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Polymicrogyria
A. James Barkovich, MD
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KEY FACTS

  • Terminology

    • Imaging

      • Top Differential Diagnoses

        • Clinical Issues

          • Diagnostic Checklist

            TERMINOLOGY

            • Abbreviations

              • Polymicrogyria (PMG)
            • Definitions

              • PMG: Heterogeneous disorder with "too many/too small" cortical gyri
                • Pathologically, clinically, radiologically, etiologically heterogeneous
              • Malformation due to abnormality in both early (impaired proliferation, migration of neuroblasts) and late (disordered postmigrational cortical maturation) neuronal migration and cortical organization
                • Neurons reach cortex, but distribute abnormally, forming multiple small undulating gyri
                • Result is cortex containing multiple small sulci with fusion of molecular layer (layer 1) of cortex
                  • 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

                              • Staging, Grading, & Classification

                                • Gross Pathologic & Surgical Features

                                  • Microscopic Features

                                    CLINICAL ISSUES

                                    • Presentation

                                      • Demographics

                                        • Natural History & Prognosis

                                          • Treatment

                                            DIAGNOSTIC CHECKLIST

                                            • Consider

                                              • Image Interpretation Pearls

                                                Selected References

                                                1. De Ciantis A et al: Ultra-high-field MR imaging in polymicrogyria and epilepsy. AJNR Am J Neuroradiol. 36(2):309-16, 2015
                                                2. Desai NA et al: GPR56-Related Polymicrogyria: Clinicoradiologic Profile of 4 Patients. J Child Neurol. ePub, 2015
                                                3. Smithers-Sheedy H et al: Neuroimaging findings in a series of children with cerebral palsy and congenital cytomegalovirus infection. Infect Disord Drug Targets. ePub, 2015
                                                4. Squier W et al: Polymicrogyria: pathology, fetal origins and mechanisms. Acta Neuropathol Commun. 2:80, 2014
                                                5. Barkovich AJ et al: A developmental and genetic classification for malformations of cortical development: update 2012. Brain. 135(Pt 5):1348-69, 2012
                                                6. 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
                                                7. Mori T et al: Neuroradiological and neurofunctional examinations for patients with 22q11.2 deletion. Neuropediatrics. 42(6):215-21, 2011
                                                8. Barkovich AJ: Current concepts of polymicrogyria. Neuroradiology. 52(6):479-87, 2010
                                                9. Leventer RJ et al: Clinical and imaging heterogeneity of polymicrogyria: a study of 328 patients. Brain. 133(Pt 5):1415-27, 2010
                                                10. Mosca AL et al: Polymicrogyria in a child with inv dup del(9p) and 22q11.2 microduplication. Am J Med Genet A. 149A(3):475-81, 2009
                                                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
                                                Related Anatomy
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                                                Related Differential Diagnoses
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                                                References
                                                Tables

                                                Tables

                                                KEY FACTS

                                                • Terminology

                                                  • Imaging

                                                    • Top Differential Diagnoses

                                                      • Clinical Issues

                                                        • Diagnostic Checklist

                                                          TERMINOLOGY

                                                          • Abbreviations

                                                            • Polymicrogyria (PMG)
                                                          • Definitions

                                                            • PMG: Heterogeneous disorder with "too many/too small" cortical gyri
                                                              • Pathologically, clinically, radiologically, etiologically heterogeneous
                                                            • Malformation due to abnormality in both early (impaired proliferation, migration of neuroblasts) and late (disordered postmigrational cortical maturation) neuronal migration and cortical organization
                                                              • Neurons reach cortex, but distribute abnormally, forming multiple small undulating gyri
                                                              • Result is cortex containing multiple small sulci with fusion of molecular layer (layer 1) of cortex
                                                                • 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

                                                                            • Staging, Grading, & Classification

                                                                              • Gross Pathologic & Surgical Features

                                                                                • Microscopic Features

                                                                                  CLINICAL ISSUES

                                                                                  • Presentation

                                                                                    • Demographics

                                                                                      • Natural History & Prognosis

                                                                                        • Treatment

                                                                                          DIAGNOSTIC CHECKLIST

                                                                                          • Consider

                                                                                            • Image Interpretation Pearls

                                                                                              Selected References

                                                                                              1. De Ciantis A et al: Ultra-high-field MR imaging in polymicrogyria and epilepsy. AJNR Am J Neuroradiol. 36(2):309-16, 2015
                                                                                              2. Desai NA et al: GPR56-Related Polymicrogyria: Clinicoradiologic Profile of 4 Patients. J Child Neurol. ePub, 2015
                                                                                              3. Smithers-Sheedy H et al: Neuroimaging findings in a series of children with cerebral palsy and congenital cytomegalovirus infection. Infect Disord Drug Targets. ePub, 2015
                                                                                              4. Squier W et al: Polymicrogyria: pathology, fetal origins and mechanisms. Acta Neuropathol Commun. 2:80, 2014
                                                                                              5. Barkovich AJ et al: A developmental and genetic classification for malformations of cortical development: update 2012. Brain. 135(Pt 5):1348-69, 2012
                                                                                              6. 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
                                                                                              7. Mori T et al: Neuroradiological and neurofunctional examinations for patients with 22q11.2 deletion. Neuropediatrics. 42(6):215-21, 2011
                                                                                              8. Barkovich AJ: Current concepts of polymicrogyria. Neuroradiology. 52(6):479-87, 2010
                                                                                              9. Leventer RJ et al: Clinical and imaging heterogeneity of polymicrogyria: a study of 328 patients. Brain. 133(Pt 5):1415-27, 2010
                                                                                              10. Mosca AL et al: Polymicrogyria in a child with inv dup del(9p) and 22q11.2 microduplication. Am J Med Genet A. 149A(3):475-81, 2009
                                                                                              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