Yokoyama T, Komurasaki Y, Uemoto M, Ogura J, Maeda K
Rinsho Shinkeigaku. 1989 Aug;29(8):1023-7.
We report here three patients with hypopituitarism accompanied by primary empty sella, whose first manifestations were various mental symptoms. Endocrine studies revealed that two patients showed panhypopituitarism and the other had isolated adrenocorticotropin (ACTH) deficiency. Although several different types of pituitary dysfunctions have been described in a mild form, empty sella is usually asymptomatic. Their first manifestations were mental symptoms; consciousness disturbance, psychomotor agitation, visual hallucination and delusion. Isolated ACTH deficiency is an uncommon disease which etiology is still undetermined. A case with isolated ACTH deficiency associated with an empty sella has been reported before. It is suggested that empty sella might have a role in pathogenesis of isolated ACTH deficiency. The empty sella was confirmed by metrizamide cisternography and magnetic resonance imaging (MRI). These imaging studies are good tools to disclose empty sella. Replacement with cortisone and levothyroxine resulted in an improvement in the mental symptoms in two patients with panhypopituitarism. No alteration was observed following cortisone administration in the patient with isolated ACTH deficiency. Delusion and visual hallucination in this patient poorly responded to treatment with neuroleptics.
我们在此报告三例伴有原发性空蝶鞍的垂体功能减退患者,其首发表现为各种精神症状。内分泌检查显示,两名患者表现为全垂体功能减退,另一名患者存在孤立性促肾上腺皮质激素(ACTH)缺乏。虽然已有几种不同类型的垂体功能障碍以轻度形式被描述,但空蝶鞍通常无症状。他们的首发表现为精神症状,如意识障碍、精神运动性激越、视幻觉和妄想。孤立性ACTH缺乏是一种病因仍未明确的罕见疾病。之前曾有一例孤立性ACTH缺乏与空蝶鞍相关的病例报道。提示空蝶鞍可能在孤立性ACTH缺乏的发病机制中起作用。通过甲泛葡胺脑池造影和磁共振成像(MRI)证实为空蝶鞍。这些影像学检查是发现空蝶鞍的良好工具。对两名全垂体功能减退患者使用可的松和左甲状腺素替代治疗后,精神症状有所改善。在孤立性ACTH缺乏的患者中,给予可的松后未观察到变化。该患者的妄想和视幻觉对抗精神病药物治疗反应不佳。