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Polymicrogyria
Luke L. Linscott, MD
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KEY FACTS

  • Terminology

    • Imaging

      • Top Differential Diagnoses

        • Pathology

          • Clinical Issues

            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. Arrigoni F et al: Characterizing white matter tract organization in polymicrogyria and lissencephaly: a multifiber diffusion mri modeling and tractography study. AJNR Am J Neuroradiol. 41(8):1495-502, 2020
                                            2. Jansen AC et al: The histopathology of polymicrogyria: a series of 71 brain autopsy studies. Dev Med Child Neurol. 58(1):39-48, 2016
                                            3. De Ciantis A et al: Ultra-high-field MR imaging in polymicrogyria and epilepsy. AJNR Am J Neuroradiol. 36(2):309-16, 2015
                                            4. Squier W et al: Polymicrogyria: pathology, fetal origins and mechanisms. Acta Neuropathol Commun. 2:80, 2014
                                            5. 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
                                            6. Barkovich AJ et al: A developmental and genetic classification for malformations of cortical development: update 2012. Brain. 135(Pt 5):1348-69, 2012
                                            7. Mavili E et al: Polymicrogyria: correlation of magnetic resonance imaging and clinical findings. Childs Nerv Syst. 28(6):905-9, 2012
                                            8. 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
                                            9. Barkovich AJ: Current concepts of polymicrogyria. Neuroradiology. 52(6):479-87, 2010
                                            10. Leventer RJ et al: Clinical and imaging heterogeneity of polymicrogyria: a study of 328 patients. Brain. 133(Pt 5):1415-27, 2010
                                            11. Brandão-Almeida IL et al: Congenital bilateral perisylvian syndrome: familial occurrence, clinical and psycholinguistic aspects correlated with MRI. Neuropediatrics. 39(3):139-45, 2008
                                            12. Righini A et al: Early prenatal MR imaging diagnosis of polymicrogyria. AJNR Am J Neuroradiol. 25(2):343-6, 2004
                                            13. 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

                                                      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. Arrigoni F et al: Characterizing white matter tract organization in polymicrogyria and lissencephaly: a multifiber diffusion mri modeling and tractography study. AJNR Am J Neuroradiol. 41(8):1495-502, 2020
                                                                                      2. Jansen AC et al: The histopathology of polymicrogyria: a series of 71 brain autopsy studies. Dev Med Child Neurol. 58(1):39-48, 2016
                                                                                      3. De Ciantis A et al: Ultra-high-field MR imaging in polymicrogyria and epilepsy. AJNR Am J Neuroradiol. 36(2):309-16, 2015
                                                                                      4. Squier W et al: Polymicrogyria: pathology, fetal origins and mechanisms. Acta Neuropathol Commun. 2:80, 2014
                                                                                      5. 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
                                                                                      6. Barkovich AJ et al: A developmental and genetic classification for malformations of cortical development: update 2012. Brain. 135(Pt 5):1348-69, 2012
                                                                                      7. Mavili E et al: Polymicrogyria: correlation of magnetic resonance imaging and clinical findings. Childs Nerv Syst. 28(6):905-9, 2012
                                                                                      8. 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
                                                                                      9. Barkovich AJ: Current concepts of polymicrogyria. Neuroradiology. 52(6):479-87, 2010
                                                                                      10. Leventer RJ et al: Clinical and imaging heterogeneity of polymicrogyria: a study of 328 patients. Brain. 133(Pt 5):1415-27, 2010
                                                                                      11. Brandão-Almeida IL et al: Congenital bilateral perisylvian syndrome: familial occurrence, clinical and psycholinguistic aspects correlated with MRI. Neuropediatrics. 39(3):139-45, 2008
                                                                                      12. Righini A et al: Early prenatal MR imaging diagnosis of polymicrogyria. AJNR Am J Neuroradiol. 25(2):343-6, 2004
                                                                                      13. Takanashi J et al: The changing MR imaging appearance of polymicrogyria: a consequence of myelination. AJNR Am J Neuroradiol. 24(5):788-93, 2003