Lymphocytic infundibulo-neurohypophysitis with hypothalamic and optic pathway involvement: Report of a case and review of the literature
Lymphocytic infundibulo-neurohypophysitis with hypothalamic and optic pathway involvement: Report of a case and review of the literature
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DOI：
10.1016/s0090-3019(01)00647-4
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发表时间：
2002-01-01
影响因子：
--
通讯作者：
Farrell, VJR
中科院分区：
文献类型：
--
作者：
Ouma, JR;Farrell, VJR
背景淋巴细胞性腺垂体炎和淋巴细胞性漏斗神经垂体炎分别是罕见的垂体前叶和后叶自身免疫介导的疾病。前者通常表现为垂体前叶激素分泌不足，许多患者伴有视力障碍。后者表现为中枢性尿崩症。它们最常见于怀孕或产后的女性。很少有关于与怀孕无关的女性和男性的报道。病例描述我们介绍了一名患有中枢性尿崩症、全垂体功能低下和视力严重受损的未生育女性。磁共振成像显示有一个大肿块，涉及下丘脑、漏斗部、视神经、交叉和视束。手术中发现视神经通路与炎性肿块密切相关。活检组织学检查显示非特异性混合炎症浸润，主要由淋巴细胞和浆细胞组成。她对地塞米松治疗反应显着，连续影像学检查显示肿块消失，视力也有所改善。此外，她还接受了激素替代疗法。结论我们提出了一例淋巴细胞性漏斗神经垂体炎病例，其视神经通路炎症受累程度独特，并记录了对类固醇的反应。 (C) 2002 年，爱思唯尔科学公司。
BACKGROUNDLymphocytic adenohypohysitis and lymphocytic infundibulo-neurohypophysitis are rare auto-immune mediated diseases of the anterior and posterior pituitary, respectively. The former usually manifests as insufficiency of anterior pituitary hormone secretion, associated in many patients with disturbances of vision. The latter presents as diabetes insipidus of central origin. They present most commonly in pregnant or postpartum females. There have been infrequent reports in females with no association with pregnancy, and in males.CASE DESCRIPTIONWe present a nulliparous female with central diabetes insipidus, pan-hypopituitarism, and severely impaired vision. Magnetic resonance imaging demonstrated a large mass involving the hypothalamus, infundibulum, optic nerves, chiasm, and tracts. At operation, the optic pathways were found to be grossly involved in the inflammatory mass. Histological examination of a biopsy demonstrated a nonspecific, mixed inflammatory infiltrate, composed predominantly of lymphocytes and plasma cells. She responded dramatically to treatment with dexamethasone, with disappearance of the mass on serial imaging studies and improvement in vision. In addition, she received hormone replacement therapy.CONCLUSIONWe present a case of lymphocytic infundibulo-neurohypophysitis unique in the degree of optic pathway inflammatory involvement, with a documented response to steroids. (C) 2002 by Elsevier Science Inc.