ONDINE'S CURSE DUE TO OSMOTIC DEMYELINATION SYNDROME

Abstract

SESSION TITLE: Critical Care 3 SESSION TYPE: Fellow Case Report Posters PRESENTED ON: 10/09/2018 01:15 PM - 02:15 PM INTRODUCTION: Osmotic Demyelination Syndrome is a life-threatening complication of sudden rise in serum osmolality after a chronic hypotonicity. The damage depends highly on the brains response to changing serum concentration and can present with many symptoms. Here we describe a patient treated for severe hyponatremia who later presented with Ondine’s curse. CASE PRESENTATION: A 67-year-old Caucasian male transferred to a tertiary facility from a rural hospital. Initially admitted and intubated for worsening shortness of breath; community-acquired pneumonia and alcohol withdrawal. History revealed a drinking habit of twelve or more bottles of alcohol daily, he also drank excessive amounts of water in the previous month with minimal eating. Laboratory data revealed serum sodium concentration was between 143 and 152 throughout hospitalization with no significant acute changes.One week after admission, multiple trails of extubation were unsuccessful and he was found to have no gag reflex, dysphagia or central hypoventilation. Therefore, Brain magnetic resonance imaging (MRI) was obtained; results showed early T2 hyperintense signal within the pons centrally in a pattern suggesting Osmotic Demyelination syndrome (ODS) (figures 1 & 2).Patient remained conscious and needed to be transferred to a long-term nursing facility after tracheostomy and percutaneous endoscopic gastrostomy. DISCUSSION: A hypotonic hyponatremia state in the body naturally forces fluid across the blood brain barrierincreasing water content. This decreased serum tonicity leads to swelling of many brain cells, mainly astrocytes. Astrocytes surround the brains capillaries with foot processes and contain the vital water channels that regulate cellular solutes and water across the blood-brain-barrier. If the brain were not able to adapt to a fall in tonicity, the cells would swell and burst causing irreversible damage. Additionally, any brain swelling would cramp the cranial cavity leading to herniation and further damage.Chronic hyponatremia leads to a more effective depletion of the osmotically active solutes that take time to replenish. So when we rapidly correct the hypotonic state in a chronically hyponatremicpatient the cells cannot osmotically compensate as well, causing them to shrink.This leads to a cascade o fapoptosis, disruption of oligodendrocytes, inflammatory cytokine release, and microglial activation. If the insult involved the respiratory center in the medulla or higher brainstem it can cause central hypoventilation syndrome which lead to decreased autonomic response to hypercapnia and hypoxiaalthough they are able to consciously control their ventilation normally and respond to non-chemoreceptive inputs for ventilation CONCLUSIONS: This case report underlines the importance of recognizing predisposing risk factors for osmotic demyelination syndrome and the potential complications if not done properly Reference #1: Jazeela Fayyaz, DO. Zab Mosenifar, M, ed. "Hypoventilation Syndromes". "Congenital central hypoventilation syndrome". Genetics Home Reference. U.S. National Library of Medicine. Reference #2: "Primary alveolar hypoventilation: Ondine's curse". A.D.A.M. Medical Encyclopedia. U.S. National Library of Medicine.Severinghaus JW; Mitchell, R. A. (1962). "Undine's curse — failure of respiratory center automaticity while awake". Clin Res. 10: 122 DISCLOSURES: No relevant relationships by Hani Badi, source=Web Response No relevant relationships by Ali Eltatawy, source=Web Response No relevant relationships by Zubair Khan, source=Web Response No relevant relationships by Tamer Said Ahmed, source=Web Response No relevant relationships by Young Sook Yoon, source=Web Response