Hemimegalencephaly
Hemimegalencephaly is a rare malformation involving one side of the brain. It may occur alone or in association with other syndromes such as syndrome/resources/1" target="_blank">Proteus syndrome, tuberous sclerosis, syndrome/resources/1" target="_blank">linear sebaceous nevus syndrome, neurofibromatosis, syndrome/Resources/1" target="_blank">Sturge-Weber syndrome, or Klippel-Trenaunay syndrome. Children with this disorder may have a large, asymmetrical head accompanied by seizures, partial paralysis, and impaired cognitive development. Because the seizures associated with hemimegalencephaly are difficult to treat with anticonvulsant medications, a surgery called hemispherectomy is often the most successful treatment. The cause of hemimegalencephaly is not fully understood, but involves a disturbance of cells early in development and likely involves genes involved in patterning and symmetry.