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Adipsic diabetes insipidus in adult patients

Abstract

Introduction

Adipsic diabetes insipidus (ADI) is a very rare disorder, characterized by hypotonic polyuria due to arginine vasopressin (AVP) deficiency and failure to generate the sensation of thirst in response to hypernatraemia. As the sensation of thirst is the key homeostatic mechanism that prevents hypernatraemic dehydration in patients with untreated diabetes insipidus (DI), adipsia leads to failure to respond to aquaresis with appropriate fluid intake. This predisposes to the development of significant hypernatraemia, which is the typical biochemical manifestation of adipsic DI.

Methods

A literature search was performed to review the background, etiology, management and associated complications of this rare condition.

Results

ADI has been reported to occur in association with clipping of an anterior communicating artery aneurysm following subarachnoid haemorrhage, major hypothalamic surgery, traumatic brain injury and toluene exposure among other conditions. Management is very difficult and patients are prone to marked changes in plasma sodium concentration, in particular to the development of severe hypernatraemia. Associated hypothalamic disorders, such as severe obesity, sleep apnoea and thermoregulatory disorders are often observed in patients with ADI.

Conclusion

The management of ADI is challenging and is associated with significant morbidity and mortality. Prognosis is variable; hypothalamic complications lead to early death in some patients, but recent reports highlight the possibility of recovery of thirst.

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References

  1. Behan LA, Sherlock M, Moyles P et al (2015) Abnormal plasma sodium concentrations in patients treated with desmopressin for cranial diabetes insipidus: results of a long-term retrospective study. Eur J Endocrinol 172:243–250. doi:10.1530/EJE-14-0719

    CAS  Article  PubMed  Google Scholar 

  2. Miljic D, Miljic P, Doknic M et al Adipsic diabetes insipidus and venous thromboembolism (VTE): recommendations for addressing its hypercoagulability. Hormones (Athens) 13:420–423

  3. Crowley RK, Sherlock M, Agha A et al (2007) Clinical insights into adipsic diabetes insipidus: a large case series. Clin Endocrinol 66:475–482. doi:10.1111/j.1365-2265.2007.02754.x

    CAS  Google Scholar 

  4. Baylis PH, Thompson CJ (1988) Osmoregulation of vasopressin secretion and thirst in health and disease. Clin Endocrinol 29:549–576

    CAS  Article  Google Scholar 

  5. McKenna K, Thompson C (1998) Osmoregulation in clinical disorders of thirst appreciation. Clin Endocrinol 49:139–152

    CAS  Google Scholar 

  6. Zimmerman EA, Ma LY, Nilaver G (1987) Anatomical basis of thirst and vasopressin secretion. Kidney Int Suppl 21:S14–S19

    CAS  PubMed  Google Scholar 

  7. Hammond DN, Moll GW, Robertson GL, Chelmicka-Schorr E (1986) Hypodipsic hypernatremia with normal osmoregulation of vasopressin. N Engl J Med 315:433–436. doi:10.1056/NEJM198608143150706

    CAS  Article  PubMed  Google Scholar 

  8. Zimmerman EA, Nilaver G, Hou-Yu A, Silverman AJ (1984) Vasopressinergic and oxytocinergic pathways in the central nervous system. Fed Proc 43:91–96

    CAS  PubMed  Google Scholar 

  9. Sofroniew MV (1980) Projections from vasopressin, oxytocin, and neurophysin neurons to neural targets in the rat and human. J Histochem Cytochem 28:475–478

    CAS  Article  PubMed  Google Scholar 

  10. Egan G, Silk T, Zamarripa F et al (2003) Neural correlates of the emergence of consciousness of thirst. Proc Natl Acad Sci USA 100:15241–15246. doi:10.1073/pnas.2136650100

    CAS  Article  PubMed  PubMed Central  Google Scholar 

  11. Farrell MJ, Zamarripa F, Shade R et al (2008) Effect of aging on regional cerebral blood flow responses associated with osmotic thirst and its satiation by water drinking: a PET study. Proc Natl Acad Sci 105:382–387. doi:10.1073/pnas.0710572105

    CAS  Article  PubMed  Google Scholar 

  12. Robinson BW, Mishkin M (1968) Alimentary responses to forebrain stimulation in monkeys. Exp Brain Res 4:330–366

    CAS  Article  PubMed  Google Scholar 

  13. Hollis JH, McKinley MJ, D’Souza M et al (2008) The trajectory of sensory pathways from the lamina terminalis to the insular and cingulate cortex: a neuroanatomical framework for the generation of thirst. Am J Physiol Regul Integr Comp Physiol 294:R1390–R1401. doi:10.1152/ajpregu.00869.2007

    CAS  Article  PubMed  Google Scholar 

  14. Pastuskovas CV, Cassell MD, Johnson AK, Thunhorst RL (2003) Increased cellular activity in rat insular cortex after water and salt ingestion induced by fluid depletion. Am J Physiol Regul Integr Comp Physiol 284:R1119–R1125. doi:10.1152/ajpregu.00189.2002

