[一例与空蝶鞍及胼胝体发育不全相关的垂体功能减退症，一种视隔-垂体发育异常的变异形式]

[A case of hypopituitarism associated with empty sella and agenesis of corpus callosum, a variant form of septo-optic-pituitary dysplasia].

作者信息

Fukutomi T, Masakado M, Takayanagi R, Ohnaka K, Sekiya K, Haji M, Ohasi M, Nawata H

机构信息

Third Department of Internal Medicine, Faculty of Medicine, Kyushu University, Fukuoka.

出版信息

Fukuoka Igaku Zasshi. 1990 Dec;81(12):396-401.

PMID:2076855

Abstract

A 41-year-old man was referred to Kyushu University Hospital for evaluation of hypothyroidism and hypocortisolemia. Pituitary function test revealed the deficiency of GH(growth hormone), ACTH(adrenocorticotropic hormone), prolactin and TSH(thyroid stimulating hormone). MRI showed empty sella and agenesis of corpus callosum. Clinical diagnosis was hypopituitarism with midline brain anomaly. Septo-optic-pituitary dysplasia (SOPD) is a syndrome characterized by agenesis of septum pellucidum or corpus callosum, optic nerve hypoplasia and congenital hypothalamic-pituitary insufficiency. Our case had no ocular anomalies, but today it is regarded as a variant form of SOPD. Evaluation of the integrity of midline brain structures in patients with congenital hypopituitarism is thus thought to be important for their etiology.

摘要

一名41岁男性因甲状腺功能减退和皮质醇减少症被转诊至九州大学医院进行评估。垂体功能测试显示生长激素（GH）、促肾上腺皮质激素（ACTH）、催乳素和促甲状腺激素（TSH）缺乏。磁共振成像（MRI）显示空蝶鞍和胼胝体发育不全。临床诊断为伴有中线脑异常的垂体功能减退症。视隔-垂体发育不良（SOPD）是一种以透明隔或胼胝体发育不全、视神经发育不全和先天性下丘脑-垂体功能不全为特征的综合征。我们的病例没有眼部异常，但如今被视为SOPD的一种变异形式。因此，对于先天性垂体功能减退症患者，评估中线脑结构的完整性对其病因研究具有重要意义。