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Pediatrics
Diagnosis
Brain
Congenital Malformations
Focal Cortical Dysplasia
Focal Cortical Dysplasia
Luke L. Linscott, MD
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KEY FACTS

  • Terminology

    • Imaging

      • Pathology

        • Clinical Issues

          TERMINOLOGY

          • Definitions

            • Focal cortical dysplasia (FCD)
              • Cortical disorganization & abnormal laminar architecture
                • Type 1: Isolated alterations in cortical organization & lamination
                • Type 2: Isolated alterations in cortical organization & lamination with dysmorphic neurons
                • Type 3: FCD in association with other lesions

          IMAGING

          • General Features

            • CT Findings

              • Nuclear Medicine Findings

                • Imaging Recommendations

                  DIFFERENTIAL DIAGNOSIS

                    PATHOLOGY

                    • General Features

                      • Staging, Grading, & Classification

                        CLINICAL ISSUES

                        • Presentation

                          • Demographics

                            • Natural History & Prognosis

                              • Treatment

                                DIAGNOSTIC CHECKLIST

                                • Consider

                                  • Image Interpretation Pearls

                                    Selected References

                                    1. Tahta A et al: Focal cortical dysplasia: etiology, epileptogenesis, classification, clinical presentation, imaging, and management. Childs Nerv Syst. 36(12):2939-47, 2020
                                    2. Jayalakshmi S et al: Focal cortical dysplasia and refractory epilepsy: role of multimodality imaging and outcome of surgery. AJNR Am J Neuroradiol. 40(5):892-8, 2019
                                    3. Chen X et al: Quantitative volume-based morphometry in focal cortical dysplasia: a pilot study for lesion localization at the individual level. Eur J Radiol. 105:240-5, 2018
                                    4. Jin B et al: Automated detection of focal cortical dysplasia type II with surface-based magnetic resonance imaging postprocessing and machine learning. Epilepsia. 59(5):982-92, 2018
                                    5. Najm IM et al: Review: The international consensus classification of focal cortical dysplasia - a critical update 2018. Neuropathol Appl Neurobiol. 44(1):18-31, 2018
                                    6. Wong-Kisiel LC et al: Challenges in managing epilepsy associated with focal cortical dysplasia in children. Epilepsy Res. 145:1-17, 2018
                                    7. Isler C et al: Comparison of clinical features and surgical outcome in focal cortical dysplasia type 1 and type 2. Epilepsy Res. 136:130-6, 2017
                                    8. Lim JS et al: Somatic mutations in TSC1 and TSC2 cause focal cortical dysplasia. Am J Hum Genet. 100(3):454-72, 2017
                                    9. Maynard LM et al: Epilepsy prevalence and severity predictors in MRI-identified focal cortical dysplasia. Epilepsy Res. 132:41-9, 2017
                                    10. Leach JL et al: Imaging spectrum of cortical dysplasia in children. Semin Roentgenol. 49(1):99-111, 2014
                                    11. Leach JL et al: Magnetic resonance imaging abnormalities in the resection region correlate with histopathological type, gliosis extent, and postoperative outcome in pediatric cortical dysplasia. J Neurosurg Pediatr. 14(1):68-80, 2014
                                    12. Mellerio C et al: 3T MRI improves the detection of transmantle sign in type 2 focal cortical dysplasia. Epilepsia. 55(1):117-22, 2014
                                    13. Mellerio C et al: The power button sign: a newly described central sulcal pattern on surface rendering MR images of type 2 focal cortical dysplasia. Radiology. 140773, 2014
                                    14. Kadom N et al: Utility of magnetization transfer T1 imaging in children with seizures. AJNR Am J Neuroradiol. 34(4):895-8, 2013
                                    15. Colombo N et al: Focal cortical dysplasia type IIa and IIb: MRI aspects in 118 cases proven by histopathology. Neuroradiology. 54(10):1065-77, 2012
                                    16. Mellerio C et al: Optimizing MR imaging detection of type 2 focal cortical dysplasia: best criteria for clinical practice. AJNR Am J Neuroradiol. 33(10):1932-8, 2012
                                    17. Bernasconi A et al: Advances in MRI for 'cryptogenic' epilepsies. Nat Rev Neurol. 7(2):99-108, 2011
                                    18. Blumcke I et al: The clinicopathologic spectrum of focal cortical dysplasias: a consensus classification proposed by an ad hoc Task Force of the ILAE Diagnostic Methods Commission. Epilepsia. 52(1):158-74, 2011
                                    19. Kim YH et al: Neuroimaging in identifying focal cortical dysplasia and prognostic factors in pediatric and adolescent epilepsy surgery. Epilepsia. 52(4):722-7, 2011
                                    20. Raybaud C et al: Imaging surgical epilepsy in children. Childs Nerv Syst. 22(8):786-809, 2006
                                    Related Anatomy
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                                    Related Differential Diagnoses
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                                    References
                                    Tables

