Increased cerebral iron uptake in Wilson's disease: A (52)Fe-citrate PET study

Toxicity of abundant copper is the main cause of brain and liver tissue damage in patients with Wilson's disease (WD). However, there is also evidence of a disturbed iron metabolism in this genetically determined disorder. This PET study was undertaken to assess cerebral iron metabolism in WD patients. Methods: We used (52)Fe-citrate, which converts to (52)Fe-transferrin in blood plasma, to study basic pharmacokinetic features of the cerebral iron transport in 6 WD patients and in 16 healthy volunteers (control subjects). A 2-tissue-compartment model and multiple time graphic plotting were used to calculate (52)Fe-transferrin distribution volumes and transport rates. Results: Net iron uptake (Ki) from plasma into brain tissue was significantly (P <0.001) higher in WD patients (Ki [mean +/- SEM] = 15.1E-05 +/- 7.13E-05 [1/min]) than in healthy volunteers (Ki = 2.66E-05 +/- 0.351E-05 [1/min]). There was no difference of tracer iron distribution in the cerebral plasma volume between patients and healthy volunteers. Iron uptake values resulting from 2 methods to model PET data of patients and healthy volunteers were highly correlated (P <0.001). Conclusion: An abnormally increased cerebral (52)Fe-transferrin uptake was found in WD patients.