Hepatic Encephalopathy Mimicking Creutzfeldt-Jakob Disease on Laboratory, Physiological, and Imaging Evaluations.

ABSTRACT

BACKGROUND Creutzfeldt-Jakob disease (CJD) is an irreversible, neurodegenerative, prion disease presenting with cognitive, behavioral, and motor dysfunction. The clinical presentations or laboratory findings of treatable autoimmune and metabolic processes may mimic those of CJD. Hepatic encephalopathy (HE) is a complication of severe hepatic failure that is characterized by neuropsychiatric manifestations. A case of HE whose laboratory, physiological, and imaging results were similar to that of Creutzfeldt-Jakob disease (CJD) in the process of treatment for HE is presented. CASE REPORT An 84-year-old woman with hepatic encephalopathy (HE) was admitted to our hospital because of acute consciousness disturbance. She had chronic hepatitis type C, liver cirrhosis, and hepatocellular carcinoma, and had had an episode of HE once. Her severe consciousness disturbance did not improve after treatment for concurrent hyperammonemia, unlike in her previous episode. Diffusion-weighted brain magnetic resonance imaging (MRI) showed widespread hyperintensity of the whole cortex. Her electroencephalogram showed periodic sharp wave complexes (PSWCs). Both total t-tau and 14-3-3 proteins were detected in her cerebrospinal fluid. According to these clinical data, CJD was highly suspected. However, the consciousness disturbance was alleviated by the tenth day of admission, and her general condition was markedly improved, which supported the initial diagnosis of HE. CONCLUSIONS The present results suggest that treatable disorders, such as HE, should be considered before making a final diagnosis of a fatal disease such as CJD, since the spectrum of diseases that CJD mimics is vast. We should also aggressively treat patients with severe conditions from which recovery is possible.