Neurosarcoidosis-associated central diabetes insipidus masked by adrenal insufficiency

Abstract

Central diabetes insipidus (CDI) is an infrequent complication of neurosarcoidosis (NS). Its presentation may be masked by adrenal insufficiency (AI) and uncovered by subsequent steroid replacement. A 45-year-old woman with a history of NS presented 2 weeks after abrupt cessation of prednisone with nausea, vomiting, decreased oral intake and confusion. She was diagnosed with secondary AI and intravenous hydrocortisone was promptly begun. Over the next few days, however, the patient developed severe thirst and polyuria exceeding 6 L of urine per day, accompanied by hypernatraemia and hypo-osmolar urine. She was presumed to have CDI due to NS, and intranasal desmopressin was administered. This eventually normalised her urine output and serum sodium. The patient was discharged improved on intranasal desmopressin and oral prednisone. AI may mask the manifestation of CDI because low serum cortisol impairs renal-free water clearance. Steroid replacement reverses this process and unmasks an underlying CDI.

Unusual Presentation of Central Diabetes Insipidus in a Patient With Neurosarcoidosis

“…Sarcoidosis is a multisystem noncaseating granuloma-forming disorder with a debate between unknown etiology and immune-mediated. 1 - 3 Central nervous system involvement is seen in <10% of sarcoidosis patients. 1 The hypothalamic-pituitary axes can be affected leading to neuroendocrine manifestations, such as diabetes insipidus (DI).…”

Pituitary metastasis: central diabetes insipidus unmasked by corticosteroids – case series and review of literature

“…This 'masking' phenomenon could be due to a multitude of factors, both from ADH-dependent and ADH-independent mechanisms, resulting in impaired renal-free water clearance. Recognition of this phenomenon in patients with adrenal deficiency and risk factors for developing CDI is important in early diagnosis and management of this phenomenon [6].…”

Magnetic resonance imaging and symptoms in patients with neurosarcoidosis and central diabetes insipidus

“…Table I provides a summary and Table II provides a detailed list of the characteristics of the 20 patients who were diagnosed with neurosarcoidosis with CDI. The patients included 12 men [8-12, 14, 15, 17] and eight women [7,10,13,16,18,19], with a mean age of 33 years. The first symptom was polyuria in 13 patients [7][8][9][11][12][13][14][15][16][17] and polydipsia in eight patients [7][8][9][11][12][13][14][15].…”

“…Steroid therapy was administered to 17 patients [8][9][10][11][12][14][15][16][17][18][19], infliximab was administered to two patients [17,19], cyclophosphamide was administered to one patient [10], and methotrexate was administered to one patient [19]. Desmopressin (1-desamino-8-D-arginine vasopressin) (DDAVP) was used to treat the symptoms of CDI in 19 patients [7][8][9][10][11][12][13][14][16][17][18][19]. Fourteen patients showed improvement in their abnormal brain MRI findings [8][9][10][11][12][13][15][16][17], but no patients showed improvement in the loss of high signal intensity in the posterior lobe of the pituitary.…”