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Related Concept Videos

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The response to stress—be it physical or psychological, acute or chronic—involves activation of the Hypothalamic-Pituitary-Adrenal (HPA) axis. The HPA axis is part of the neuroendocrine system because it involves both neuronal and hormonal communication. Its function is to regulate homeostatic systems—metabolic, cardiovascular, and immune—providing the necessary means to respond to a stressor.
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The pituitary is a small endocrine organ in the sphenoid bone under the hypothalamus. Primarily, the pituitary in adults has two distinct anatomical and functional regions— the anterior and posterior lobes. During human fetal development, a third pituitary gland region called the pars intermedia atrophies and disappears. However, some of its cells migrate and exist adjacent to the anterior pituitary in adults.
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Related Experiment Video

Updated: Oct 22, 2025

Preparation of Mouse Pituitary Immunogen for the Induction of Experimental Autoimmune Hypophysitis
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IgG4-related hypothalamo-hypophysitis.

Yuki Urushida1, Daisuke Ishikawa1, Mayuri Yanaizumi1

  • 1Neurology, Geriatrics Research Institute and Hospital, Maebashi, Japan.

Eneurologicalsci
|September 1, 2021
PubMed
Summary

A patient with IgG4-related hypothalamo-hypophysitis showed swelling in the brain and optic nerves. Steroid therapy led to rapid improvement in MRI findings, indicating effectiveness.

Keywords:
ACE, angiotensin converting enzymeACTH, adrenocorticotropic hormoneAQP4, aquaporin-4BP, blood pressureCT, computed tomographyDDAVP, 1-desamino-8-D-arginine vasopressinFLAIR, fluid attenuated inversion recoveryFSH, follicle stimulating hormoneIgG4-related hypothalamo-hypophysitisLH, luteinizing hormoneMPO-ANCA, myeloperoxidase-antineutrophil cytoplasmic antibodyMRI, magnetic resonance imagingNeurohypophysitisOptic tract edemaPR3-ANCA, proteinase-3-antineutrophil cytoplasmic antibodyRapid recoveryT1WI, T1 weighted imageT2WI, T2 weighted imagesIL-2R, soluble interleukin 2 receptor

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Area of Science:

  • Neuroendocrinology
  • Immunology
  • Radiology

Background:

  • IgG4-related disease (IgG4-RD) is a fibroinflammatory condition affecting various organs.
  • Hypothalamo-hypophysitis, inflammation of the hypothalamus and pituitary gland, can be a manifestation of IgG4-RD.
  • Accurate diagnosis and prompt treatment are crucial for managing pituitary dysfunction.

Purpose of the Study:

  • To present a case of IgG4-related hypothalamo-hypophysitis.
  • To describe the characteristic magnetic resonance imaging (MRI) findings.
  • To highlight the rapid response to steroid therapy.

Main Methods:

  • A single patient case was analyzed.
  • Magnetic resonance imaging (MRI) with T1-weighted imaging (T1WI) and contrast media was utilized.
  • Clinical and radiological outcomes were monitored during steroid treatment.

Main Results:

  • MRI revealed prominent high-signal swelling in the hypothalamus, tuber cinereum, infundibulum, and optic nerves.
  • Contrast-enhanced MRI showed enhanced neurohypophysis, cystic swelling, and anterior pituitary compression.
  • Significant radiological improvement was observed within 4 days of initiating steroid therapy.

Conclusions:

  • IgG4-related hypothalamo-hypophysitis can present with specific MRI features.
  • Early steroid administration is effective in managing IgG4-related hypothalamo-hypophysitis.
  • MRI is a valuable tool for diagnosing and monitoring this condition.