Those eyes don't lie: a case of osmotic demyelination syndrome in a patient with hepatic encephalopathy.

Diagnosis (Berl)

3Departments of Medicine and Pediatrics, University of Minnesota Medical School, 420 Delaware Street, SE, MMC 741, Minneapolis, MN 55455, USA.

Published: June 2016

Osmotic demyelination syndrome (ODS), previously known as central pontine myelinolysis, is a rare neurological condition characterized by demyelination of the pons or extrapontine areas including the midbrain, thalamus, basal nuclei, and cerebellum, resulting in upper motor neuron dysfunction and pseudobulbar palsy. We report a case of a 45-year-old woman with a history of alcohol dependence and end stage liver disease complicated by hepatic encephalopathy who developed symptoms suspicious of recurrent hepatic encephalopathy and experienced a generalized seizure during an inpatient stay. After 10 days of treatment with no improvement, it was noted that the patient had locked-in syndrome and that her sodium levels had rapidly risen 2 days prior. This led to a clinical suspicion of ODS, which was confirmed on T2-weighted MRI and subsequently on autopsy. In this clinical vignette, we review the clinical presentation, prognosis, and diagnostic considerations of ODS.

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http://dx.doi.org/10.1515/dx-2016-0004DOI Listing

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