Osmotic Demyelination Syndrome Without Significant Sodium Fluctuation in a Patient with NASH Cirrhosis (1328)

Objective: To present a case of osmotic demyelination syndrome (ODS) in a patient with nonalcoholic steatohepatitis (NASH) cirrhosis without clinically significant changes in serum sodium and subsequent improvement with intravenous immunoglobulin therapy (IVIG). Background: ODS is a disorder encompassing central pontine myelinolysis and extrapontine myelinolysis, in which rapid shifts in serum sodium (>8mEq/24hr) result in the demyelination of the pons and extrapontine regions, usually in the context of chronic hyponatremia. Clinical symptoms range from encephalopathy to seizures, and patients can present with pontine or extrapontine features. Recently both IVIG and plasmapheresis have shown potential therapeutic benefit in limited case reports. Design/Methods: NA Results: A 68-year-old male was admitted for acute encephalopathy and mild dysphagia in the setting of decompensated NASH cirrhosis. Serum sodium was 127 and serum ammonia was 205 at the time of admission. He was treated for presumed hepatic encephalopathy with the standard of care including lactulose and rifaximin, with some symptomatic improvement. No critical sodium levels or rapid shifts in sodium (highest was 7mEq/24hr) were detected throughout his prolonged hospital stay. Approximately three weeks after admission, the patient developed dysarthria, right-sided hyperreflexia and progressive generalized weakness. Subsequent MRI brain revealed T2 FLAIR signal abnormalities in the pons, thalami, and right midbrain, consistent with ODS. The patient was then started on IVIG treatment (0.4g/kg × 5 doses) with improvement in dysarthria by time of discharge on hospital day 36, and improvement in strength and pathologic reflexes by outpatient follow up at 5 weeks. Conclusions: This presents a unique case of ODS in a patient with cirrhosis without significant fluctuation in serum sodium. Thus, more conservative sodium correction goals should be considered in these patients. Furthermore, this case adds to the literature for the potential success with IVIG in patients who do not improve with supportive care. Disclosure: Dr. Gullo has nothing to disclose. Dr. Kamdar has nothing to disclose. Dr. Thakur has nothing to disclose. Dr. Kisanuki has nothing to disclose. Dr. Alhafez has nothing to disclose.