Hemimegalencephaly

  • Etiology: defective stem cells with abnormal proliferation, migration, organization
  • Imaging: unilateral generalized / focal enlargement of hemisphere + ventricle, dysmyelination, thick cortical ribbon, cortical dysplasia, pachygyria, polymicrogyria, heterotopia, ipsilateral advanced myelination
  • Clinical: associated with epidermal nevus syndrome, Proteus syndrome, Klippel-Trenauny-Weber syndrome, neurofibromatois-1, hypomelanosis of Ito

Cases of Hemimegalencephaly