Neurosarcoidosis With Panhypopituitarism: Two Cases and Literature Review.

JCEM case reports Pub Date : 2024-08-06 eCollection Date: 2024-08-01 DOI:10.1210/jcemcr/luae141
Ewelina Niedzialkowska, Tatjana Blazin, Daniel Shelden, Eric D Buras
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Abstract

Neurosarcoidosis (NS) with hypothalamic-pituitary (HP) involvement (HP-NS) is a rare clinical condition, conferring variable hormonal deficits that are typically irreversible. Here, we present 2 cases of NS with panhypopituitarism. The first patient presented with cauda equina syndrome and arginine vasopressin deficiency, while the second developed recurrent optic neuritis and vision loss in the setting of a sellar mass. In the first case, neurological symptoms resolved after therapy with high-dose glucocorticoids, infliximab, and methotrexate; while in the second, visual restoration followed resection of the granulomatous tissue and immunosuppressive therapy. In both cases, pituitary dysfunction persisted despite neurological improvement. We contextualized the presentations and outcomes through a literature review of HP-NS case reports and case series. This revealed high rates of extraneurologic sarcoidosis in HP-NS patients with panhypopituitarism, while underscoring the need for hormonal replacement-as endocrinopathies rarely respond to sarcoidosis-directed immunosuppression.

伴有垂体功能减退症的神经肉芽肿病:两例病例和文献综述
神经肉芽肿病(NS)伴下丘脑-垂体(HP)受累(HP-NS)是一种罕见的临床病症,可导致不同程度的荷尔蒙失调,通常是不可逆的。在此,我们介绍两例伴有泛垂体功能障碍的NS病例。第一例患者出现马尾综合征和精氨酸加压素缺乏症,第二例患者在蝶窦肿块的情况下出现复发性视神经炎和视力下降。第一例患者在接受大剂量糖皮质激素、英夫利昔单抗和甲氨蝶呤治疗后,神经系统症状得到缓解;第二例患者在切除肉芽肿组织并接受免疫抑制治疗后,视力得到恢复。在这两个病例中,尽管神经功能有所改善,但垂体功能障碍依然存在。通过对 HP-NS 病例报告和系列病例的文献回顾,我们了解了这些病例的表现和结果。这揭示了在患有泛垂体功能障碍的HP-NS患者中,神经外肉芽肿病的发病率很高,同时强调了激素替代的必要性--因为内分泌病很少对肉芽肿病导向的免疫抑制产生反应。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
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