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Disorders of Cortical Development and Epilepsy
Brenda E. Porter, MD, PhD;
Amy Brooks-Kayal, MD;
Jeff A. Golden, MD
Arch Neurol. 2002;59:361-365.
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.
From the Pediatric Regional Epilepsy Program, Divisions of Child Neurology
(Drs Porter and Brooks-Kayal) and Neuropathology (Dr Golden), Children's Hospital
of Philadelphia and the University of Pennsylvania School of Medicine, Philadelphia.
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