link
Bookmarks
Schizencephaly
Karen Y. Oh, MD
To access 4,300 diagnoses written by the world's leading experts in radiology, please log in or subscribe.Log inSubscribe
0
8
2
4

KEY FACTS

  • Terminology

    • Imaging

      • Top Differential Diagnoses

        • Pathology

          • Clinical Issues

            • Diagnostic Checklist

              TERMINOLOGY

              • Definitions

                • Gray matter-lined cleft extending from brain surface to ventricle
                  • Closed lip (type 1)
                    • Gray matter lips, which are in contact with each other
                  • Open lip (type 2)
                    • Separated gray matter lips with intervening cleft of cerebral spinal fluid (CSF)
                    • CSF cleft extends to underlying ventricle

              IMAGING

              • General Features

                • Ultrasonographic Findings

                  • MR Findings

                    DIFFERENTIAL DIAGNOSIS

                      PATHOLOGY

                      • General Features

                        • Gross Pathologic & Surgical Features

                          CLINICAL ISSUES

                          • Presentation

                            • Natural History & Prognosis

                              • Treatment

                                DIAGNOSTIC CHECKLIST

                                • Image Interpretation Pearls

                                  Selected References

                                  1. Halabuda A et al: Schizencephaly-diagnostics and clinical dilemmas. Childs Nerv Syst. 31(4):551-6, 2015
                                  2. Kutuk MS et al: Prenatal diagnosis and postnatal outcome of schizencephaly. J Child Neurol. 30(10):1388-94, 2014
                                  3. Nabavizadeh SA et al: Correlation of prenatal and postnatal MRI findings in schizencephaly. AJNR Am J Neuroradiol. 35(7):1418-24, 2014
                                  4. 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
                                  5. Abdel Razek AA et al: Disorders of cortical formation: MR imaging features. AJNR Am J Neuroradiol. 30(1):4-11, 2009
                                  6. Gedikbasi A et al: Prenatal diagnosis of schizencephaly with 2D-3D sonography and MRI. J Clin Ultrasound. 37(8):467-70, 2009
                                  7. Govaert P: Prenatal stroke. Semin Fetal Neonatal Med. 14(5):250-66, 2009
                                  8. Lee W et al: Prenatal diagnostic challenges and pitfalls for schizencephaly. J Ultrasound Med. 28(10):1379-84, 2009
                                  9. Oh KY et al: Fetal schizencephaly: pre- and postnatal imaging with a review of the clinical manifestations. Radiographics. 25(3):647-57, 2005
                                  10. Barkovich AJ et al: Malformations of cortical development. Neuroimaging Clin N Am. 14(3):401-23, 2004
                                  11. Barkovich AJ et al: Neuroimaging in disorders of cortical development. Neuroimaging Clin N Am. 14(2):231-54, viii, 2004
                                  12. Hayashi N et al: Morphological features and associated anomalies of schizencephaly in the clinical population: detailed analysis of MR images. Neuroradiology. 44(5):418-27, 2002
                                  13. Liang JS et al: Schizencephaly: correlation between clinical and neuroimaging features. Acta Paediatr Taiwan. 43(4):208-13, 2002
                                  14. Montenegro MA et al: Interrelationship of genetics and prenatal injury in the genesis of malformations of cortical development. Arch Neurol. 59(7):1147-53, 2002
                                  15. Denis D et al: Prenatal diagnosis of schizencephaly by fetal magnetic resonance imaging. Fetal Diagn Ther. 16(6):354-9, 2001
                                  16. Guerrini R et al: Epileptogenic brain malformations: clinical presentation, malformative patterns and indications for genetic testing. Seizure. 10(7):532-43; quiz 544-7, 2001
                                  17. Denis D et al: Schizencephaly: clinical and imaging features in 30 infantile cases. Brain Dev. 22(8):475-83, 2000
                                  18. Iannetti P et al: Cytomegalovirus infection and schizencephaly: case reports. Ann Neurol. 43(1):123-7, 1998
                                  19. Granata T et al: Familial schizencephaly associated with EMX2 mutation. Neurology. 48(5):1403-6, 1997
                                  20. Brunelli S et al: Germline mutations in the homeobox gene EMX2 in patients with severe schizencephaly. Nat Genet. 12(1):94-6, 1996
                                  21. Barkovich AJ et al: Schizencephaly: correlation of clinical findings with MR characteristics. AJNR Am J Neuroradiol. 13(1):85-94, 1992
                                  22. Aniskiewicz AS et al: Magnetic resonance imaging and neurobehavioral correlates in schizencephaly. Arch Neurol. 47(8):911-6, 1990
                                  23. Komarniski CA et al: Prenatal diagnosis of schizencephaly. J Ultrasound Med. 9(5):305-7, 1990
                                  24. Kuban KC et al: Septo-optic-dysplasia-schizencephaly. Radiographic and clinical features. Pediatr Radiol. 19(3):145-50, 1989
                                  25. Barkovich AJ et al: MR imaging of schizencephaly. AJR Am J Roentgenol. 150(6):1391-6, 1988
                                  Related Anatomy
                                  Loading...
                                  Related Differential Diagnoses
                                  Loading...
                                  References
                                  Tables

