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Children with Focal Cortical Dysplasia Should Be Assessed for Epilepsy and Potentially Surgery

Patients diagnosed with focal cortical dysplasias (FCD), malformations of cortical development, often develop epilepsy, which is likely to be medication-resistant, researchers reported at AES2020, the virtual annual meeting of the American Epilepsy Society.

And they may be good candidates for epilepsy surgery, the researchers said.

"Eighty-two percent of 137 patients with FCD in this cohort developed epilepsy," Nathan Cohen, MD, assistant professor of neurology and pediatrics at George Washington University School of Medicine, and attending epileptologist at Children's National Hospital in Washington, DC, told Neurology Today At the Meetings.

"Of those with FCD-related epilepsy, two-thirds are pharmacoresistant," Dr. Nathan said. "We also showed that children with FCD-related epilepsy can benefit from epilepsy surgery. About 35 percent of our cohort had resective surgery and 83 percent of them had Engel I outcome, that is, seizure-freedom or freedom from disabling seizures."

"Neurologists and families should be aware that children with FCD-related epilepsy should be referred for early evaluation by a pediatric epileptologist and consideration for surgical intervention when appropriate," Dr. Cohen said.

For the retrospective cohort study, the investigators identified 137 children who fit the study criteria, accessing their electronic medical records at Children's National Hospital. The average age of those patients included in the study was 11.5 years. Of the children who were diagnosed with FCD, Dr. Nathan said 82 percent developed epilepsy, and 66 percent of these cases proved to be pharmacoresistant.

Of the 112 children with epilepsy, 75 percent were seizure-free at the last visit. Thirty-six percent of that group were seizure-free without surgery but still on anti-seizure medication; 4 percent were seizure-free without surgery and off anti-seizure medication; 28 percent were seizure-free after surgery but still on anti-seizure medication; and 8 percent were seizure-free after surgery and off anti-seizure medication.​

Commenting on the study, Jennifer Hopp, MD, associate professor of neurology and director of the epilepsy division at the University of Maryland School of Medicine, said: “Unfortunately, there remain several challenges to connecting patients with epilepsy surgery evaluations and treatment. These include lack of access to tertiary care epilepsy centers and referrals to specialized epilepsy centers that perform pre-surgical evaluations or that offer surgery."

“In fact, a study in Neurology in 2010 showed that despite the publication of an AAN Practice Parameter in 2003 that recommended referral for epilepsy surgery evaluation after appropriate anti-seizure medication trials, there was a trend to earlier referral but this was not a statistically significant finding," Dr. Hopp said.

“There seem to be several reasons that patients are not referred or sent for epilepsy surgery, despite success rates sometimes above 80 percent," she said. “Some studies have demonstrated that physicians may underestimate the level of disability experienced by patients with regard to seizure frequency or severity. Inaccurate assumptions about the success of surgery, intellectual disability and psychiatric comorbidity as well as older age of the patient have all been factors identified that influence physician referral behaviors. Patient factors certainly also play a role and early and clear discussion of the risks and benefits of epilepsy surgery for that individual are paramount in the patient decision-making process."

“Surgical therapy for FCD can be very effective," Dr. Hopp continued. “FCD is classified in different types, she said, and outcomes after resection of FCD may vary by type, but with a thorough evaluation and testing, patients may do quite well after surgical treatment."

Drs. Cohen and Hopp had no relevant disclosures.

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AES Abstract 97: Cohen N, Havens K, Whitehead M, Gaillard W. A natural history study of epilepsy in pediatric focal cortical dysplasia.​​