Transmantle cleft in brain parenchyma extending from cortical surface to ventricle (i.e., pia to ependyma)
Lined by dysplastic gray matter (GM)
IMAGING
General Features
CT Findings
MR Findings
Ultrasonographic 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
Park KB et al: The spectrum of brain malformations and disruptions in twins. Am J Med Genet A. 185(9):2690-718, 2021
Barkovich AJ et al: Pediatric Neuroimaging. 6th ed. Wolters Kluwer, 2019
Benson JC et al: Septo-optic dysplasia: assessment of associated findings with special attention to the olfactory sulci and tracts. Clin Neuroradiol. 29(3):505-13, 2019
Braga VL et al: Schizencephaly: a review of 734 patients. Pediatr Neurol. 87:23-9, 2018
Khalid R et al: COL4A1 and fetal vascular origins of schizencephaly. Neurology. 90(5):232-4, 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
Howe DT et al: Schizencephaly prevalence, prenatal diagnosis and clues to etiology: a register-based study. Ultrasound Obstet Gynecol. 39(1):75-82, 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
Merello E et al: No major role for the EMX2 gene in schizencephaly. Am J Med Genet A. 146A(9):1142-50, 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
Related Anatomy
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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)
IMAGING
General Features
CT Findings
MR Findings
Ultrasonographic 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
Park KB et al: The spectrum of brain malformations and disruptions in twins. Am J Med Genet A. 185(9):2690-718, 2021
Barkovich AJ et al: Pediatric Neuroimaging. 6th ed. Wolters Kluwer, 2019
Benson JC et al: Septo-optic dysplasia: assessment of associated findings with special attention to the olfactory sulci and tracts. Clin Neuroradiol. 29(3):505-13, 2019
Braga VL et al: Schizencephaly: a review of 734 patients. Pediatr Neurol. 87:23-9, 2018
Khalid R et al: COL4A1 and fetal vascular origins of schizencephaly. Neurology. 90(5):232-4, 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
Howe DT et al: Schizencephaly prevalence, prenatal diagnosis and clues to etiology: a register-based study. Ultrasound Obstet Gynecol. 39(1):75-82, 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
Merello E et al: No major role for the EMX2 gene in schizencephaly. Am J Med Genet A. 146A(9):1142-50, 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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