title: Radiological remission and recovery of thirst appreciation after infliximab therapy in adipsic diabetes insipidus secondary to neurosarcoidosis.
abstract: BACKGROUND
  Neurosarcoidosis is a rare and aggressive variant of systemic sarcoidosis which may result in hypothalamic-pituitary dysfunction. We report a case of hypothalamic hypopituitarism secondary to neurosarcoidosis complicated by adipsic diabetes insipidus (ADI). Initiation of anti-tumour necrosis factor-α (TNF-α) therapy resulted in both radiological disease remission and recovery of osmoregulated thirst appreciation after 3 months.
  
  
  METHODS
  A 22-year-old man was referred to the endocrinology service with profound weight gain, polyuria and lethargy. Biochemical testing confirmed anterior hypopituitarism while posterior pituitary failure was confirmed by hypotonic polyuria responding to desmopressin. Magnetic resonance imaging (MRI) demonstrated extensive hypothalamic infiltration; neurosarcoidosis was confirmed histologically after excisional cervical lymph node biopsy. Osmoregulated thirst appreciation was normal early in the disease course despite severe hypotonic polyuria. However, subsequent subjective loss of thirst appreciation and development of severe hypernatraemia in the setting of normal cognitive function indicated onset of ADI.
  
  
  RESULTS
  Clinical management involved daily weighing, regular plasma sodium measurement, fixed daily fluid intake and oral desmopressin. We initiated immunosuppressive therapy with pulsed intravenous anti-TNF-α therapy (infliximab) after multidisciplinary team consultation.
  
  
  RESULTS
  Infliximab therapy resulted in successful radiological disease remission and complete recovery of osmoregulated thirst appreciation. This was confirmed by subjective return of thirst response and maintenance of plasma sodium in the normal range in the absence of close biochemical monitoring.
authors: From the Department of Endocrinology, m.oreilly@bham.ac.uk.
authors: M W
authors: MW
authors: O'Reilly
authors: Department of Respiratory Medicine and.
authors: D J
authors: DJ
authors: Sexton
authors: From the Department of Endocrinology.
authors: M C
authors: MC
authors: Dennedy
authors: Department of Neurology, University College Hospital/National University of Ireland, Galway, Republic of Ireland.
authors: T J
authors: TJ
authors: Counihan
authors: From the Department of Endocrinology.
authors: F M
authors: FM
authors: Finucane
authors: From the Department of Endocrinology.
authors: T
authors: T
authors: O'Brien
authors: Department of Respiratory Medicine and.
authors: A W
authors: AW
authors: O'Regan
mesh_terms: D002493:Central Nervous System Diseases / Q000150:complications* / Q000175:diagnosis / Q000188:drug therapy; D020790:Diabetes Insipidus, Neurogenic / Q000209:etiology* / Q000523:psychology; D006801:Humans; D007018:Hypopituitarism / Q000209:etiology; D007166:Immunosuppressive Agents / Q000627:therapeutic use*; D000069285:Infliximab / Q000627:therapeutic use*; D008279:Magnetic Resonance Imaging; D008297:Male; D012074:Remission Induction; D012507:Sarcoidosis / Q000150:complications* / Q000175:diagnosis / Q000188:drug therapy; D013894:Thirst / Q000187:drug effects*; D014409:Tumor Necrosis Factor-alpha / Q000037:antagonists & inhibitors; D055815:Young Adult
chemical_list: D007166:Immunosuppressive Agents; D014409:Tumor Necrosis Factor-alpha; D000069285:Infliximab