Paraparesis, hypermanganesaemia, and polycythaemia: a novel presentation of cirrhosis

Progressive myelopathy is a rare complication of chronic hepatic disease wh ich has never been reported in the paediatric age group. We describe the 11 year course of an adolescent male with hepatic myelopathy caused by crypto genic micronodular cirrhosis. His condition has been associated with persis tent polycythaemia and extraordinary increases of whole blood manganese, wi th magnetic resonance imaging evidence of manganese deposition within the b asal ganglia and other regions of the brain. The patient has developed neit her liver failure nor parkinsonism. The pathophysiological bases of this mu ltiorgan system disorder are described.