J Korean Med Sci.  2016 Aug;31(8):1345-1348. 10.3346/jkms.2016.31.8.1345.

Hypopituitarism Presenting as Adrenal Insufficiency and Hypothyroidism in a Patient with Wilson's Disease: a Case Report

• Lee HW
• Kang JD
• Yeo CW
• Yoon SW
• Lee KJ
• Choi MK

• KMID: 2373766
• DOI: http://doi.org/10.3346/jkms.2016.31.8.1345

Abstract

Wilson's disease typically presents symptoms associated with liver damage or neuropsychiatric disturbances, while endocrinologic abnormalities are rare. We report an unprecedented case of hypopituitarism in a patient with Wilson's disease. A 40-year-old woman presented with depression, general weakness and anorexia. Laboratory tests and imaging studies were compatible with liver cirrhosis due to Wilson's disease. Basal hormone levels and pituitary function tests indicated secondary hypothyroidism and adrenal insufficiency due to hypopituitarism. Brain MRI showed T2 hyperintense signals in both basal ganglia and midbrain but the pituitary imaging was normal. She is currently receiving chelation therapy along with thyroid hormone and steroid replacement. There may be a relationship between Wilson's disease and hypopituitarism. Copper deposition or secondary neuronal damage in the pituitary may be a possible explanation for this theory.

Keyword

Hepatolenticular Degeneration; Hypopituitarism; Hypothyroidism; Adrenal Insufficiency

MeSH Terms

Adrenal Insufficiency/diagnosis/etiology
Adult
Brain/diagnostic imaging
Depression/etiology
Female
Hepatolenticular Degeneration/*complications
Humans
Hypopituitarism/complications/*diagnosis/drug therapy
Hypothyroidism/diagnosis/etiology
Liver Cirrhosis/complications/diagnostic imaging
Magnetic Resonance Imaging
Steroids/therapeutic use
Thyrotropin-Releasing Hormone/therapeutic use
Steroids
Thyrotropin-Releasing Hormone

Figure

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  Fig. 1 Copper deposits in the Descemet's membrane of the cornea (Kayser-Fleischer ring).

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  Fig. 2 Bilateral symmetric increased signals of the basal ganglia on T2 weighted MR image (A) while no focal lesion was found in the pituitary gland (B).

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