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Wilson Disease
Anne G. Osborn, MD, FACR; James M. Provenzale, MD
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KEY FACTS

  • Terminology

    • Imaging

      • Top Differential Diagnoses

        • Clinical Issues

          • Diagnostic Checklist

            TERMINOLOGY

            • Abbreviations

              • Wilson disease (WD)
            • Synonyms

              • Progressive hepatolenticular degeneration
            • Definitions

              • Autosomal recessive inherited copper metabolism disorder characterized by abnormal accumulation of copper in various tissues

            IMAGING

            • General Features

              • CT Findings

                • MR Findings

                  • Nuclear Medicine Findings

                    • Imaging Recommendations

                      DIFFERENTIAL DIAGNOSIS

                        PATHOLOGY

                        • General Features

                          • Staging, Grading, & Classification

                            • Gross Pathologic & Surgical Features

                              • Microscopic Features

                                CLINICAL ISSUES

                                • Presentation

                                  • Demographics

                                    • Natural History & Prognosis

                                      • Treatment

                                        DIAGNOSTIC CHECKLIST

                                        • Image Interpretation Pearls

                                          Selected References

                                          1. Wu F et al: Wilson's Disease: A Comprehensive Review of the Molecular Mechanisms. Int J Mol Sci. 16(3):6419-6431, 2015
                                          2. Fritzsch D et al: Seven-tesla magnetic resonance imaging in Wilson disease using quantitative susceptibility mapping for measurement of copper accumulation. Invest Radiol. 49(5):299-306, 2014
                                          3. Dusek P et al: The neurotoxicity of iron, copper and manganese in Parkinson's and Wilson's diseases. J Trace Elem Med Biol. ePub, 2014
                                          4. Gupta S: Cell therapy to remove excess copper in Wilson's disease. Ann N Y Acad Sci. 1315:70-80, 2014
                                          5. Hermann W: Morphological and functional imaging in neurological and non-neurological Wilson's patients. Ann N Y Acad Sci. 1315:24-9, 2014
                                          6. Kozić DB et al: Reversible lesions in the brain parenchyma in Wilson's disease confirmed by magnetic resonance imaging: earlier administration of chelating therapy can reduce the damage to the brain. Neural Regen Res. 9(21):1912-6, 2014
                                          7. Krishna SH et al: Congenital genetic inborn errors of metabolism presenting as an adult or persisting into adulthood: neuroimaging in the more common or recognizable disorders. Semin Ultrasound CT MR. 35(2):160-91, 2014
                                          8. Patell R et al: Atypical neuroimaging in Wilson's disease. BMJ Case Rep. 2014, 2014
                                          9. Pulai S et al: Clinical features, MRI brain, and MRS abnormalities of drug-naïve neurologic Wilson's disease. Neurol India. 62(2):153-8, 2014
                                          10. Trocello JM et al: Extensive striatal, cortical, and white matter brain MRI abnormalities in Wilson disease. Neurology. 81(17):1557, 2013
                                          11. Lorincz MT: Recognition and treatment of neurologic Wilson's disease. Semin Neurol. 32(5):538-43, 2012
                                          12. Ala A et al: Wilson's disease. Lancet. 369(9559):397-408, 2007
                                          13. Kim TJ et al: MR imaging of the brain in Wilson disease of childhood: findings before and after treatment with clinical correlation. AJNR Am J Neuroradiol. 27(6):1373-8, 2006
                                          14. Sinha S et al: Wilson's disease: cranial MRI observations and clinical correlation. Neuroradiology. 48(9):613-21, 2006
                                          Related Anatomy
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                                          Related Differential Diagnoses
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                                          References
                                          Tables

                                          Tables

                                          KEY FACTS

                                          • Terminology

                                            • Imaging

                                              • Top Differential Diagnoses

                                                • Clinical Issues

                                                  • Diagnostic Checklist

                                                    TERMINOLOGY

                                                    • Abbreviations

                                                      • Wilson disease (WD)
                                                    • Synonyms

                                                      • Progressive hepatolenticular degeneration
                                                    • Definitions

                                                      • Autosomal recessive inherited copper metabolism disorder characterized by abnormal accumulation of copper in various tissues

                                                    IMAGING

                                                    • General Features

                                                      • CT Findings

                                                        • MR Findings

                                                          • Nuclear Medicine Findings

                                                            • Imaging Recommendations

                                                              DIFFERENTIAL DIAGNOSIS

                                                                PATHOLOGY

                                                                • General Features

                                                                  • Staging, Grading, & Classification

                                                                    • Gross Pathologic & Surgical Features

                                                                      • Microscopic Features

                                                                        CLINICAL ISSUES

                                                                        • Presentation

                                                                          • Demographics

                                                                            • Natural History & Prognosis

                                                                              • Treatment

                                                                                DIAGNOSTIC CHECKLIST

                                                                                • Image Interpretation Pearls

                                                                                  Selected References

                                                                                  1. Wu F et al: Wilson's Disease: A Comprehensive Review of the Molecular Mechanisms. Int J Mol Sci. 16(3):6419-6431, 2015
                                                                                  2. Fritzsch D et al: Seven-tesla magnetic resonance imaging in Wilson disease using quantitative susceptibility mapping for measurement of copper accumulation. Invest Radiol. 49(5):299-306, 2014
                                                                                  3. Dusek P et al: The neurotoxicity of iron, copper and manganese in Parkinson's and Wilson's diseases. J Trace Elem Med Biol. ePub, 2014
                                                                                  4. Gupta S: Cell therapy to remove excess copper in Wilson's disease. Ann N Y Acad Sci. 1315:70-80, 2014
                                                                                  5. Hermann W: Morphological and functional imaging in neurological and non-neurological Wilson's patients. Ann N Y Acad Sci. 1315:24-9, 2014
                                                                                  6. Kozić DB et al: Reversible lesions in the brain parenchyma in Wilson's disease confirmed by magnetic resonance imaging: earlier administration of chelating therapy can reduce the damage to the brain. Neural Regen Res. 9(21):1912-6, 2014
                                                                                  7. Krishna SH et al: Congenital genetic inborn errors of metabolism presenting as an adult or persisting into adulthood: neuroimaging in the more common or recognizable disorders. Semin Ultrasound CT MR. 35(2):160-91, 2014
                                                                                  8. Patell R et al: Atypical neuroimaging in Wilson's disease. BMJ Case Rep. 2014, 2014
                                                                                  9. Pulai S et al: Clinical features, MRI brain, and MRS abnormalities of drug-naïve neurologic Wilson's disease. Neurol India. 62(2):153-8, 2014
                                                                                  10. Trocello JM et al: Extensive striatal, cortical, and white matter brain MRI abnormalities in Wilson disease. Neurology. 81(17):1557, 2013
                                                                                  11. Lorincz MT: Recognition and treatment of neurologic Wilson's disease. Semin Neurol. 32(5):538-43, 2012
                                                                                  12. Ala A et al: Wilson's disease. Lancet. 369(9559):397-408, 2007
                                                                                  13. Kim TJ et al: MR imaging of the brain in Wilson disease of childhood: findings before and after treatment with clinical correlation. AJNR Am J Neuroradiol. 27(6):1373-8, 2006
                                                                                  14. Sinha S et al: Wilson's disease: cranial MRI observations and clinical correlation. Neuroradiology. 48(9):613-21, 2006