                                  Tables

                                  KEY FACTS

                                  • Terminology

                                    • Imaging

                                      • Top Differential Diagnoses

                                        • Pathology

                                          • Clinical Issues

                                            • Diagnostic Checklist

                                              TERMINOLOGY

                                              • Definitions

                                                • Gray matter-lined cleft extending from brain surface to ventricle
                                                  • Closed lip (type 1)
                                                    • Gray matter lips, which are in contact with each other
                                                  • Open lip (type 2)
                                                    • Separated gray matter lips with intervening cleft of cerebral spinal fluid (CSF)
                                                    • CSF cleft extends to underlying ventricle

                                              IMAGING

                                              • General Features

                                                • Ultrasonographic Findings

                                                  • MR Findings

                                                    DIFFERENTIAL DIAGNOSIS

                                                      PATHOLOGY

                                                      • General Features

                                                        • Gross Pathologic & Surgical Features

                                                          CLINICAL ISSUES

                                                          • Presentation

                                                            • Natural History & Prognosis

                                                              • Treatment

                                                                DIAGNOSTIC CHECKLIST

                                                                • Image Interpretation Pearls

                                                                  Selected References

                                                                  1. Halabuda A et al: Schizencephaly-diagnostics and clinical dilemmas. Childs Nerv Syst. 31(4):551-6, 2015
                                                                  2. Kutuk MS et al: Prenatal diagnosis and postnatal outcome of schizencephaly. J Child Neurol. 30(10):1388-94, 2014
                                                                  3. Nabavizadeh SA et al: Correlation of prenatal and postnatal MRI findings in schizencephaly. AJNR Am J Neuroradiol. 35(7):1418-24, 2014
                                                                  4. 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
                                                                  5. Abdel Razek AA et al: Disorders of cortical formation: MR imaging features. AJNR Am J Neuroradiol. 30(1):4-11, 2009
                                                                  6. Gedikbasi A et al: Prenatal diagnosis of schizencephaly with 2D-3D sonography and MRI. J Clin Ultrasound. 37(8):467-70, 2009
                                                                  7. Govaert P: Prenatal stroke. Semin Fetal Neonatal Med. 14(5):250-66, 2009
                                                                  8. Lee W et al: Prenatal diagnostic challenges and pitfalls for schizencephaly. J Ultrasound Med. 28(10):1379-84, 2009
                                                                  9. Oh KY et al: Fetal schizencephaly: pre- and postnatal imaging with a review of the clinical manifestations. Radiographics. 25(3):647-57, 2005
                                                                  10. Barkovich AJ et al: Malformations of cortical development. Neuroimaging Clin N Am. 14(3):401-23, 2004
                                                                  11. Barkovich AJ et al: Neuroimaging in disorders of cortical development. Neuroimaging Clin N Am. 14(2):231-54, viii, 2004
                                                                  12. Hayashi N et al: Morphological features and associated anomalies of schizencephaly in the clinical population: detailed analysis of MR images. Neuroradiology. 44(5):418-27, 2002
                                                                  13. Liang JS et al: Schizencephaly: correlation between clinical and neuroimaging features. Acta Paediatr Taiwan. 43(4):208-13, 2002
                                                                  14. Montenegro MA et al: Interrelationship of genetics and prenatal injury in the genesis of malformations of cortical development. Arch Neurol. 59(7):1147-53, 2002
                                                                  15. Denis D et al: Prenatal diagnosis of schizencephaly by fetal magnetic resonance imaging. Fetal Diagn Ther. 16(6):354-9, 2001
                                                                  16. Guerrini R et al: Epileptogenic brain malformations: clinical presentation, malformative patterns and indications for genetic testing. Seizure. 10(7):532-43; quiz 544-7, 2001
                                                                  17. Denis D et al: Schizencephaly: clinical and imaging features in 30 infantile cases. Brain Dev. 22(8):475-83, 2000
                                                                  18. Iannetti P et al: Cytomegalovirus infection and schizencephaly: case reports. Ann Neurol. 43(1):123-7, 1998
                                                                  19. Granata T et al: Familial schizencephaly associated with EMX2 mutation. Neurology. 48(5):1403-6, 1997
                                                                  20. Brunelli S et al: Germline mutations in the homeobox gene EMX2 in patients with severe schizencephaly. Nat Genet. 12(1):94-6, 1996
                                                                  21. Barkovich AJ et al: Schizencephaly: correlation of clinical findings with MR characteristics. AJNR Am J Neuroradiol. 13(1):85-94, 1992
                                                                  22. Aniskiewicz AS et al: Magnetic resonance imaging and neurobehavioral correlates in schizencephaly. Arch Neurol. 47(8):911-6, 1990
                                                                  23. Komarniski CA et al: Prenatal diagnosis of schizencephaly. J Ultrasound Med. 9(5):305-7, 1990
                                                                  24. Kuban KC et al: Septo-optic-dysplasia-schizencephaly. Radiographic and clinical features. Pediatr Radiol. 19(3):145-50, 1989
                                                                  25. Barkovich AJ et al: MR imaging of schizencephaly. AJR Am J Roentgenol. 150(6):1391-6, 1988