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KEY FACTS
Imaging
Top Differential Diagnoses
Pathology
Clinical Issues
TERMINOLOGY
Synonyms
- Agenetic porencephaly
Definitions
- Transmantle cleft in brain parenchyma extending from cortical surface to ventricle (i.e., pia to ependyma)
- Lined by dysplastic gray matter (GM)
- Types I (closed-lip) & II (open-lip) defects
- Most consider schizencephaly to be severe form of polymicrogyria (PMG) given their common association with one another
- Transmantle cleft in brain parenchyma extending from cortical surface to ventricle (i.e., pia to ependyma)
IMAGING
General Features
CT Findings
MR Findings
Ultrasonographic Findings
Nuclear Medicine Findings
Imaging Recommendations
DIFFERENTIAL DIAGNOSIS
PATHOLOGY
General Features
Gross Pathologic & Surgical Features
Microscopic Features
CLINICAL ISSUES
Presentation
Demographics
Natural History & Prognosis
Treatment
DIAGNOSTIC CHECKLIST
Consider
Image Interpretation Pearls
Selected References
- Okunlola AI et al: Adult-onset seizure disorder secondary to schizencephaly. Asian J Neurosurg. 15(1):159-61, 2020
- Reghunath A et al: A journey through formation and malformations of the neo-cortex. Childs Nerv Syst. 36(1):27-38, 2020
- Vitale G et al: Cortical malformations and COL4A1 mutation: three new cases. Eur J Paediatr Neurol. 23(3):410-7, 2019
- Braga VL et al: Schizencephaly: a review of 734 patients. Pediatr Neurol. 87:23-9, 2018
- Griffiths PD: Schizencephaly revisited. Neuroradiology. 60(9):945-60, 2018
- Hung PC et al: Schizencephaly in children: a single medical center retrospective study. Pediatr Neonatol. 59(6):573-80, 2018
- Halabuda A et al: Schizencephaly-diagnostics and clinical dilemmas. Childs Nerv Syst. 31(4):551-6, 2015
- Kutuk MS et al: Prenatal diagnosis and postnatal outcome of schizencephaly. J Child Neurol. 30(10):1388-94, 2014
- Nabavizadeh SA et al: Correlation of prenatal and postnatal MRI findings in schizencephaly. AJNR Am JNeuroradiol. 35(7):1418-24, 2014
- Stopa J et al: Diagnostic imaging and problems of schizencephaly. Pol J Radiol. 79:444-9, 2014
- 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
- Cui Z et al: Resection or multi-lobe disconnection for intractable epilepsy with open-lip schizencephaly. J Clin Neurosci. 20(12):1780-2, 2013
- 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
- Yoneda Y et al: Phenotypic spectrum of COL4A1 mutations: porencephaly to schizencephaly. Ann Neurol. 73(1):48-57, 2013
- Barkovich AJ et al: Pediatric Neuroimaging. 5th ed. Lippincott Williams & Wilkins, 2012
- Howe DT et al: Schizencephaly prevalence, prenatal diagnosis and clues to etiology: a register-based study. Ultrasound Obstet Gynecol. 39(1):75-82, 2012
- Rios LT et al: Prenatal and postnatal schizencephaly findings by 2D and 3D ultrasound: pictorial essay. J Clin Imaging Sci. 2:30, 2012
- Mellado C et al: Candidate gene sequencing of LHX2, HESX1, and SOX2 in a large schizencephaly cohort. Am J Med Genet A. 152A(11):2736-42, 2010
- Heuer GG et al: Anatomic hemispherectomy for intractable epilepsy in a patient with unilateral schizencephaly. J Neurosurg Pediatr. 2(2):146-9, 2008
- 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
- Vandermeeren Y et al: Functional relevance of abnormal fMRI activation pattern after unilateral schizencephaly. Neuroreport. 13(14):1821-4, 2002
- Denis D et al: Schizencephaly: clinical and imaging features in 30 infantile cases. Brain Dev. 22(8):475-83, 2000
