Cognitive functioning in chronic acquired hepatocerebral degeneration

CNS involvement is frequent in patients with chronic liver disease, resulting in overt or subclinical ("minimal") encephalopathy. Occasionally, patients liver cirrhosis may develop a progressive encephalopathy known as chronic acquired hepatocerebral degeneration (CAHD), presenting with neuropsychiatric changes and movement disorders. In patients affected by CAHD cognitive dysfunction is the rule, but to date this aspect has not been systematically studied. Our aim was to characterize the neuropsychological profile of cognitive impairment associated with CAHD. Eight patients with CAHD received extensive neuropsychological assessment, far from episodes of acute liver decompensation. Their cognitive performances were compared with those of 8 patients with cirrhosis free from CAHD or overt hepatic encephalopathy (HE) and with those of 8 healthy controls matched for age, sex and educational level. Patients with CAHD revealed a significant impairment of visuo-spatial attention compared to healthy controls, and a lower performance on a single task of visual search and sequencing when compared to cirrhotics without CAHD. Our findings support the hypothesis of a linear decline in attentional performances of patients with chronic liver disease, starting from cognitively intact patients, moving toward patients with minimal HE, and finally progressing to those with overt HE and CAHD.