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漏斗神经垂体炎伴自身免疫性血小板减少症和视交叉综合征:病例报告。

Infundibuloneurohypophysitis associated with autoimmune thrombocytopenia and chiasmal syndrome: a case report.

机构信息

Department of Neurology and Psychiatry, Neurosurgery, 'Sapienza' University of Rome, Rome, Italy.

Department of Radiological, Oncological and Pathological Science, 'Sapienza' University of Rome, Rome, Italy.

出版信息

Acta Neurol Belg. 2019 Sep;119(3):337-342. doi: 10.1007/s13760-018-1007-8. Epub 2018 Aug 20.

Abstract

Lymphocytic hypophysitis is an uncommon autoimmune disease. It may involve the adenohypophysis, neurohypophysis and pituitary stalk. It is most often encountered in pregnant women. Its diagnosis and management pose a significant challenge, as its clinical manifestation and appearance in imaging studies are difficult to distinguish from more common lesion of the sellar region (e.g., pituitary adenomas). We present the case of a 42-year-old man who presented with a chiasmatic syndrome, diabetes insipidus and hypopituitarism. Brain MRI documented a sellar lesion with suprasellar extension and optic chiasm dislocation. He underwent an endoscopic endonasal transsphenoidal resection of the mass. Histopathological examination revealed a lymphocytic hypophysitis. Visual symptoms improved dramatically postoperatively with permanent diabetes insipidus and panhypopituitarism. We discuss the indication for surgical resection in patients with inflammatory lesions extending to the suprasellar region associated with visual impairment.

摘要

淋巴细胞性垂体炎是一种罕见的自身免疫性疾病。它可能累及腺垂体、神经垂体和垂体柄。它最常发生在孕妇中。其诊断和治疗极具挑战性,因为其临床表现和影像学表现难以与鞍区更常见的病变(例如,垂体腺瘤)相区别。我们报告了一例 42 岁男性患者,表现为视交叉综合征、尿崩症和垂体功能减退症。脑 MRI 记录了一个鞍内病变,伴有鞍上延伸和视交叉移位。他接受了内镜经鼻蝶窦入路切除该肿块。组织病理学检查显示为淋巴细胞性垂体炎。术后视觉症状显著改善,但出现永久性尿崩症和全垂体功能减退症。我们讨论了对伴有视力损害的延伸至鞍上区域的炎症性病变患者进行手术切除的适应证。

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