Journal de pathologie clinique et expérimentale

Abstrait

An Unusual Cause of Central Diabetes Insipidus in a Young Female

Kush Dev Singh Jarial, Abhishek Hajela, Ashu Rastogi, Kirti Gupta, Louis Balan Gasper, Anil Bhansali, Pravin Salunke and Pinaki Dutta

Central Diabetes Insipidus (CDI) in adults is most commonly occurs as a result of hypothalamo- pituitary surgery, head injury or various inflammatory and infiltrative disorders. CDI with mass lesions in the sellar-suprasellar area occurs due to infiltrative disorders like lymphoma, Langerhan’s cell histiocytosis, and inflammatory disorders like hypophysitis, mass lesions like germinoma, craniopharyngioma and even metastases. Treatment and correct diagnosis depends on histopathology. Rosai-Dorfman Disease (RDD) is a rare disorder of unknown etiology characterized by abnormal proliferation of histiocytes. Extra nodal involvement is found in 40-50% of cases; with Central nervous system (CNS) involvement being uncommon hence in majority of instances intracranial disease is not suspected. Due to paucity of the reported cases, optimal treatment options are not known. We report a young female who presented with CDI, hyperprolactinemia and other features of hypopituitarism along with systemic manifestations including nodal and skeletal involvement. The patient was treated with combination of surgical debulking followed by oral glucocorticoid for 6 months with gratifying results.