A Rare Case of Adipsic Diabetes Insipidus Associated with Langerhans Cell Histiocytosis

Abstract

Objective: We describe a case of adipsic diabetes insipidus, hypopituitarism, and cognitive impairment in a patient with a hypothalamic mass diagnosed with Langerhans cell histiocytosis. We aim to highlight the difficult and complex management of such a rare case. Methods: We report the initial presentation, laboratory results, imaging, management, complications, and outcome of a patient with adipsic diabetes insipidus. We conducted a literature search to review the reported cases of adipsic diabetes insipidus. Results: A previously well 36-year-old woman presented with amenorrhea, headaches, polyuria, polydipsia, and cognitive decline. Initial investigations demonstrated serum hypernatremia (156 mmol/L), hyperosmolality (322 mmol/kg), and inappropriate hypotonic polyuria (urine osmolality 100 mmol/kg, urine output 500 mL/hour). Pituitary investigations showed hyperprolactinemia and secondary hypothyroidism, hypocortisolemia, and hypogonadism. Magnetic resonance imaging brain scan revealed a 1.6 × 2.3 × 1.9 cm enhancing hypothalamic mass. She displayed an appropriate renal response to desmopressin (urine osmolality 400 to 500 mmol/kg, urine output <100 mL/hour) and thus was diagnosed with

Potential indication of chemotherapy for hypodipsia and arginine vasopressin deficiency secondary to hypothalamic-pituitary Langerhans cell histiocytosis: a case report and literature review

“…A literature search of PubMed and Google Scholar was conducted in October 2023. The following search terms were used: “adipsia,” “adipsic,” “hypodipsia, ” “hypodipsic,” “diabetes insipidus, ” “arginine vasopressin deficiency,” and “Langerhans cell histiocytosis.” We found five patients with AVD due to HP-LCH reported as adipsic ( 4 , 5 , 6 , 7 , 8 ), and no patients were hypodipsic. The clinical manifestations of patients were reviewed ( Table 1…”

Persistent hypernatremia secondary to adipsic central diabetes insipidus in a patient with herpes-induced meningoencephalitis and COVID-19 infection: a case report

“… 7 , 8 This is an extremely rare disorder, with only a few cases having been reported. 3 , 6 , 9 – 11 Because of osmoreceptor dysfunction, these patients are unable to experience thirst and to respond to an increase in osmolarity by an increase in fluid intake. 8 The osmoreceptors are located in the organum vasculosum of the lamina terminalis in the anterior hypothalamus.…”

Langerhans Cell Histiocytosis Manifests with Acute Severe Hypernatremia during Hospitalization

“…A previous case of adipsic DI caused by LCH has been previously reported. e infiltration of the thirst center in the hypothalamus could be the possible mechanism underlying adipsic DI [12]. In addition, CDI can be the first manifestation, even before LCH is diagnosed.…”