Endocrine Abstracts

Introduction: Neurohormonal diabetes insipidus (DI) is a rare disorder caused by partial or total vasopressin deficiency which results in diluted urine characterized by polyuria and polydipsia. In 30–50% of the cases the cause of the disease is unknown; it is the so-called idiopathic diabetes insipidus. Other causes include: tumors of the CNS most commonly craniopharyngioma, pituitary macroadenomas, central nervous system trauma, inflammatory state of hypothalamus or pituitary gland, CNS surgery, congenital organic diseases or genetic defects. The treatment includes long-action vasopressin analogues. We present the similarity of the clinical course of infundibuloma irrespective of the patient’s gender.

Case report: A 39-year-old male perfectly mentally and physically healthy three years ago diagnosed with central diabetes insipidus with focal lesion 5×5 mm in size, confined to the pituitary stalk supporting optic chiasm and characteristic of DI low signal intensity on T1-weighted MR images of the nerves. The field of vision was normal. After a year the patient presented symptoms of hypogonadism (hypogonadotrophic hypogonadism), which required additional replacement treatment with desmopressin and parenteral testosterone with a good clinical result.

A 42-year-old female with central diabetes insipidus diagnosed at the age of 33 caused by thickening pituitary stalk to 5 mm with no accompanying symptoms. After a year of replacement therapy with desmopressin appeared the symptoms of hypogonadism in the form of secondary amenorrhea at the age of 34.

Conclusions: The primary clinical symptom of infundibuloma is diabetes insipidus followed by hypopituitarism in the course of which the gonadotrophic axis defect prevails irrespective of the patient’s gender.