IgG4-related hypothalamo-hypophysitis

Yuki Urushida; Daisuke Ishikawa; Mayuri Yanaizumi; Takumi Nakamura; Masakuni Amari; Takeshi Kawarabayashi; Masahiko Tosaka; Yoshio Ikeda; Masamitsu Takatama; Mikio Shoji

doi:10.1016/j.ensci.2021.100362

IgG4-related hypothalamo-hypophysitis

eNeurologicalSci. 2021 Jul 28:24:100362. doi: 10.1016/j.ensci.2021.100362. eCollection 2021 Sep.

Affiliations

¹ Neurology, Geriatrics Research Institute and Hospital, Maebashi, Japan.
² Neurology, Gunma University Graduate School of Medicine, Maebashi, Japan.
³ Neurosurgery, Gunma University Graduate School of Medicine, Maebashi, Japan.

Abstract

•A patient exhibited IgG4-related hypothalamo-hypophysitis.•Prominent high-signal areas of swelling were observed in the hypothalamus, tuber cinereum, infundibulum, and bilateral optic nerve systems.•MRI T1WI with contrast media demonstrated enhanced neurohypophysis and cystic swelling, and compressed anterior pituitary.•MRI findings improved rapidly after 4 days of steroid therapy.

Keywords: ACE, angiotensin converting enzyme; ACTH, adrenocorticotropic hormone; AQP4, aquaporin-4; BP, blood pressure; CT, computed tomography; DDAVP, 1-desamino-8-D-arginine vasopressin; FLAIR, fluid attenuated inversion recovery; FSH, follicle stimulating hormone; IgG4-related hypothalamo-hypophysitis; LH, luteinizing hormone; MPO-ANCA, myeloperoxidase-antineutrophil cytoplasmic antibody; MRI, magnetic resonance imaging; Neurohypophysitis; Optic tract edema; PR3-ANCA, proteinase-3-antineutrophil cytoplasmic antibody; Rapid recovery; T1WI, T1 weighted image; T2WI, T2 weighted image; sIL-2R, soluble interleukin 2 receptor.