Diabetes insipidus

Abstract

Diabetes insipidus is a heterogeneous condition characterized by polyuria and polydipsia caused by a lack of secretion of vasopressin, its physiological suppression following excessive water intake, or kidney resistance to its action. In many patients, it is caused by the destruction or degeneration of the neurons that originate in the supraoptic and paraventricular nuclei of the hypothalamus. Known causes of these lesions include: germinoma or craniopharyngioma; Langerhans cell histiocytosis and sarcoidosis of the central nervous system; local inflammatory, autoimmune or vascular diseases; trauma following surgery or accident; and, rarely, genetic defects in vasopressin biosynthesis inherited as autosomal dominant or X-linked recessive traits. Thirty to fifty percent of cases are considered idiopathic. Magnetic resonance imaging (MRI) allows identification of the posterior pituitary hyperintensity and of hypothalamic-pituitary abnormalities. Thickening of the pituitary stalk is the second most common finding on MRI scans in several local inflammatory pathologies and autoimmune diseases or germinoma, but it is not specific to any single subtype. A progressive increase in the size of the anterior pituitary gland should alert physicians to the possibility that a germinoma is present, whereas a decrease can suggest the presence of an inflammatory or autoimmune process. Most children with acquired central diabetes insipidus and a thickened pituitary stalk have anterior pituitary hormone deficiencies during follow-up. Biopsy of enlarged pituitary stalk should be reserved for patients with a hypothalamic-pituitary mass and progressive thickening of the pituitary stalk, since spontaneous recovery may occur.

Cutting Edge: Neuronal Recognition by CD8 T Cells Elicits Central Diabetes Insipidus

“…2A). The clinical signs observed in chronically affected animals were strongly evocative of those seen in patients suffering from diabetes insipidus, the clinical consequence of reduced secretion of AVP or kidney resistance to its action (9). AVP is synthesized by magnocellular neurons of the supraoptic and paraventricular nuclei of the hypothalamus, which send their axons to the neurohypophysis and release this neurohormone in the blood in response to osmotic stimuli.…”

“…AVP is synthesized by magnocellular neurons of the supraoptic and paraventricular nuclei of the hypothalamus, which send their axons to the neurohypophysis and release this neurohormone in the blood in response to osmotic stimuli. Central diabetes insipidus results either from the degeneration of AVPproducing neurons or from decreased AVP release from the neurohypophysis (9). In CamK-HA mice having received the anti-HA CTLs, polyuria was corrected by injection of the AVP analogue desmopressin (Fig.…”

“…In humans, 30-50% of central diabetes insipidus cases are considered idiopathic (9). Strong but indirect evidence gives credence to the autoimmune nature of at least some of these cases.…”

Erdheim-Chester disease and pituitary involvement: a unique case and the literature

“…Our current patient improved in symptoms of CDI without DDAVP, accompanied with decreased thickening of pituitary stalk after treatment for several months. Considering CDI, especially in children and young adults, is caused by destruction or degeneration of the neurons that originate in the supraoptic and paraventricular nuclei of the hypothalamus [38] and does not tend to ameliorate even if primary disease such as inflammation, infiltration or tumor is successfully treated, our case is unique.…”

Imaging Manifestations and Techniques in Diabetes Insipidus

“…Diabetes insipidus (DI) is a clinical condition characterized by excretion of large volume of low specific gravity urine (1). Diabetes insipidus can be central in which there is deficient production of arginine vasopressin (AVP) also known as anti-diuretric hormone (ADH) or nephrogenic in which there is end organ resistance to ADH action (1).…”