Rinsho Shinkeigaku (Clinical Neurology)

Case Report

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

Hitoshi Kubo, M.D.1)2), Haruki Ohtsubo, M.D.1), Akira Shiraoka, M.D.1), Masashi Watanabe, M.D.1), Itaru Kyoraku, M.D.1) and Kensho Okamoto, M.D.1)

1)Department of Neurology Ehime Prefectural Central Hospital
2)Department of Internal Medicine Kitauwa Hospital

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.
Full Text of this Article in Japanese PDF (1567K)

(CLINICA NEUROL, 64: 398−402, 2024)
key words: IgG4-related hypophysitis, panhypopituitarism, central diabetes insipidus, multimorbidity

(Received: 11-Oct-23)