                                    Tables

                                    KEY FACTS

                                    • Terminology

                                      • Imaging

                                        • Pathology

                                          • Clinical Issues

                                            TERMINOLOGY

                                            • Definitions

                                              • Focal cortical dysplasia (FCD)
                                                • Cortical disorganization & abnormal laminar architecture
                                                  • Type 1: Isolated alterations in cortical organization & lamination
                                                  • Type 2: Isolated alterations in cortical organization & lamination with dysmorphic neurons
                                                  • Type 3: FCD in association with other lesions

                                            IMAGING

                                            • General Features

                                              • CT Findings

                                                • Nuclear Medicine Findings

                                                  • Imaging Recommendations

                                                    DIFFERENTIAL DIAGNOSIS

                                                      PATHOLOGY

                                                      • General Features

                                                        • Staging, Grading, & Classification

                                                          CLINICAL ISSUES

                                                          • Presentation

                                                            • Demographics

                                                              • Natural History & Prognosis

                                                                • Treatment

                                                                  DIAGNOSTIC CHECKLIST

                                                                  • Consider

                                                                    • Image Interpretation Pearls

                                                                      Selected References

                                                                      1. Tahta A et al: Focal cortical dysplasia: etiology, epileptogenesis, classification, clinical presentation, imaging, and management. Childs Nerv Syst. 36(12):2939-47, 2020
                                                                      2. Jayalakshmi S et al: Focal cortical dysplasia and refractory epilepsy: role of multimodality imaging and outcome of surgery. AJNR Am J Neuroradiol. 40(5):892-8, 2019
                                                                      3. Chen X et al: Quantitative volume-based morphometry in focal cortical dysplasia: a pilot study for lesion localization at the individual level. Eur J Radiol. 105:240-5, 2018
                                                                      4. Jin B et al: Automated detection of focal cortical dysplasia type II with surface-based magnetic resonance imaging postprocessing and machine learning. Epilepsia. 59(5):982-92, 2018
                                                                      5. Najm IM et al: Review: The international consensus classification of focal cortical dysplasia - a critical update 2018. Neuropathol Appl Neurobiol. 44(1):18-31, 2018
                                                                      6. Wong-Kisiel LC et al: Challenges in managing epilepsy associated with focal cortical dysplasia in children. Epilepsy Res. 145:1-17, 2018
                                                                      7. Isler C et al: Comparison of clinical features and surgical outcome in focal cortical dysplasia type 1 and type 2. Epilepsy Res. 136:130-6, 2017
                                                                      8. Lim JS et al: Somatic mutations in TSC1 and TSC2 cause focal cortical dysplasia. Am J Hum Genet. 100(3):454-72, 2017
                                                                      9. Maynard LM et al: Epilepsy prevalence and severity predictors in MRI-identified focal cortical dysplasia. Epilepsy Res. 132:41-9, 2017
                                                                      10. Leach JL et al: Imaging spectrum of cortical dysplasia in children. Semin Roentgenol. 49(1):99-111, 2014
                                                                      11. Leach JL et al: Magnetic resonance imaging abnormalities in the resection region correlate with histopathological type, gliosis extent, and postoperative outcome in pediatric cortical dysplasia. J Neurosurg Pediatr. 14(1):68-80, 2014
                                                                      12. Mellerio C et al: 3T MRI improves the detection of transmantle sign in type 2 focal cortical dysplasia. Epilepsia. 55(1):117-22, 2014
                                                                      13. Mellerio C et al: The power button sign: a newly described central sulcal pattern on surface rendering MR images of type 2 focal cortical dysplasia. Radiology. 140773, 2014
                                                                      14. Kadom N et al: Utility of magnetization transfer T1 imaging in children with seizures. AJNR Am J Neuroradiol. 34(4):895-8, 2013
                                                                      15. Colombo N et al: Focal cortical dysplasia type IIa and IIb: MRI aspects in 118 cases proven by histopathology. Neuroradiology. 54(10):1065-77, 2012
                                                                      16. Mellerio C et al: Optimizing MR imaging detection of type 2 focal cortical dysplasia: best criteria for clinical practice. AJNR Am J Neuroradiol. 33(10):1932-8, 2012
                                                                      17. Bernasconi A et al: Advances in MRI for 'cryptogenic' epilepsies. Nat Rev Neurol. 7(2):99-108, 2011
                                                                      18. Blumcke I et al: The clinicopathologic spectrum of focal cortical dysplasias: a consensus classification proposed by an ad hoc Task Force of the ILAE Diagnostic Methods Commission. Epilepsia. 52(1):158-74, 2011
                                                                      19. Kim YH et al: Neuroimaging in identifying focal cortical dysplasia and prognostic factors in pediatric and adolescent epilepsy surgery. Epilepsia. 52(4):722-7, 2011
                                                                      20. Raybaud C et al: Imaging surgical epilepsy in children. Childs Nerv Syst. 22(8):786-809, 2006