Central Diabetes Insipidus (CDI) is mainly associated with structural pathologies
of the hypothalamic-pituitary area. Etiologies underlying CDI are identified in most
patients, however idiopathic CDI is reported in 13–17% of cases after excluding other
etiologies. The Hypopituitarism ENEA Rare Observational Study (HEROS study) retrospectively
collected data of patients with idiopathic CDI from 14 pituitary centers in 9 countries.
The cohort included 92 patients (59 females 64%), mean age at diagnosis was 35.4 ±
20.7 years, and a mean follow up of 19.1 ± 13.5 years following CDI diagnosis. In
6 women, diagnosis was related to pregnancy. Of 83 patients with available data on
pituitary imaging, 40(48%) had normal sellar imaging, and 43(52%) had pathology of
the posterior pituitary or the stalk, including loss of the bright spot, posterior
pituitary atrophy or stalk enlargement. Anterior pituitary hormone deficiencies at
presentation included hypogonadism in 6 (6.5%) patients (5 females), and hypocortisolism
in one; during follow-up new anterior pituitary deficiencies developed in 6 patients.
Replacement treatment with desmopressin was given to all patients except one, usually
with an oral preparation. During follow up, no underlying disease causing CDI was
identified in any patient. Patients with idiopathic CDI following investigation at
baseline are stable with no specific etiology depicted during long-term follow-up.
