[Creutzfeldt Jakob disease and hepatic encephalopathy: coexistence in the same patient]

Since detection of a series of cases of a new variant of Creutzfeldt Jakob disease (CJD) in the United Kingdom, prionic diseases are frequently mentioned by the media. The neurologists always consider them in daily clinical practice, although the incidence is relatively low (0.5-2 cases/1,000,000 inhabitants/year for CJD). In Spain, in recent years, we have seen an increased number of probable and possible cases, perhaps due to it always being included in the differential diagnosis, and to the greater use of diagnostic tests such as 14-3-3 protein in cerebrospinal fluid (CSF). There is also a reduction in the number of definite cases, probably due to the reticence to carrying out necropsies. In the differential diagnosis, one must remember structural, infectious, degenerative, toxic, vascular and metabolic alterations, including hepatic encephalopathy (HE).

We report the case of a woman in whom CJD and EH co existed. This made diagnosis difficult. It was finally confirmed on necropsy.