NEUROLOGIC DISORDERS DUE TO BRAIN MANGANESE DEPOSITION IN A JAUNDICEDPATIENT RECEIVING LONG-TERM PARENTERAL-NUTRITION

Background: Neurologic and radiologic disorders have been reported in patients receiving long-term parenteral nutrition (PN). On the basis o f elevated serum manganese levels, some of these abnormalities have be en attributed to manganese intoxication. Alterations of the basal gang lia signal intensity on T1-weighted magnetic resonance images (MRIs) h ave been previously reported, but the precise nature of these alterati ons remains controversial although the deposition of manganese has bee n suggested in patients with chronic hepatic encephalopathy due to liv er 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 a nd white matter was found on T1-weighted MRIs of the brain in associat ion with elevated serum and manganese levels. Results: Elevated autops ic concentrations of manganese were found in the radiologic abnormal c erebral areas. Conclusion: Our observation is the first demonstration of a relationship between high intracerebral manganese levels, radiolo gic abnormalities, and neurologic disorders during longterm PN. Moreov er, 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.