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Pediatrics
Diagnosis
Brain
Pathology-Based Diagnoses
Congenital Malformations
Malformations of Cortical Development
Polymicrogyria
Polymicrogyria
Luke L. Linscott, MD
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KEY FACTS

  • Terminology

    • Imaging

      • Top Differential Diagnoses

        • Pathology

          • Clinical Issues

            • Diagnostic Checklist

              TERMINOLOGY

              • Abbreviations

                • Polymicrogyria (PMG)

              • Definitions

                • Traditionally considered malformation of late neuronal migration & cortical organization resulting in abnormal distribution of neurons; disputed by some literature
                  • Regardless, macroscopic result is nodular cortex with disorganized small undulating gyri & shallow sulci
                    • May give false impression of several large, thick gyri

              IMAGING

              • General Features

                • CT Findings

                  • MR Findings

                    • Angiographic Findings

                      • Nuclear Medicine Findings

                        • Other Modality Findings

                          • Imaging Recommendations

                            DIFFERENTIAL DIAGNOSIS

                              PATHOLOGY

                              • General Features

                                • Gross Pathologic & Surgical Features

                                  • Microscopic Features

                                    CLINICAL ISSUES

                                    • Presentation

                                      • Demographics

                                        • Natural History & Prognosis

                                          • Treatment

                                            DIAGNOSTIC CHECKLIST

                                            • Image Interpretation Pearls

                                              Selected References

                                              1. Jalloh I et al: The role of surgery in refractory epilepsy secondary to polymicrogyria in the pediatric population. Epilepsia. 59(10):1982-96, 2018
                                              2. Diamandis P et al: The pathology of incipient polymicrogyria. Brain Dev. 39(1):23-39, 2017
                                              3. Desikan RS et al: Malformations of cortical development. Ann Neurol. 80(6):797-810, 2016
                                              4. Jansen AC et al: The histopathology of polymicrogyria: a series of 71 brain autopsy studies. Dev Med Child Neurol. 58(1):39-48, 2016
                                              5. De Ciantis A et al: Ultra-high-field MR imaging in polymicrogyria and epilepsy. AJNR Am J Neuroradiol. 36(2):309-16, 2015
                                              6. Squier W et al: Polymicrogyria: pathology, fetal origins and mechanisms. Acta Neuropathol Commun. 2:80, 2014
                                              7. Stutterd CA et al: Polymicrogyria: a common and heterogeneous malformation of cortical development. Am J Med Genet C Semin Med Genet. 166C(2):227-39, 2014
                                              8. Barkovich AJ et al: A developmental and genetic classification for malformations of cortical development: update 2012. Brain. 135(Pt 5):1348-69, 2012
                                              9. Mavili E et al: Polymicrogyria: correlation of magnetic resonance imaging and clinical findings. Childs Nerv Syst. 28(6):905-9, 2012
                                              10. Judkins AR et al: Polymicrogyria includes fusion of the molecular layer and decreased neuronal populations but normal cortical laminar organization. J Neuropathol Exp Neurol. 70(6):438-43, 2011
                                              11. Barkovich AJ: Current concepts of polymicrogyria. Neuroradiology. 52(6):479-87, 2010
                                              12. Leventer RJ et al: Clinical and imaging heterogeneity of polymicrogyria: a study of 328 patients. Brain. 133(Pt 5):1415-27, 2010
                                              13. Brandão-Almeida IL et al: Congenital bilateral perisylvian syndrome: familial occurrence, clinical and psycholinguistic aspects correlated with MRI. Neuropediatrics. 39(3):139-45, 2008
                                              14. Righini A et al: Early prenatal MR imaging diagnosis of polymicrogyria. AJNR Am J Neuroradiol. 25(2):343-6, 2004
                                              15. Takanashi J et al: The changing MR imaging appearance of polymicrogyria: a consequence of myelination. AJNR Am J Neuroradiol. 24(5):788-93, 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

                                                            • Polymicrogyria (PMG)

                                                          • Definitions

                                                            • Traditionally considered malformation of late neuronal migration & cortical organization resulting in abnormal distribution of neurons; disputed by some literature
                                                              • Regardless, macroscopic result is nodular cortex with disorganized small undulating gyri & shallow sulci
                                                                • May give false impression of several large, thick gyri

                                                          IMAGING

                                                          • General Features

                                                            • CT Findings

                                                              • MR Findings

                                                                • Angiographic Findings

                                                                  • Nuclear Medicine Findings

                                                                    • Other Modality Findings

                                                                      • Imaging Recommendations

                                                                        DIFFERENTIAL DIAGNOSIS

                                                                          PATHOLOGY

                                                                          • General Features

                                                                            • Gross Pathologic & Surgical Features

                                                                              • Microscopic Features

                                                                                CLINICAL ISSUES

                                                                                • Presentation

                                                                                  • Demographics

                                                                                    • Natural History & Prognosis

                                                                                      • Treatment

                                                                                        DIAGNOSTIC CHECKLIST

                                                                                        • Image Interpretation Pearls

                                                                                          Selected References

                                                                                          1. Jalloh I et al: The role of surgery in refractory epilepsy secondary to polymicrogyria in the pediatric population. Epilepsia. 59(10):1982-96, 2018
                                                                                          2. Diamandis P et al: The pathology of incipient polymicrogyria. Brain Dev. 39(1):23-39, 2017
                                                                                          3. Desikan RS et al: Malformations of cortical development. Ann Neurol. 80(6):797-810, 2016
                                                                                          4. Jansen AC et al: The histopathology of polymicrogyria: a series of 71 brain autopsy studies. Dev Med Child Neurol. 58(1):39-48, 2016
                                                                                          5. De Ciantis A et al: Ultra-high-field MR imaging in polymicrogyria and epilepsy. AJNR Am J Neuroradiol. 36(2):309-16, 2015
                                                                                          6. Squier W et al: Polymicrogyria: pathology, fetal origins and mechanisms. Acta Neuropathol Commun. 2:80, 2014
                                                                                          7. Stutterd CA et al: Polymicrogyria: a common and heterogeneous malformation of cortical development. Am J Med Genet C Semin Med Genet. 166C(2):227-39, 2014
                                                                                          8. Barkovich AJ et al: A developmental and genetic classification for malformations of cortical development: update 2012. Brain. 135(Pt 5):1348-69, 2012
                                                                                          9. Mavili E et al: Polymicrogyria: correlation of magnetic resonance imaging and clinical findings. Childs Nerv Syst. 28(6):905-9, 2012
                                                                                          10. Judkins AR et al: Polymicrogyria includes fusion of the molecular layer and decreased neuronal populations but normal cortical laminar organization. J Neuropathol Exp Neurol. 70(6):438-43, 2011
                                                                                          11. Barkovich AJ: Current concepts of polymicrogyria. Neuroradiology. 52(6):479-87, 2010
                                                                                          12. Leventer RJ et al: Clinical and imaging heterogeneity of polymicrogyria: a study of 328 patients. Brain. 133(Pt 5):1415-27, 2010
                                                                                          13. Brandão-Almeida IL et al: Congenital bilateral perisylvian syndrome: familial occurrence, clinical and psycholinguistic aspects correlated with MRI. Neuropediatrics. 39(3):139-45, 2008
                                                                                          14. Righini A et al: Early prenatal MR imaging diagnosis of polymicrogyria. AJNR Am J Neuroradiol. 25(2):343-6, 2004
                                                                                          15. Takanashi J et al: The changing MR imaging appearance of polymicrogyria: a consequence of myelination. AJNR Am J Neuroradiol. 24(5):788-93, 2003