- 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
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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
- Transmantle cleft in brain parenchyma extending from cortical surface to ventricle (i.e., pia to ependyma)
- Lined by dysplastic gray matter (GM)
- Types I (closed-lip) & II (open-lip) defects
- Most consider schizencephaly to be severe form of polymicrogyria (PMG) given their common association with one another
- Transmantle cleft in brain parenchyma extending from cortical surface to ventricle (i.e., pia to ependyma)
IMAGING
General Features
CT Findings
MR Findings
Ultrasonographic Findings
Nuclear Medicine Findings
Imaging Recommendations
DIFFERENTIAL DIAGNOSIS
PATHOLOGY
General Features
Gross Pathologic & Surgical Features
Microscopic Features
CLINICAL ISSUES
Presentation
Demographics
Natural History & Prognosis
Treatment
DIAGNOSTIC CHECKLIST
Consider
Image Interpretation Pearls
Selected References
- Okunlola AI et al: Adult-onset seizure disorder secondary to schizencephaly. Asian J Neurosurg. 15(1):159-61, 2020
- Reghunath A et al: A journey through formation and malformations of the neo-cortex. Childs Nerv Syst. 36(1):27-38, 2020
- Vitale G et al: Cortical malformations and COL4A1 mutation: three new cases. Eur J Paediatr Neurol. 23(3):410-7, 2019
- Braga VL et al: Schizencephaly: a review of 734 patients. Pediatr Neurol. 87:23-9, 2018
- Griffiths PD: Schizencephaly revisited. Neuroradiology. 60(9):945-60, 2018
- Hung PC et al: Schizencephaly in children: a single medical center retrospective study. Pediatr Neonatol. 59(6):573-80, 2018
- Halabuda A et al: Schizencephaly-diagnostics and clinical dilemmas. Childs Nerv Syst. 31(4):551-6, 2015
- Kutuk MS et al: Prenatal diagnosis and postnatal outcome of schizencephaly. J Child Neurol. 30(10):1388-94, 2014
- Nabavizadeh SA et al: Correlation of prenatal and postnatal MRI findings in schizencephaly. AJNR Am JNeuroradiol. 35(7):1418-24, 2014
- Stopa J et al: Diagnostic imaging and problems of schizencephaly. Pol J Radiol. 79:444-9, 2014
- 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
- Cui Z et al: Resection or multi-lobe disconnection for intractable epilepsy with open-lip schizencephaly. J Clin Neurosci. 20(12):1780-2, 2013
- 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
- Yoneda Y et al: Phenotypic spectrum of COL4A1 mutations: porencephaly to schizencephaly. Ann Neurol. 73(1):48-57, 2013
- Barkovich AJ et al: Pediatric Neuroimaging. 5th ed. Lippincott Williams & Wilkins, 2012
- Howe DT et al: Schizencephaly prevalence, prenatal diagnosis and clues to etiology: a register-based study. Ultrasound Obstet Gynecol. 39(1):75-82, 2012
- Rios LT et al: Prenatal and postnatal schizencephaly findings by 2D and 3D ultrasound: pictorial essay. J Clin Imaging Sci. 2:30, 2012
- Mellado C et al: Candidate gene sequencing of LHX2, HESX1, and SOX2 in a large schizencephaly cohort. Am J Med Genet A. 152A(11):2736-42, 2010
- Heuer GG et al: Anatomic hemispherectomy for intractable epilepsy in a patient with unilateral schizencephaly. J Neurosurg Pediatr. 2(2):146-9, 2008
- 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
- Vandermeeren Y et al: Functional relevance of abnormal fMRI activation pattern after unilateral schizencephaly. Neuroreport. 13(14):1821-4, 2002
- Denis D et al: Schizencephaly: clinical and imaging features in 30 infantile cases. Brain Dev. 22(8):475-83, 2000
- 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
