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Heterotopic Gray Matter
Luke L. Linscott, MD
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KEY FACTS

  • Terminology

    • Imaging

      • Top Differential Diagnoses

        • Pathology

          • Clinical Issues

            • Diagnostic Checklist

              TERMINOLOGY

              • Synonyms

                • Gray matter (GM) heterotopia (HTP)
                • Double cortex ~ band HTP
              • Definitions

                • Abnormally located GM due to foreshortened or prolonged migration of neuronal groups
                  • Occurs anywhere from periventricular germinal zone (GZ) to pia

              IMAGING

              • General Features

                • CT Findings

                  • MR Findings

                    • Ultrasonographic 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. Cossu M et al: RF-ablation in periventricular heterotopia-related epilepsy. Epilepsy Res. 142:121-5, 2018
                                              2. Yang E et al: A practical approach to supratentorial brain malformations: what radiologists should know. Radiol Clin North Am. 55(4):609-27, 2017
                                              3. Hung PC et al: Clinical and neuroimaging findings in children with gray matter heterotopias: a single institution experience of 36 patients. Eur J Paediatr Neurol. 20(5):732-7, 2016
                                              4. Nagaraj UD et al: Evaluation of subependymal gray matter heterotopias on fetal MRI. AJNR Am J Neuroradiol. 37(4):720-5, 2016
                                              5. Watrin F et al: Causes and consequences of gray matter heterotopia. CNS Neurosci Ther. 21(2):112-22, 2015
                                              6. Lega B et al: Hemispheric malformations of cortical development: surgical indications and approach. Childs Nerv Syst. 30(11):1831-7, 2014
                                              7. Bahi-Buisson N et al: New insights into genotype-phenotype correlations for the doublecortin-related lissencephaly spectrum. Brain. 136(Pt 1):223-44, 2013
                                              8. Conti V et al: Periventricular heterotopia in 6q terminal deletion syndrome: role of the C6orf70 gene. Brain. 136(Pt 11):3378-94, 2013
                                              9. Barkovich AJ et al: A developmental and genetic classification for malformations of cortical development: update 2012. Brain. 135(Pt 5):1348-69, 2012
                                              10. Christodoulou JA et al: Abnormal structural and functional brain connectivity in gray matter heterotopia. Epilepsia. 53(6):1024-32, 2012
                                              11. Raybaud C et al: Development and dysgenesis of the cerebral cortex: malformations of cortical development. Neuroimaging Clin N Am. 21(3):483-543, vii, 2011
                                              12. Guerrini R et al: Abnormal development of the human cerebral cortex: genetics, functional consequences and treatment options. Trends Neurosci. 31(3):154-62, 2008
                                              13. Widjaja E et al: Evaluation of subcortical white matter and deep white matter tracts in malformations of cortical development. Epilepsia. 48(8):1460-9, 2007
                                              14. Eriksson SH et al: Exploring white matter tracts in band heterotopia using diffusion tractography. Ann Neurol. 52(3):327-34, 2002
                                              15. Barkovich AJ et al: Gray matter heterotopia. Neurology. 55(11):1603-8, 2000
                                              16. Barkovich AJ: Morphologic characteristics of subcortical heterotopia: MR imaging study. AJNR Am J Neuroradiol. 21(2):290-5, 2000
                                              17. Gressens P: Mechanisms and disturbances of neuronal migration. Pediatr Res. 48(6):725-30, 2000
                                              18. Hannan AJ et al: Characterization of nodular neuronal heterotopia in children. Brain. 122 ( Pt 2):219-38, 1999
                                              19. Morioka T et al: Functional imaging in periventricular nodular heterotopia with the use of FDG-PET and HMPAO-SPECT. Neurosurg Rev. 22(1):41-4, 1999
                                              20. Marsh L et al: Proton magnetic resonance spectroscopy of a gray matter heterotopia. Neurology. 47(6):1571-4, 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

