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Corticobasal Degeneration
Santhosh Gaddikeri, MDMiral D. Jhaveri, MD, MBA
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KEY FACTS

  • Terminology

    • Imaging

      • Top Differential Diagnoses

        • Pathology

          • Clinical Issues

            TERMINOLOGY

            • Abbreviations

              • Corticobasal degeneration (CBD)
            • Synonyms

              • Corticobasal ganglionic degeneration (CBGD)
              • Corticodentatonigral degeneration with neuronal achromasia
            • Definitions

              • Progressive neurodegenerative disease
                • Presents with cognitive dysfunction & progressive "asymmetrical" parkinsonism
                • Characterized pathologically by cortical & striatal tau protein accumulation (tauopathy)

            IMAGING

            • General Features

              • Imaging Recommendations

                • CT Findings

                  • MR Findings

                    • Nuclear Medicine Findings

                      DIFFERENTIAL DIAGNOSIS

                        PATHOLOGY

                        • General Features

                          • Gross Pathologic & Surgical Features

                            • Microscopic Features

                              CLINICAL ISSUES

                              • Presentation

                                • Demographics

                                  • Natural History & Prognosis

                                    • Treatment

                                      DIAGNOSTIC CHECKLIST

                                      • Consider

                                        • Image Interpretation Pearls

                                          Selected References

                                          1. Fabbrini G et al: Progressive supranuclear palsy, multiple system atrophy and corticobasal degeneration. Handb Clin Neurol. 165:155-77, 2019
                                          2. Saranza GM et al: Corticobasal degeneration. Int Rev Neurobiol. 149:87-136, 2019
                                          3. Ali F et al: Corticobasal degeneration: key emerging issues. J Neurol. 265(2):439-45, 2018
                                          4. Booth TC et al: The role of functional dopamine-transporter SPECT imaging in parkinsonian syndromes, part 2. AJNR Am J Neuroradiol. 36(2):236-44, 2015
                                          5. Josephs KA: Key emerging issues in progressive supranuclear palsy and corticobasal degeneration. J Neurol. 262(3):783-8, 2015
                                          6. Shimohata T et al: [Criteria for the diagnosis of corticobasal degeneration.] Brain Nerve. 67(4):513-23, 2015
                                          7. Grijalvo-Perez AM et al: Corticobasal degeneration. Semin Neurol. 34(2):160-73, 2014
                                          8. Shimohata T et al: [Clinical aspects of corticobasal syndrome.] Brain Nerve. 65(1):31-40, 2013
                                          9. Erbetta A et al: Diffusion tensor imaging shows different topographic involvement of the thalamus in progressive supranuclear palsy and corticobasal degeneration. AJNR Am J Neuroradiol. 30(8):1482-7, 2009
                                          10. Vitali P et al: Neuroimaging in dementia. Semin Neurol. 28(4):467-83, 2008
                                          11. Koyama M et al: Imaging of corticobasal degeneration syndrome. Neuroradiology. 49(11):905-12, 2007
                                          12. Klaffke S et al: Dopamine transporters, D2 receptors, and glucose metabolism in corticobasal degeneration. Mov Disord. 21(10):1724-7, 2006
                                          13. Mahapatra RK et al: Corticobasal degeneration. Lancet Neurol. 3(12):736-43, 2004
                                          14. Laureys S et al: Fluorodopa uptake and glucose metabolism in early stages of corticobasal degeneration. J Neurol. 246(12):1151-8, 1999
                                          15. Hauser RA et al: Magnetic resonance imaging of corticobasal degeneration. J Neuroimaging. 6(4):222-6, 1996
                                          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

                                                    TERMINOLOGY

                                                    • Abbreviations

                                                      • Corticobasal degeneration (CBD)
                                                    • Synonyms

                                                      • Corticobasal ganglionic degeneration (CBGD)
                                                      • Corticodentatonigral degeneration with neuronal achromasia
                                                    • Definitions

                                                      • Progressive neurodegenerative disease
                                                        • Presents with cognitive dysfunction & progressive "asymmetrical" parkinsonism
                                                        • Characterized pathologically by cortical & striatal tau protein accumulation (tauopathy)

                                                    IMAGING

                                                    • General Features

                                                      • Imaging Recommendations

                                                        • CT Findings

                                                          • MR Findings

                                                            • Nuclear Medicine Findings

                                                              DIFFERENTIAL DIAGNOSIS

                                                                PATHOLOGY

                                                                • General Features

                                                                  • Gross Pathologic & Surgical Features

                                                                    • Microscopic Features

                                                                      CLINICAL ISSUES

                                                                      • Presentation

                                                                        • Demographics

                                                                          • Natural History & Prognosis

                                                                            • Treatment

                                                                              DIAGNOSTIC CHECKLIST

                                                                              • Consider

                                                                                • Image Interpretation Pearls

                                                                                  Selected References

                                                                                  1. Fabbrini G et al: Progressive supranuclear palsy, multiple system atrophy and corticobasal degeneration. Handb Clin Neurol. 165:155-77, 2019
                                                                                  2. Saranza GM et al: Corticobasal degeneration. Int Rev Neurobiol. 149:87-136, 2019
                                                                                  3. Ali F et al: Corticobasal degeneration: key emerging issues. J Neurol. 265(2):439-45, 2018
                                                                                  4. Booth TC et al: The role of functional dopamine-transporter SPECT imaging in parkinsonian syndromes, part 2. AJNR Am J Neuroradiol. 36(2):236-44, 2015
                                                                                  5. Josephs KA: Key emerging issues in progressive supranuclear palsy and corticobasal degeneration. J Neurol. 262(3):783-8, 2015
                                                                                  6. Shimohata T et al: [Criteria for the diagnosis of corticobasal degeneration.] Brain Nerve. 67(4):513-23, 2015
                                                                                  7. Grijalvo-Perez AM et al: Corticobasal degeneration. Semin Neurol. 34(2):160-73, 2014
                                                                                  8. Shimohata T et al: [Clinical aspects of corticobasal syndrome.] Brain Nerve. 65(1):31-40, 2013
                                                                                  9. Erbetta A et al: Diffusion tensor imaging shows different topographic involvement of the thalamus in progressive supranuclear palsy and corticobasal degeneration. AJNR Am J Neuroradiol. 30(8):1482-7, 2009
                                                                                  10. Vitali P et al: Neuroimaging in dementia. Semin Neurol. 28(4):467-83, 2008
                                                                                  11. Koyama M et al: Imaging of corticobasal degeneration syndrome. Neuroradiology. 49(11):905-12, 2007
                                                                                  12. Klaffke S et al: Dopamine transporters, D2 receptors, and glucose metabolism in corticobasal degeneration. Mov Disord. 21(10):1724-7, 2006
                                                                                  13. Mahapatra RK et al: Corticobasal degeneration. Lancet Neurol. 3(12):736-43, 2004
                                                                                  14. Laureys S et al: Fluorodopa uptake and glucose metabolism in early stages of corticobasal degeneration. J Neurol. 246(12):1151-8, 1999
                                                                                  15. Hauser RA et al: Magnetic resonance imaging of corticobasal degeneration. J Neuroimaging. 6(4):222-6, 1996