Central Diabetes Insipidus in a Patient with Stiff Person Syndrome

Fatima Zarzour,Abir Bou Khalil ,Sami T Azar

doi:10.11648/j.ajim.20190706.13

Fatima Zarzour, Abir Bou Khalil + Show 1 more

Open Access

https://doi.org/10.11648/j.ajim.20190706.13

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Abstract

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.

Highlights

Central diabetes insipidus (CDI) is a rare hypothalamic-pituitary disease due to the deficiency of arginine vasopressin (AVP) synthesis from the hypothalamus and/or secretion from the neurohypophysis
CDI is caused by inadequate synthesis/release of AVP, often secondary to surgery or head injury which causes traumatic injury to the hypothalamus or posterior pituitary gland and destruction/degeneration of neurons originating in the supraoptic and paraventricular nuclei of the hypothalamus [3]
Rosario et al found that autoimmunity is associated with one third of patients with apparently idiopathic CDI, which should be classified as autoimmune CDI

Summary

Introduction

CDI is a rare hypothalamic-pituitary disease due to the deficiency of AVP synthesis from the hypothalamus and/or secretion from the neurohypophysis. The etiology of CDI is unknown in over one third of cases, and it is classified as idiopathic CDI. We report a case of CDI associated SPS

Case Report

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Conclusion