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.

CASE SUMMARY:

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.

MANAGEMENT:

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.

OUTCOME:

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: M W O'Reilly, D J Sexton, M C Dennedy, T J Counihan, F M Finucane, T O'Brien, A W O'Regan
• Journal: QJM : monthly journal of the Association of Physicians (QJM) Vol. 108 Issue 8 Pg. 657-9 (Aug 2015) ISSN: 1460-2393 [Electronic] England
• PMID: 23417911 (Publication Type: Case Reports, Journal Article)
• Copyright: © The Author 2013. Published by Oxford University Press on behalf of the Association of Physicians. All rights reserved. For Permissions, please email: [email protected].

Chemical References

• Immunosuppressive Agents
• Tumor Necrosis Factor-alpha
• Infliximab

Topics

• Central Nervous System Diseases (complications, diagnosis, drug therapy)
• Diabetes Insipidus, Neurogenic (etiology, psychology)
• Humans
• Hypopituitarism (etiology)
• Immunosuppressive Agents (therapeutic use)
• Infliximab (therapeutic use)
• Magnetic Resonance Imaging
• Male
• Remission Induction
• Sarcoidosis (complications, diagnosis, drug therapy)
• Thirst (drug effects)
• Tumor Necrosis Factor-alpha (antagonists & inhibitors)
• Young Adult