Neurologic disorders due to brain manganese deposition in a jaundiced patient receiving long-term parenteral nutrition

Background: Neurologic and radiologic disorders have been reported in patients receiving long-term parenteral nutrition (PN). On the basis of elevated serum manganese levels, some of these abnormalities have been attributed to manganese intoxication. Alterations of the basal ganglia signal intensity on T1-weighted magnetic resonance images (MRIs) have been previously reported, but the precise nature of these alterations remains controversial although the deposition of manganese has been suggested in patients with chronic hepatic encephalopathy due to liver failure. Methods: We report the case of a patient who was receiving PN and exhibited a chronic cholestasis. Neurologic disorders appeared after several months of PN, when a hypersignal in the basal ganglia and white matter was found on T1-weighted MRIs of the brain in association with elevated serum and manganese levels. Results: Elevated autopsic concentrations of manganese were found in the radiologic abnormal cerebral areas. Conclusion: Our observation is the first demonstration of a relationship between high intracerebral manganese levels, radiologic abnormalities, and neurologic disorders during longterm PN. Moreover, serum manganese levels are not a good indicator of cerebral levels. In fact, in our patient, serum mangenese levels returned to normal, whereas those of cerebral manganese remained increased.