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Canavan Disease
P. Ellen Grant, MD
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KEY FACTS

  • Terminology

    • Imaging

      • Top Differential Diagnoses

        • Pathology

          • Clinical Issues

            TERMINOLOGY

            • Abbreviations

              • Canavan disease (CD)
            • Synonyms

              • Spongiform leukodystrophy, spongy degeneration of CNS, Canavan-van Bogaert-Bertrand disease, aspartoacylase deficiency, ASPA deficiency, ASP deficiency, aminoacylase 2 deficiency, ACY2 deficiency
            • Definitions

              • Progressive fatal autosomal-recessive spongiform leukodystrophy

            IMAGING

            • General Features

              • CT Findings

                • MR 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. Guo F et al: Ablating N-acetylaspartate prevents leukodystrophy in a Canavan disease model. Ann Neurol. 77(5):884-8, 2015
                                        2. Baslow MH et al: Canavan disease, a rare early-onset human spongiform leukodystrophy: insights into its genesis and possible clinical interventions. Biochimie. 95(4):946-56, 2013
                                        3. van der Knaap MS et al: Defining and categorizing leukoencephalopathies of unknown origin: MR imaging approach. Radiology. 213(1):121-33, 1999
                                        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

                                                    • Canavan disease (CD)
                                                  • Synonyms

                                                    • Spongiform leukodystrophy, spongy degeneration of CNS, Canavan-van Bogaert-Bertrand disease, aspartoacylase deficiency, ASPA deficiency, ASP deficiency, aminoacylase 2 deficiency, ACY2 deficiency
                                                  • Definitions

                                                    • Progressive fatal autosomal-recessive spongiform leukodystrophy

                                                  IMAGING

                                                  • General Features

                                                    • CT Findings

                                                      • MR 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. Guo F et al: Ablating N-acetylaspartate prevents leukodystrophy in a Canavan disease model. Ann Neurol. 77(5):884-8, 2015
                                                                              2. Baslow MH et al: Canavan disease, a rare early-onset human spongiform leukodystrophy: insights into its genesis and possible clinical interventions. Biochimie. 95(4):946-56, 2013
                                                                              3. van der Knaap MS et al: Defining and categorizing leukoencephalopathies of unknown origin: MR imaging approach. Radiology. 213(1):121-33, 1999