Central diabetes insipidus is a rare disorder that can result as a consequence of diverse etiologies, including malformations, autoimmune, infiltrative(e.g. neoplastic or histiocytosis) or traumatic processes, as well as mutations in the gene encoding arginine vasopressin. Idiopathic central diabetes insipidus is a diagnosis of exclusion, one that has been made less frequently through the decades.
Idiopathic central diabetes insipidus in children and adolescent requires a frequent follow-up regimen using serial brain MRI and CSF examinations especially if an isolated pituitary stalk thickening or loss of a hyperintense signal in the posterior lobe is observed. Also, so-called "idiopathic" central diabetes insipidus warrants close follow-up to determine the etiology, especially if anterior pituitary hormone deficiencies are detected. We report a case of idiopathic central diabetes insipidus with growh hormone deficiency and loss of a hyperintense signal in the posterior lobe. He is a 13 years old. We are scheduled to follow-up with serial contrast enhanced brain MRI and CSF evaluation for the early detection of an evolving occult hypothalamic-stalk lesion. |