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Wilson Disease
Surjith Vattoth, MD, FRCR
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KEY FACTS

  • Terminology

    • Imaging

      • Top Differential Diagnoses

        • Pathology

          • Clinical Issues

            • Diagnostic Checklist

              TERMINOLOGY

              • Abbreviations

                • Wilson disease (WD)
              • Synonyms

                • Progressive hepatolenticular degeneration
              • Definitions

                • Autosomal recessive copper metabolism disorder with 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. Kalita J et al: Predictors of seizure in Wilson disease: a clinico-radiological and biomarkers study. Neurotoxicology. 71:87-92, 2018
                                            2. Dusek P et al: Brain iron accumulation in Wilson disease: a post mortem 7 Tesla MRI - histopathological study. Neuropathol Appl Neurobiol. 43(6):514-32, 2017
                                            3. Wu F et al: Wilson's disease: a comprehensive review of the molecular mechanisms. Int J Mol Sci. 16(3):6419-31, 2015
                                            4. 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
                                            5. Dusek P et al: The neurotoxicity of iron, copper and manganese in Parkinson's and Wilson's diseases. J Trace Elem Med Biol. 31:193-203, 2014
                                            6. Gupta S: Cell therapy to remove excess copper in Wilson's disease. Ann N Y Acad Sci. 1315:70-80, 2014
                                            7. Hermann W: Morphological and functional imaging in neurological and non-neurological Wilson's patients. Ann N Y Acad Sci. 1315:24-9, 2014
                                            8. 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
                                            9. 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
                                            10. Parekh JR et al: Wilson's disease: 'face of giant panda' and 'trident' signs together. Oxf Med Case Reports. 2014(1):16-7, 2014
                                            11. Patell R et al: Atypical neuroimaging in Wilson's disease. BMJ Case Rep. 2014, 2014
                                            12. 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
                                            13. Zhou XX et al: Characterizing brain mineral deposition in patients with Wilson disease using susceptibility-weighted imaging. Neurol India. 62(4):362-6, 2014
                                            14. Trocello JM et al: Extensive striatal, cortical, and white matter brain MRI abnormalities in Wilson disease. Neurology. 81(17):1557, 2013
                                            15. Lorincz MT: Recognition and treatment of neurologic Wilson's disease. Semin Neurol. 32(5):538-43, 2012
                                            16. Ala A et al: Wilson's disease. Lancet. 369(9559):397-408, 2007
                                            17. 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
                                            18. Sinha S et al: Wilson's disease: cranial MRI observations and clinical correlation. Neuroradiology. 48(9):613-21, 2006
                                            19. Jacobs DA et al: The "double panda sign" in Wilson's disease. Neurology. 61(7):969, 2003
                                            Related Anatomy
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                                            Related Differential Diagnoses
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                                            References
                                            Tables

                                            Tables

                                            KEY FACTS

                                            • Terminology

                                              • Imaging

                                                • Top Differential Diagnoses

                                                  • Pathology

                                                    • Clinical Issues

                                                      • Diagnostic Checklist

                                                        TERMINOLOGY

                                                        • Abbreviations

                                                          • Wilson disease (WD)
                                                        • Synonyms

                                                          • Progressive hepatolenticular degeneration
                                                        • Definitions

                                                          • Autosomal recessive copper metabolism disorder with 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. Kalita J et al: Predictors of seizure in Wilson disease: a clinico-radiological and biomarkers study. Neurotoxicology. 71:87-92, 2018
                                                                                      2. Dusek P et al: Brain iron accumulation in Wilson disease: a post mortem 7 Tesla MRI - histopathological study. Neuropathol Appl Neurobiol. 43(6):514-32, 2017
                                                                                      3. Wu F et al: Wilson's disease: a comprehensive review of the molecular mechanisms. Int J Mol Sci. 16(3):6419-31, 2015
                                                                                      4. 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
                                                                                      5. Dusek P et al: The neurotoxicity of iron, copper and manganese in Parkinson's and Wilson's diseases. J Trace Elem Med Biol. 31:193-203, 2014
                                                                                      6. Gupta S: Cell therapy to remove excess copper in Wilson's disease. Ann N Y Acad Sci. 1315:70-80, 2014
                                                                                      7. Hermann W: Morphological and functional imaging in neurological and non-neurological Wilson's patients. Ann N Y Acad Sci. 1315:24-9, 2014
                                                                                      8. 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
                                                                                      9. 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
                                                                                      10. Parekh JR et al: Wilson's disease: 'face of giant panda' and 'trident' signs together. Oxf Med Case Reports. 2014(1):16-7, 2014
                                                                                      11. Patell R et al: Atypical neuroimaging in Wilson's disease. BMJ Case Rep. 2014, 2014
                                                                                      12. 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
                                                                                      13. Zhou XX et al: Characterizing brain mineral deposition in patients with Wilson disease using susceptibility-weighted imaging. Neurol India. 62(4):362-6, 2014
                                                                                      14. Trocello JM et al: Extensive striatal, cortical, and white matter brain MRI abnormalities in Wilson disease. Neurology. 81(17):1557, 2013
                                                                                      15. Lorincz MT: Recognition and treatment of neurologic Wilson's disease. Semin Neurol. 32(5):538-43, 2012
                                                                                      16. Ala A et al: Wilson's disease. Lancet. 369(9559):397-408, 2007
                                                                                      17. 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
                                                                                      18. Sinha S et al: Wilson's disease: cranial MRI observations and clinical correlation. Neuroradiology. 48(9):613-21, 2006
                                                                                      19. Jacobs DA et al: The "double panda sign" in Wilson's disease. Neurology. 61(7):969, 2003