Disorders of cortical development and epilepsy.
There has been an impressive increase in our ability to identify and categorize patients with cortical development lesions over the past decade. The clinical features associated with disorders of cortical development (DCD) have been described, and epilepsy has been shown to be a frequent symptom. In this review, we categorize DCD based on their structure and discuss their underlying causes and clinical features. Just as the cause of each type of disorder is thought to be unique, each disorder also has distinct types of seizures, treatment strategies, and electroencephalographic features. Studies in human tissue and animal models of DCD have begun to shed light on why DCD are associated with epilepsy. Aberrant synaptic connections within the dysplastic tissue and between the dysplastic tissue and more normal-appearing adjacent tissue form an abnormal, hyperexcitable network that increases seizure susceptibility. In the future, strategies for blocking formation of the aberrant networks may prevent the development of epilepsy.
Porter
Brenda E
BE
Division of Child Neurology, Children's Hospital of Philadelphia, Wood Bldg 6th Floor, Philadelphia, PA 19104, USA. porterb@email.chop.edu
Brooks-Kayal
Amy
A
Golden
Jeff A
JA
D000818:Animals; D001927:Brain Diseases / Q000150:complications; D002540:Cerebral Cortex / Q000002:abnormalities*; D002828:Choristoma / Q000150:complications; D000013:Congenital Abnormalities / Q000628:therapy; D004827:Epilepsy / Q000209:etiology*; D006801:Humans; D010487:Periaqueductal Gray