    CAS  Article  PubMed  Google Scholar 

  15. Sorenson CA, Ellison GD (1970) Striatal organization of feeding behavior in the decorticate rat. Exp Neurol 29:162–174

    CAS  Article  PubMed  Google Scholar 

  16. Sewards TV, Sewards MA (2003) Representations of motivational drives in mesial cortex, medial thalamus, hypothalamus and midbrain. Brain Res Bull 61:25–49

    Article  PubMed  Google Scholar 

  17. Thompson CJ, Baylis PH (1987) Thirst in diabetes insipidus: clinical relevance of quantitative assessment. QJM 65:853–862

    CAS  PubMed  Google Scholar 

  18. Smith D, Finucane F, Phillips J, et al (2004) Abnormal regulation of thirst and vasopressin secretion following surgery for craniopharyngioma. Clin Endocrinol 61:273–279. doi:10.1111/j.1365-2265.2004.02086.x

    CAS  Article  Google Scholar 

  19. Gill G, Baylis P, Burn J (1985) A case of “essential” hypernatraemia due to resetting of the osmostat. Clin Endocrinol 22:545–551

    CAS  Article  Google Scholar 

  20. Thompson CJ, Freeman J, Record CO, Baylis PH (1987) Hypernatraemia due to a reset osmostat for vasopressin release and thirst, complicated by nephrogenic diabetes insipidus. Postgrad Med J 63:979–982

    CAS  Article  PubMed  PubMed Central  Google Scholar 

  21. Robertson GL, Aycinena P, Zerbe RL (1982) Neurogenic disorders of osmoregulation. Am J Med 72:339–353

    CAS  Article  PubMed  Google Scholar 

  22. McIver B, Connacher A, Whittle I et al (1991) Adipsic hypothalamic diabetes insipidus after clipping of anterior communicating artery aneurysm. BMJ 303:1465–1467

    CAS  Article  PubMed  PubMed Central  Google Scholar 

  23. Pearce SH, Argent NB, Baylis PH (1991) Chronic hypernatremia due to impaired osmoregulated thirst and vasopressin secretion. Acta Endocrinol 125:234–239

    CAS  PubMed  Google Scholar 

  24. Ball SG, Vaidja B, Baylis PH (1997) Hypothalamic adipsic syndrome: diagnosis and management. Clin Endocrinol 47:405–409

    CAS  Article  Google Scholar 

  25. Perlmutter D, Rhoton AL (1976) Microsurgical anatomy of anterior cerebral anterior communicating recurrent artery complex. Surg Forum 27:464–465

    CAS  PubMed  Google Scholar 

  26. Eisenberg Y, Frohman LA (2015) Adipsic diabetes insipidus: a review. Endocr Pract. doi:10.4158/EP15940.RA

    PubMed Central  Google Scholar 

  27. Sinha A, Ball S, Jenkins A et al (2011) Objective assessment of thirst recovery in patients with adipsic diabetes insipidus. Pituitary 14:307–311. doi:10.1007/s11102-011-0294-3

    CAS  Article  PubMed  Google Scholar 

  28. Cuesta M, Gupta S, Salehmohamed R et al (2016) Heterogenous patterns of recovery of thirst in adult patients with adipsic diabetes insipidus. QJM 109:303–308. doi:10.1093/qjmed/hcv175

    CAS  Article  PubMed  Google Scholar 

  29. Sherlock M, Agha A, Crowley R et al (2006) Adipsic diabetes insipidus following pituitary surgery for a macroprolactinoma. Pituitary 9:59–64. doi:10.1007/s11102-006-8280-x

    CAS  Article  PubMed  Google Scholar 

  30. Macías Batista A, Martínez Martín FJ, de Pablos Velasco PL (1999) [Diabetes insipidus and adipsic hypernatremia in a patient with a craniopharyngioma]. An Med Interna 16:87–88

    PubMed  Google Scholar 

  31. Arai K, Akimoto H, Inokami T, et al (1999) Marked hypernatremia in suprasellar germinoma lacking a sense of thirst. Nihon Jinzo Gakkai Shi 41:804–812

    CAS  PubMed  Google Scholar 

  32. Ronconi GF, Ronconi M, Stella M et al [Neurogenic hypernatremia with adipsia and cerebral malformations in a child with ectrodactyly-ectodermal dysplasia-cleft lip-palate syndrome]. Pediatr Med Chir 7:893–897

  33. Masera N, Grant DB, Stanhope R, Preece MA (1994) Diabetes insipidus with impaired osmotic regulation in septo-optic dysplasia and agenesis of the corpus callosum. Arch Dis Child 70:51–53

    CAS  Article  PubMed  PubMed Central  Google Scholar 

  34. Steinbrugger B, Kurz R (1983) “Essential hypernatremia” as a result of on increased osmoreceptor threshold in a boy with Pierre-Robin disease and corpus callosum agenesis. Pädiatrie und Pädologie 18:181–185

