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Generalized polymicrogyria

Gross lateral view of cerebral hemisphere of a term neonate with generalized bilateral polymicrogyria. The normal pattern of gyration is not observed in any lobe. Gyri are small and excessive in number, and many of the sulci separating them are shallow because of gyral fusion. The lateral cerebral (Sylvian) fissure is formed, and the operculum is closed. Genetic testing was not performed in this case (parental decision) but might have shown a mutation causing tubulinopathy. (Reproduced from: Blumcke I, Sarnat HB, Coras R. Surgical Neuropathology of Focal Epilepsies: Textbook and Atlas. Montrouge, France: John Libbey Eurotext, 2015:44-6.)

Associated Disorders

  • Aicardi syndrome
  • Chiari I malformation
  • Cortical dysplasia
  • Foix-Chavany-Marie syndrome
  • Glioneuronal leptomeningeal heterotopia
  • Glutaric academia type II
  • Histidinemia
  • Leigh syndrome
  • Lissencephaly, types I and II
  • Maple syrup urine disease
  • Mitochondrial respiratory chain deficiency
  • Neonatal adrenoleukodystrophy
  • Nodular heterotopia
  • Pachygyria
  • Pelizaeus-Merzbacher disease
  • Porencephaly
  • Schizencephaly
  • Spinal muscular atrophy in BICD2 mutation
  • Thanatophoric dysplasia
  • Walker-Warburg syndrome
  • Zellweger syndrome