Kubo Hitoshi, Ohtsubo Haruki, Shiraoka Akira, Watanabe Masashi, Kyoraku Itaru, Okamoto Kensho
Department of Neurology Ehime Prefectural Central Hospital.
Department of Internal Medicine Kitauwa Hospital.
Rinsho Shinkeigaku. 2024 Jun 27;64(6):398-402. doi: 10.5692/clinicalneurol.cn-001934. Epub 2024 May 25.
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.
一名78岁男性在出现复视和步态障碍后,主诉亚急性全身乏力和厌食。他表现为宽基小步步态,眼部运动无客观异常。脑部MRI显示垂体柄和腺体增大,对比增强均匀。PET-CT显示垂体、纵隔淋巴结和左肺门淋巴结有FDG摄取。血液检查显示全垂体功能减退,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无IgG4阳性细胞浸润。然而,基于临床症状和MRI表现,我们怀疑为IgG4相关性垂体炎,使用类固醇治疗后症状明显缓解。出现了中枢性隐匿性尿崩症,但口服去氨加压素后有所改善。我们应密切关注这样一个事实,即IgG4相关性垂体炎可能表现出各种被视为与衰老或基础疾病相关的不确定主诉的症状,尤其是在患有多种疾病的老年患者中。