[A case of suspected IgG4-related hypophysitis presented with panhypopituitarism and central diabetes insipidus].

Q4 Medicine Clinical Neurology Pub Date : 2024-06-27 Epub Date: 2024-05-25 DOI:10.5692/clinicalneurol.cn-001934
Hitoshi Kubo, Haruki Ohtsubo, Akira Shiraoka, Masashi Watanabe, Itaru Kyoraku, Kensho Okamoto
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Abstract

A 78-year-old man complained of subacute general fatigue and anorexia, following diplopia and gait disturbance. He demonstrated wide-based and small-stepped gait without objectively abnormal ocular movements. Brain ‍MRI showed enlargement of the pituitary stalk and gland with uniform contrast enhancement. PET-CT showed FDG ‍uptake in the pituitary gland, mediastinal lymph nodes, and left hilar lymph nodes. Blood investigations revealed panhypopituitarism and high serum IgG4 levels up to 265 ‍mg/dl. Histopathological examination revealed no IgG4-positive cell infiltration in the biopsied mediastinal lymph nodes. However, we suspected IgG4-associated hypophysitis based on the clinical symptoms and MRI findings, which were markedly resolved with steroid. Central masked diabetes insipidus was manifested, but was improved with oral desmopressin. We should pay close attention to the fact that IgG4-related hypophysitis may present with various symptoms regarded as indefinite complaints related to aging or underlying diseases, especially in elderly patients with multimorbidity.

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[一例疑似 IgG4 相关性垂体功能减退症的病例,伴有泛垂体功能减退症和中枢性尿崩症]。
一名 78 岁的男性主诉亚急性全身乏力和厌食,继而出现复视和步态障碍。他的步态表现为大步和小步,眼球运动无客观异常。脑‍MRI显示垂体柄和腺体增大,对比度均匀增强。PET-CT 显示垂体、纵隔淋巴结和左侧肺门淋巴结有 FDG ‍ 摄取。血液检查显示泛垂体功能减退,血清IgG4水平高达265‍mg/dl。组织病理学检查显示,活检的纵隔淋巴结中没有 IgG4 阳性细胞浸润。然而,根据患者的临床症状和核磁共振成像结果,我们怀疑患者患有 IgG4 相关性肾上腺皮质功能减退症。患者出现了中枢性掩蔽性糖尿病,但口服去氨加压素后病情有所好转。我们应该密切注意的是,IgG4 相关性肾上腺皮质功能减退症可能会出现各种症状,这些症状被认为是与衰老或潜在疾病相关的不确定主诉,尤其是在患有多病的老年患者中。
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来源期刊
Clinical Neurology
Clinical Neurology Medicine-Neurology (clinical)
CiteScore
0.30
自引率
0.00%
发文量
147
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