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Schizencephaly revisited.

Paul D Griffiths1

  • 1Academic Unit of Radiology, University of Sheffield, Floor C, Glossop Road, Sheffield, England, S10 2JF, UK. p.griffiths@sheffield.ac.uk.

Neuroradiology
|July 21, 2018
PubMed
Summary
This summary is machine-generated.

This study reviews 32 cases of schizencephaly in children and fetuses, proposing a new classification system and suggesting an acquired destructive cause for this brain malformation.

Keywords:
FetusMR imagingPediatricSchizencephaly

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Area of Science:

  • Neuroscience
  • Developmental Biology
  • Pediatric Neurology

Background:

  • Schizencephaly is a rare congenital brain malformation characterized by a cleft in the cerebral hemisphere.
  • Understanding its diverse presentations and underlying causes is crucial for diagnosis and management.

Purpose of the Study:

  • To report the range of schizencephaly appearances in pediatric and fetal cases over a 10-year period.
  • To detail classification systems for different forms of schizencephaly.
  • To re-assess potential etiological and mechanistic causes.

Main Methods:

  • A retrospective review of 21 children and 11 fetuses diagnosed with schizencephaly between 2007 and 2016.
  • Analysis of schizencephaly type, location, and associated brain abnormalities, including the septum pellucidum and fornices.

Main Results:

  • Schizencephaly type 2 was the most common form in children (67%) and fetuses (45%).
  • Type 3 was more prevalent in fetuses (55%) than children (24%).
  • Associated brain abnormalities were frequent, present in 67% of children and 55% of fetuses.

Conclusions:

  • A novel classification system for schizencephaly is proposed, integrating literature definitions.
  • The study describes the appearances and associations of pediatric and fetal schizencephaly.
  • Evidence suggests an acquired destructive etiology for most cases, with a proposed mechanism for associated cortical abnormalities.