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Progressive myelopathy is a rare complication of chronic
hepatic disease which has never been reported in the paediatric age group. We describe the 11 year course of an adolescent male with hepatic myelopathy caused by cryptogenic micronodular cirrhosis. His
condition has been associated with persistent polycythaemia and
extraordinary increases of whole blood manganese, with magnetic resonance imaging evidence of manganese deposition within the basal
ganglia and other regions of the brain. The patient has developed
neither liver failure nor parkinsonism. The pathophysiological bases of
this multiorgan system disorder are described.