Malformations of Cortical Development

Malformations of cortical development classification by Barkovich 2012

Group I: abnormal cell proliferation or apoptosis
  • Disorders of neuronal and/or glial proliferation or apoptosis.
  • Subgrouped by abnormalities of brain size
    • Group I.A (small)- microcephaly
      • with normal to simplified cortical pattern
      • with lissencephaly
      • with extensive polymicrogyria
    • Group I.B (large)
      • megalencephaly/macrocephaly
    • Group I.C (those with cortical dysgenesis with abnormal cells)
      • Non-neoplastic
        • Cortical hamartomas of tuberous sclerosis
        • cortical dysplasia with balloon cells
        • Hemimegalencephaly
        • type II focal cortical dysplasia
      • Neoplastic
        • Gangliogliomas
        • Gangliocytomas
        • Dysembryoplastic neuroepithelial tumours (DNET)
Group II: abnormal neuronal migration
  • Group II.C
Group III: abnormal post migrational development
  • Group III.A
    • Polymicrogyria and schizencephaly
    • bilateral polymicrogyria syndromes
    • Schizencephaly
    • polymicrogyria or schizencephaly as part of multiple congenital anomaly/intellectual disability syndromes
  • Group III.B
    • cortical dysgenesis secondary to inborn errors of metabolism
    • mitochondrial and pyruvate metabolic disorders
    • peroxisomal disorders
  • Group III.D
    • Post-migrational microcephaly (also in group I)

Reference