American Journal of Internal Medicine
Volume 7, Issue 6, November 2019, Pages: 151-153
Received: Aug. 14, 2019;
Accepted: Sep. 6, 2019;
Published: Nov. 19, 2019
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Fatima Zarzour, Department of Endocrinology and Metabolism, American University of Beirut Medical Center, Beirut, Lebanon
Abir Bou Khalil, Department of Endocrinology and Metabolism, American University of Beirut Medical Center, Beirut, Lebanon
Sami Toufic Azar, Department of Endocrinology and Metabolism, American University of Beirut Medical Center, Beirut, Lebanon
Background: Central diabetes insipidus (CDI) is a rare hypothalamic-pituitary disease due to the deficiency of arginine vasopressin (AVP) synthesis of which more than one third of etiologies are unidentified. Autoimmunity is associated with one third of patients with apparently idiopathic CDI. The most common antibody detected in auto immune CDI is autoantibodies to AVP-secreting cells (AVPcAb). Stiff-person syndrome (SPS) is a rare neurological disorder with features of an autoimmune disease. It is characterized by fluctuating muscle rigidity in the trunk and limbs and a heightened sensitivity to stimuli. It is well known that SPS is associated with multiple auto immune endocrinopathy including auto immune diabetes and auto immune thyroid disease. The association between SPS and CDI is not well documented in the literature. Case information: We report a case of 51 year old female who developed CDI while being treated for SPS unmasked by high dose steroids. Result: Anti amphiphysin is the only antibody detected in our patient. Animal studies showed a high expression of amphiphysin in anterior and posterior pituitary gland, data in human are still vague .One theory is that anti-amphiphysin antibodies attack the amphiphysin molecules in the AVP secreting cells and inhibit release of AVP. Conclusion: This antibody could hint towards the autoimmune cause of CDI.
Abir Bou Khalil,
Sami Toufic Azar,
Central Diabetes Insipidus in a Patient with Stiff Person Syndrome, American Journal of Internal Medicine.
Vol. 7, No. 6,
2019, pp. 151-153.
Copyright © 2019 Authors retain the copyright of this article.
This article is an open access article distributed under the Creative Commons Attribution License (http://creativecommons.org/licenses/by/4.0/
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Malve H, Kalra S, Zargar A, Jain S, Sethi B, Chowdhury S, et al. Diabetes insipidus: The other diabetes. Indian Journal of Endocrinology and Metabolism. 2016; 20 (1): 9.
Juul KV, Bichet DG, Nielsen S, Nørgaard JP. The physiological and pathophysiological functions of renal and extrarenal vasopressin V2 receptors. American Journal of Physiology-Renal Physiology. 2014; 306 (9).
Iorgi ND, Napoli F, Allegri AEM, Olivieri I, Bertelli E, Gallizia A, et al. Diabetes Insipidus – Diagnosis and Management. Hormone Research in Paediatrics. 2012; 77 (2): 69–84.
Murdaca G, Russo R, Spanò F, Ferone D, Albertelli M, Schenone A, Contatore M, Guastalla A, De Bellis A, Garibotto G, Puppo F. Autoimmune central diabetes insipidus in a patient with ureaplasma urealyticum infection and review on new triggers of immune response. Arch Endocrinol Metab. 2015 Dec; 59 (6): 554-8.
Christ-Crain M, Bichet D G, Fenske W K, Goldman M B, Rittig S, Verbalis J G, & Verkman A S. Diabetes insipidus. Nature Reviews Disease Primers. 2019; 5 (1).
Maghnie M, Ghirardello S, Bellis AD, Iorgi ND, Ambrosini L, Secco A, et al. Idiopathic central diabetes insipidus in children and young adults is commonly associated with vasopressin-cell antibodies and markers of autoimmunity. Clinical Endocrinology. 2006; 65 (4): 470–8.
Pivonello R, Bellis AD, Faggiano A, Salle FD, Petretta M, Somma CD, et al. Central Diabetes Insipidus and Autoimmunity: Relationship between the Occurrence of Antibodies to Arginine Vasopressin-Secreting Cells and Clinical, Immunological, and Radiological Features in a Large Cohort of Patients with Central Diabetes Insipidus of Known and Unknown Etiology. The Journal of Clinical Endocrinology & Metabolism. 2003; 88 (4): 1629–36.
Garrahy A, Moran C, Thompson C et al. Diagnosis and management of central diabetes insipidus in adults. Clinical Endocrinology. 2019; 90: 23–30.
Martinez-Hernandez E, Arino H, McKeon A, et al. Clinical and immunologic investigations in patients with stiff-person spectrum disorder. JAMA Neurol. 2016; 73: 714–20.
Rathbun J, Imber J et al. Stiff Person Syndrome and Type 1 Diabetes Mellitus: a Case of the Chicken or the Egg? Journal of Genral Internal Medicine. 2019; 34 (6): 1053-10574.
Robert L, Julius C et al. Atlas of immunology. 2004 (book).
Beate L, Rudiger WV, Helmut E. M, Manfred WK. et al. Amphiphysin, a novel protein associated with synaptic vesicles. The EMBO Journal vol. 1 1 no. 7 pp. 2521-2530, 1 992.
Holst B, Madsen KL, Jansen AM, Jin C, Rickhag M, Lund VK, et al. PICK1 Deficiency Impairs Secretory Vesicle Biogenesis and Leads to Growth Retardation and Decreased Glucose Tolerance. PLoS Biology. 2013; 11 (4).
L Qiaou. Et al. Paraneoplastic neurological syndromes associated with anti-amphiphysin antibodie. October 2016.
Chin H, Quek T, Leow M. Central diabetes insipidus unmasked by corticosteroid therapy for cerebral metastases: beware the case with pituitary involvement and hypopituitarism. Journal of the Royal College of Physicians of Edinburgh. 2018; 47 (3): 247–9.