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Schizencephaly
Blaise V. Jones, MD
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KEY FACTS

  • Imaging

    • Top Differential Diagnoses

      • Pathology

        • Clinical Issues

          TERMINOLOGY

          • Synonyms

            • Agenetic porencephaly
          • Definitions

            • Clefts in brain parenchyma that extend from cortical surface to ventricle (pia to ependyma), lined by dysplastic gray matter (GM)

          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

                                        • Consider

                                          • Image Interpretation Pearls

                                            Selected References

                                            1. Halabuda A et al: Schizencephaly-diagnostics and clinical dilemmas. Childs Nerv Syst. 31(4):551-6, 2015
                                            2. Nabavizadeh SA et al: Correlation of prenatal and postnatal MRI findings in schizencephaly. AJNR Am J Neuroradiol. Epub ahead of print, 2014
                                            3. Choi HY et al: Long-term outcome of surgical treatment of patients with intractable epilepsy associated with schizencephaly. Acta Neurochir (Wien). 155(9):1717-24, 2013
                                            4. Cui Z et al: Resection or multi-lobe disconnection for intractable epilepsy with open-lip schizencephaly. J Clin Neurosci. 20(12):1780-2, 2013
                                            5. Dies KA et al: Schizencephaly: association with young maternal age, alcohol use, and lack of prenatal care. J Child Neurol. 28(2):198-203, 2013
                                            6. da Rocha FF et al: Borderline personality features possibly related to cingulate and orbitofrontal cortices dysfunction due to schizencephaly. Clin Neurol Neurosurg. 110(4):396-9, 2008
                                            7. Heuer GG et al: Anatomic hemispherectomy for intractable epilepsy in a patient with unilateral schizencephaly. J Neurosurg Pediatr. 2(2):146-9, 2008
                                            8. Merello E et al: No major role for the EMX2 gene in schizencephaly. Am J Med Genet A. 146A(9):1142-50, 2008
                                            9. Vinayan KP et al: A case of congenital bilateral perisylvian syndrome due to bilateral schizencephaly. Epileptic Disord. 9(2):190-3, 2007
                                            10. Witters I et al: Prenatal diagnosis of schizencephaly after inhalation of organic solvents. Ultrasound Obstet Gynecol. 29(3):356-7, 2007
                                            11. Huang WM et al: Schizencephaly in a dysgenetic fetal brain: prenatal sonographic, magnetic resonance imaging, and postmortem correlation. J Ultrasound Med. 25(4):551-4, 2006
                                            12. Curry CJ et al: Schizencephaly: heterogeneous etiologies in a population of 4 million California births. Am J Med Genet A. 137(2):181-9, 2005
                                            13. Cecchi C: Emx2: a gene responsible for cortical development, regionalization and area specification. Gene. 291(1-2):1-9, 2002
                                            14. Dale ST et al: Neonatal alloimmune thrombocytopenia: antenatal and postnatal imaging findings in the pediatric brain. AJNR Am J Neuroradiol. 23(9):1457-65, 2002
                                            15. Vandermeeren Y et al: Functional relevance of abnormal fMRI activation pattern after unilateral schizencephaly. Neuroreport. 13(14):1821-4, 2002
                                            16. Morioka T et al: Functional imaging in schizencephaly using [18F]fluoro-2-deoxy-D-glucose positron emission tomography (FDG-PET) and single photon emission computed tomography with technetium-99m-hexamethyl-propyleneamine oxime (HMPAO-SPECT). Neurosurg Rev. 22(2-3):99-101, 1999
                                            17. Takano T et al: Experimental schizencephaly induced by Kilham strain of mumps virus: pathogenesis of cleft formation. Neuroreport. 10(15):3149-54, 1999
                                            Related Anatomy
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                                            Related Differential Diagnoses
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                                            References
                                            Tables

                                            Tables

                                            KEY FACTS

                                            • Imaging

                                              • Top Differential Diagnoses

                                                • Pathology

                                                  • Clinical Issues

                                                    TERMINOLOGY

                                                    • Synonyms

                                                      • Agenetic porencephaly
                                                    • Definitions

                                                      • Clefts in brain parenchyma that extend from cortical surface to ventricle (pia to ependyma), lined by dysplastic gray matter (GM)

                                                    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

                                                                                  • Consider

                                                                                    • Image Interpretation Pearls

                                                                                      Selected References

                                                                                      1. Halabuda A et al: Schizencephaly-diagnostics and clinical dilemmas. Childs Nerv Syst. 31(4):551-6, 2015
                                                                                      2. Nabavizadeh SA et al: Correlation of prenatal and postnatal MRI findings in schizencephaly. AJNR Am J Neuroradiol. Epub ahead of print, 2014
                                                                                      3. Choi HY et al: Long-term outcome of surgical treatment of patients with intractable epilepsy associated with schizencephaly. Acta Neurochir (Wien). 155(9):1717-24, 2013
                                                                                      4. Cui Z et al: Resection or multi-lobe disconnection for intractable epilepsy with open-lip schizencephaly. J Clin Neurosci. 20(12):1780-2, 2013
                                                                                      5. Dies KA et al: Schizencephaly: association with young maternal age, alcohol use, and lack of prenatal care. J Child Neurol. 28(2):198-203, 2013
                                                                                      6. da Rocha FF et al: Borderline personality features possibly related to cingulate and orbitofrontal cortices dysfunction due to schizencephaly. Clin Neurol Neurosurg. 110(4):396-9, 2008
                                                                                      7. Heuer GG et al: Anatomic hemispherectomy for intractable epilepsy in a patient with unilateral schizencephaly. J Neurosurg Pediatr. 2(2):146-9, 2008
                                                                                      8. Merello E et al: No major role for the EMX2 gene in schizencephaly. Am J Med Genet A. 146A(9):1142-50, 2008
                                                                                      9. Vinayan KP et al: A case of congenital bilateral perisylvian syndrome due to bilateral schizencephaly. Epileptic Disord. 9(2):190-3, 2007
                                                                                      10. Witters I et al: Prenatal diagnosis of schizencephaly after inhalation of organic solvents. Ultrasound Obstet Gynecol. 29(3):356-7, 2007
                                                                                      11. Huang WM et al: Schizencephaly in a dysgenetic fetal brain: prenatal sonographic, magnetic resonance imaging, and postmortem correlation. J Ultrasound Med. 25(4):551-4, 2006
                                                                                      12. Curry CJ et al: Schizencephaly: heterogeneous etiologies in a population of 4 million California births. Am J Med Genet A. 137(2):181-9, 2005
                                                                                      13. Cecchi C: Emx2: a gene responsible for cortical development, regionalization and area specification. Gene. 291(1-2):1-9, 2002
                                                                                      14. Dale ST et al: Neonatal alloimmune thrombocytopenia: antenatal and postnatal imaging findings in the pediatric brain. AJNR Am J Neuroradiol. 23(9):1457-65, 2002
                                                                                      15. Vandermeeren Y et al: Functional relevance of abnormal fMRI activation pattern after unilateral schizencephaly. Neuroreport. 13(14):1821-4, 2002
                                                                                      16. Morioka T et al: Functional imaging in schizencephaly using [18F]fluoro-2-deoxy-D-glucose positron emission tomography (FDG-PET) and single photon emission computed tomography with technetium-99m-hexamethyl-propyleneamine oxime (HMPAO-SPECT). Neurosurg Rev. 22(2-3):99-101, 1999
                                                                                      17. Takano T et al: Experimental schizencephaly induced by Kilham strain of mumps virus: pathogenesis of cleft formation. Neuroreport. 10(15):3149-54, 1999