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

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.

Marchiafava-Bignami disease with hyperintensity on late diffusion-weighted imaging.

A 69-year-old man with a decades-long history of chronic alcohol consumption was admitted with gait disturbance (short steps and spasticity), deterioration of activity, and stuporous consciousness. Head magnetic resonance imaging (MRI) revealed hyperintensity on fluid-attenuated inversion recovery imaging in the corpus callosum and frontal white matter. The lesion later became more apparent on diffusion-weighted imaging. The clinical diagnosis was Marchiafava-Bignami disease (MBD). As temporary treatment, refraining from alcohol consumption and administration of vitamins were prescribed. The condition of the patient gradually improved. The purposes of this study were to demonstrate the clinical and radiological variety of MBD and to identify practical methods of treatment of this pathology.