上腺功能低于正常。他的肾素-血管紧张素-醛固酮系统和儿茶酚胺水平正常。他对促性腺激素释放激素(GnRH)、促甲状腺激素释放激素(TRH)和胰岛素的垂体反应正常,血浆促黄体生成素(LH)、促卵泡生成素(FSH)、促甲状腺激素(TSH)、催乳素(PRL)和生长激素(GH)水平升高,但ACTH未受刺激。重复的促肾上腺皮质激素释放激素(CRH)试验显示ACTH和皮质醇未受刺激。未检测到循环中的抗ACTH、抗肾上腺或抗垂体抗体。我们得出结论,这名老年患者患有罕见的选择性促肾上腺皮质激素功能障碍综合征,导致继发性肾上腺功能衰竭,并加重了他的精神和神经肌肉异常。据我们所知,这些明显与低皮质醇血症相关的症状此前尚未见报道。