    CAS  PubMed  Google Scholar 

  35. Komatsu H, Miyake H, Kakita S, Ikuta H (2001) Hypoplasia of the corpus callosum associated with adipsic hypernatremia and hypothalamic hypogonadotropinism: a case report and review of the literature. Pediatr Int 43:683–687

    CAS  Article  PubMed  Google Scholar 

  36. Radetti G, Rizza F, Mengarda G, Pittschieler K (1991) Adipsic hypernatremia in two sisters. Am J Dis Child 145:321–325

    CAS  PubMed  Google Scholar 

  37. Kaltsas GA, Powles TB, Evanson J et al (2000) Hypothalamo-pituitary abnormalities in adult patients with langerhans cell histiocytosis: clinical, endocrinological, and radiological features and response to treatment. J Clin Endocrinol Metab 85:1370–1376. doi:10.1210/jcem.85.4.6501

    CAS  Article  PubMed  Google Scholar 

  38. Teelucksingh S, Steer CR, Thompson CJ et al (1991) Hypothalamic syndrome and central sleep apnoea associated with toluene exposure. QJM 78:185–190

    CAS  PubMed  Google Scholar 

  39. Seckl J, Dunger D (1989) Postoperative diabetes insipidus. BMJ 298:2–3

    CAS  Article  PubMed  PubMed Central  Google Scholar 

  40. Baylis PH, Robertson GL (1980) Plasma vasopressin response to hypertonic saline infusion to assess posterior pituitary function. J R Soc Med 73:255–260

    CAS  PubMed  PubMed Central  Google Scholar 

  41. Thompson CJ, Selby P, Baylis PH (1991) Reproducibility of osmotic and nonosmotic tests of vasopressin secretion in men. Am J Physiol 260:R533–R539

    CAS  PubMed  Google Scholar 

  42. Smith D, McKenna K, Moore K et al (2002) Baroregulation of vasopressin release in adipsic diabetes insipidus. J Clin Endocrinol Metab 87:4564–4568. doi:10.1210/jc.2002-020090

    CAS  Article  PubMed  Google Scholar 

  43. Crowley RK, Woods C, Fleming M, et al (2011) Somnolence in adult craniopharyngioma patients is a common, heterogeneous condition that is potentially treatable. Clin Endocrinol 74:750–755. doi:10.1111/j.1365-2265.2011.03993.x

    CAS  Article  Google Scholar 

  44. Zantut-Wittmann DE, Garmes HM, Panzan AD et al (2007) Severe rhabdomyolysis due to adipsic hypernatremia after craniopharyngioma surgery. Arq Bras Endocrinol Metabol 51:1175–1179

    Article  PubMed  Google Scholar 

  45. Bode HH, Harley BM, Crawford JD (1971) Restoration of normal drinking behavior by chlorpropamide in patients with hypodipsia and diabetes insipidus. Am J Med 51:304–313

    CAS  Article  PubMed  Google Scholar 

  46. Nandi M, Harrington AR (1978) Successful treatment of hypernatremic thirst deficiency with chlorpropamide. Clin Nephrol 10:90–95

    CAS  PubMed  Google Scholar 

  47. Moses AM, Howanitz J, van Gemert M, Miller M (1973) Clofibrate-induced antidiuresis. J Clin Invest 52:535–542. doi:10.1172/JCI107213

    CAS  Article  PubMed  PubMed Central  Google Scholar 

  48. Johnston S, Burgess J, McMillan T, Greenwood R (1991) Management of adipsia by a behavioural modification technique. J Neurol Neurosurg Psychiatry 54:272–274

    CAS  Article  PubMed  PubMed Central  Google Scholar 

  49. Verbalis JG, Goldsmith SR, Greenberg A et al (2013) Diagnosis, evaluation, and treatment of hyponatremia: expert panel recommendations. Am J Med 126:S1–S42. doi:10.1016/j.amjmed.2013.07.006

    Article  PubMed  Google Scholar 

  50. O’Reilly MW, Sexton DJ, Dennedy MC et al (2015) Radiological remission and recovery of thirst appreciation after infliximab therapy in adipsic diabetes insipidus secondary to neurosarcoidosis. QJM 108:657–659. doi:10.1093/qjmed/hct023

    Article  PubMed  Google Scholar 

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Acknowledgements

This research did not receive any specific grant from any funding agency in the public, commercial or not-for-profit sector.

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Correspondence to Christopher J. Thompson.

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The authors declare that there is no conflict of interest that could be perceived as prejudicing the impartiality of the review.

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This article does not contain any studies with human participants or animals performed by any of the authors. All procedures performed in prior studies involving human participants were in accordance with the ethical standards of the institutional and/or national research committee and with the 1964 Helsinki declaration and its later amendments or comparable ethical standards.

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Cuesta, M., Hannon, M.J. & Thompson, C.J. Adipsic diabetes insipidus in adult patients. Pituitary 20, 372–380 (2017). https://doi.org/10.1007/s11102-016-0784-4

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Keywords

  • Diabetes insipidus
  • Adipsia
  • Vasopressin
  • Thirst
  • Hypernatraemia