THUR 038 Encephalopathy with MRI cortical ribbon and reversible white matter hyperintensities

Abstract

We present an unusual case of encephalopathy with profound MR white matter and cortical abnormalities secondary to, and responsive to treatment for, hepatic encephalopathy alone.A 52-year-old man with known alcohol related liver disease (now abstinent), previous intravenous drug abuse and treated Hepatitis C presented over 18 months with discrete episodes of fluctuating confusion, and altered consciousness progressing to coma.Neuroimaging demonstrated symmetrical confluent white matter T2/ FLAIR hyperintensities in the posterior frontal and parietal lobes with symmetrical dWI cortical ribboning in the precentral gyrus. Due to his clinical state and the unusual neuroimaging findings he was transferred for further neurological investigation and management.On admission a serum ammonia was 111 µmol/L (normal range 16 – 60 µmol/L). Further to this a detailed systemic blood, imaging and CSF analysis did not yield an alternative diagnosis. Electroencephalography revealed generalised slowing.Serial imaging showed persistent, fluctuating, confluent, and symmetrical white matter T2/ FLAIR hyperintensities. Fluctuations in his clinical state correlated with serum ammonia levels, bowel motions, neuroimaging and the EEG findings. He was treated for hepatic encephalopathy alone, on one occasion in intensive care, with lactulose, phosphate enemas and diuretics. He was discharged home independently after a period of sustained clinical improvement.