                                                        • Diagnostic Checklist

                                                          TERMINOLOGY

                                                          • Synonyms

                                                            • Gray matter (GM) heterotopia (HTP)
                                                            • Double cortex ~ band HTP
                                                          • Definitions

                                                            • Abnormally located GM due to foreshortened or prolonged migration of neuronal groups
                                                              • Occurs anywhere from periventricular germinal zone (GZ) to pia

                                                          IMAGING

                                                          • General Features

                                                            • CT Findings

                                                              • MR Findings

                                                                • Ultrasonographic 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. Cossu M et al: RF-ablation in periventricular heterotopia-related epilepsy. Epilepsy Res. 142:121-5, 2018
                                                                                          2. Yang E et al: A practical approach to supratentorial brain malformations: what radiologists should know. Radiol Clin North Am. 55(4):609-27, 2017
                                                                                          3. Hung PC et al: Clinical and neuroimaging findings in children with gray matter heterotopias: a single institution experience of 36 patients. Eur J Paediatr Neurol. 20(5):732-7, 2016
                                                                                          4. Nagaraj UD et al: Evaluation of subependymal gray matter heterotopias on fetal MRI. AJNR Am J Neuroradiol. 37(4):720-5, 2016
                                                                                          5. Watrin F et al: Causes and consequences of gray matter heterotopia. CNS Neurosci Ther. 21(2):112-22, 2015
                                                                                          6. Lega B et al: Hemispheric malformations of cortical development: surgical indications and approach. Childs Nerv Syst. 30(11):1831-7, 2014
                                                                                          7. Bahi-Buisson N et al: New insights into genotype-phenotype correlations for the doublecortin-related lissencephaly spectrum. Brain. 136(Pt 1):223-44, 2013
                                                                                          8. Conti V et al: Periventricular heterotopia in 6q terminal deletion syndrome: role of the C6orf70 gene. Brain. 136(Pt 11):3378-94, 2013
                                                                                          9. Barkovich AJ et al: A developmental and genetic classification for malformations of cortical development: update 2012. Brain. 135(Pt 5):1348-69, 2012
                                                                                          10. Christodoulou JA et al: Abnormal structural and functional brain connectivity in gray matter heterotopia. Epilepsia. 53(6):1024-32, 2012
                                                                                          11. Raybaud C et al: Development and dysgenesis of the cerebral cortex: malformations of cortical development. Neuroimaging Clin N Am. 21(3):483-543, vii, 2011
                                                                                          12. Guerrini R et al: Abnormal development of the human cerebral cortex: genetics, functional consequences and treatment options. Trends Neurosci. 31(3):154-62, 2008
                                                                                          13. Widjaja E et al: Evaluation of subcortical white matter and deep white matter tracts in malformations of cortical development. Epilepsia. 48(8):1460-9, 2007
                                                                                          14. Eriksson SH et al: Exploring white matter tracts in band heterotopia using diffusion tractography. Ann Neurol. 52(3):327-34, 2002
                                                                                          15. Barkovich AJ et al: Gray matter heterotopia. Neurology. 55(11):1603-8, 2000
                                                                                          16. Barkovich AJ: Morphologic characteristics of subcortical heterotopia: MR imaging study. AJNR Am J Neuroradiol. 21(2):290-5, 2000
                                                                                          17. Gressens P: Mechanisms and disturbances of neuronal migration. Pediatr Res. 48(6):725-30, 2000
                                                                                          18. Hannan AJ et al: Characterization of nodular neuronal heterotopia in children. Brain. 122 ( Pt 2):219-38, 1999
                                                                                          19. Morioka T et al: Functional imaging in periventricular nodular heterotopia with the use of FDG-PET and HMPAO-SPECT. Neurosurg Rev. 22(1):41-4, 1999
                                                                                          20. Marsh L et al: Proton magnetic resonance spectroscopy of a gray matter heterotopia. Neurology. 47(6):1571-4, 1996