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神经结节病伴垂体功能减退。

Neurosarcoidosis presenting with hypopituitarism.

机构信息

Department of Internal Medicine, The University of British Columbia, Vancouver, British Columbia, Canada

Department of Neuroradiology, The University of British Columbia, Vancouver, British Columbia, Canada.

出版信息

BMJ Case Rep. 2020 Oct 4;13(10):e235077. doi: 10.1136/bcr-2020-235077.

Abstract

Hypothalamo-pituitary sarcoidosis is a rare manifestation of sarcoidosis, usually presenting alongside other symptoms of neurosarcoidosis. We describe the case of a 58-year-old man from Ghana who presented with progressive gait disturbance, cognitive dysfunction, hypothermia and bradycardia. He was found to have pituitary stalk thickening on imaging, and lymph node biopsy identified non-caseating granulomatous disease. Serology revealed gonadotropin deficiency, hypothyroidism and central adrenal insufficiency. Treatment with immunomodulatory therapy resulted in resolution of findings on imaging and improved cognition, though pituitary function never recovered. Treatment for his sarcoidosis unfortunately resulted in recurrent infections and avascular necrosis. Work-up, management and ongoing care required multidisciplinary cooperation between the admitting internal medicine team, infectious diseases, respirology, rheumatology, endocrinology and neurology.

摘要

脑垂体-垂体肉瘤是肉状病的一种罕见表现,通常与神经肉状病的其他症状一起出现。我们描述了一名来自加纳的 58 岁男子的病例,他表现为进行性步态障碍、认知功能障碍、低温和心动过缓。影像学检查发现垂体柄增厚,淋巴结活检发现非干酪样肉芽肿性疾病。血清学检查显示促性腺激素缺乏、甲状腺功能减退和中枢肾上腺功能不全。免疫调节治疗后,影像学发现的病变得到缓解,认知功能得到改善,但垂体功能从未恢复。不幸的是,他的肉状病的治疗导致反复感染和缺血性坏死。入院内科团队、传染病学、呼吸内科、风湿病学、内分泌学和神经病学之间需要多学科合作进行检查、治疗和持续护理。

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本文引用的文献

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Neurosarcoidosis.神经结节病
Semin Respir Crit Care Med. 2017 Aug;38(4):499-513. doi: 10.1055/s-0037-1604165. Epub 2017 Jul 27.
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Imaging of neurosarcoidosis: common, uncommon, and rare.神经结节病的影像学表现:常见、不常见及罕见情况。
Clin Radiol. 2016 Jan;71(1):96-106. doi: 10.1016/j.crad.2015.09.007. Epub 2015 Oct 23.

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