关键词: IgG4-related hypophysitis central diabetes insipidus multimorbidity panhypopituitarism

Mesh : Humans Male Aged Hypopituitarism / diagnosis etiology immunology Diabetes Insipidus, Neurogenic / etiology diagnosis Immunoglobulin G / blood Deamino Arginine Vasopressin / administration & dosage Magnetic Resonance Imaging Autoimmune Hypophysitis / complications diagnosis Positron Emission Tomography Computed Tomography Hypophysitis / diagnosis complications diagnostic imaging Biomarkers / blood Immunoglobulin G4-Related Disease / complications diagnosis Treatment Outcome

来  源:   DOI:10.5692/clinicalneurol.cn-001934

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.
摘要:
一名78岁的男子抱怨亚急性全身疲劳和厌食症,复视和步态障碍。他表现出广泛的步态和小步步态,客观上没有异常的眼球运动。脑MRI显示垂体柄和腺体增大,对比度均匀增强。PET-CT显示垂体吸收FDG,纵隔淋巴结,和左肺门淋巴结。血液检查显示垂体功能减退症和高血清IgG4水平,最高可达265µmg/dl。组织病理学检查显示活检纵隔淋巴结中无IgG4阳性细胞浸润。然而,我们根据临床症状和MRI表现怀疑IgG4相关的垂体炎,用类固醇显着解决。出现了中央掩盖尿崩症,但口服去氨加压素改善。我们应该密切关注IgG4相关的垂体炎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有关的不确定的主诉,尤其是老年多发病患者。
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