A case of hypothalamic panhypopituitarism with empty sella syndrome: case report and review of the literature.
Empty sella syndrome is frequently accompanied with pituitary dysfunction. Most of the patients with empty sella syndrome demonstrate primary pituitary or stalk dysfunction and few cases show hypothalamic dysfunction. A 71-year-old man manifested appetite loss, nausea and vomiting with hyponatremia and adrenal insufficiency. Hormonal evaluation and cranial MRI revealed a panhypopituitarism with empty sella. Intriguingly, while the response of ACTH to CRH administration was exaggerated, the response to insulin hypoglycemia was blunted. Serum PRL levels were normal. Further, decreased level of fT4, slightly elevated basal levels of TSH, and delayed response of TSH to TRH administration were observed. These findings strongly suggest that the panhypopituitarism is caused by hypothalamic dysfunction. The presence of autoantibodies to pituitary and cerebrum in the patient's serum implies an autoimmune mechanism as a pathogenesis.
Division of Diabetes, Metabolism, and Endocrinology, Department of Internal Medicine, Kobe University Graduate school of Medicine, Kobe, Japan.
Hisako
H
Komada
Masaaki
M
Yamamoto
Saki
S
Okubo
Kanto
K
Nagai
Keiji
K
Iida
Takehiro
T
Nakamura
Yushi
Y
Hirota
Kazuhiko
K
Sakaguchi
Masato
M
Kasuga
Yutaka
Y
Takahashi
D000368:Aged; D004652:Empty Sella Syndrome / Q000209:etiology*; D020803:Encephalitis, Herpes Simplex / Q000150:complications; D006801:Humans; D007018:Hypopituitarism / Q000150:complications*; D007031:Hypothalamus / Q000503:physiopathology; D008297:Male; D013973:Thyrotropin-Releasing Hormone
D013973:Thyrotropin-Releasing Hormone