Acquired (Non– Wilsonian) hepatocerebral degeneration is common in patients with large porto–systemic shunts and usually does not respond to therapy for hepatic encephalopathy. The neuropsychiatric changes include apathy, psychomotor retardation, memory failure and deficits in attention and concentration suggestive of a subcortical dementia. The pathogenesis of the disease is not known but diversion of portal blood in to the systemic circulation appears to underlie the condition. It is conceivable that both Wilson‘s disease and AHD are characterised by an early stage neuropathological process mainly affecting the basal ganglia, where MRI detectable hepatocerebral degeneration is slowly reversible and liver transplantation can rapidly improve neurological symptoms.