Brain manganese deposition and blood levels in patients undergoing home parenteral nutrition

Background: Extrapyramidal syndrome and alterations in brain magnetic reson ance images are described in patients undergoing long-term home parenteral nutrition (HPN) and in cholestatic patients. These abnormalities have been correlated to basal ganglia manganese (Mn) accumulation. Methods: A longitu dinal 1-year study was conducted on 15 patients undergoing HPN (median dura tion, 3.8 years; range, 1.7-10; median Mn parenteral supplementation, 0.1 m g/d). Whole-blood, plasma, intra-erythrocytes, and urinary Mn concentration s were measured and brain magnetic resonance was performed at tho beginning (time 0) and after 1 year of Mn intravenous supplementation withdrawal (ti me 1). No patients showed psychosis, extrapyramidal syndrome, or cholestasi s. Results: At time zero, 10 of 15 patients (67%) showed paramagnetic accum ulation on cerebral magnetic resonance images; at time 1 there was a reduct ion of cerebral Mn accumulation. In all patients, blood-Mn levels were sign ificantly reduced after 1 year of Mn intravenous supplementation withdrawal . Conclusions: Patients receiving long-term HPN showed an elevated incidenc e of alterations in brain magnetic resonance images with a median Mn intrav enous supplementation of 0.1 mg/d. Mn supplementation withdrawal significan tly decreased metal levels in blood and brain storage. We noticed that the intra-erythrocyte Mn level was a good index of Mn status.