Rethinking Seizure Care Blog

Focal Cortical Dysplasia, a Congenital Cause of Epilepsy

Posted by Lucy Sullivan, R. EEG T., CLTM on Mar 7, 2016

Focal_Cortical_Dysplasia_BLOGFocal cortical dysplasia (FCD) is a variety of congenital (present at birth) abnormalities associated with focal epilepsy. FCD affects the development of the cerebral cortex (top layer of the brain) in both children and adults. When in utero neurons migrate from the center of the brain in the germinal zones to the outer areas and the neurons do not migrate in the proper formation, areas of focal cortical dysplasia are formed. These areas are highly epileptogenic – meaning that they are likely to produce seizures. Focal cortical dysplasia is a common cause of drug-resistant epilepsy and about 76% of patients with it will not respond to anti-epileptic drugs (AEDs).

How prevalent is FCD?

Focal cortical dysplasia is a congenital abnormality that usually occurs sporadically without any obvious cause. It has not been linked to any medications taken by the mother during pregnancy. In some cases, FCD may be part of a larger genetic abnormality such as Tuberous Sclerosis. FCD is estimated to be present in 1 in 2,500 newborn infants.

How is FCD Detected?

The most common symptom of FCD is focal seizures and focal seizures that secondarily generalize to cause a generalized tonic-clonic seizure. Although focal cortical dysplasia is a congenital malformation and present at birth, the patient may not develop epilepsy until childhood, adolescence, or adulthood. During the investigative work-up to search for a cause of the seizures, brain MRI often reveals the focal cortical dysplasia. The FCD can be subtle on MRI and very difficult to see on CT scans. Prior to high resolution MRI, focal cortical dysplasia was discovered on autopsy and thought to be very rare. With the continuous improvement of MRI and other neuroimaging techniques, recognition and localization of the FCD can be more precise.

What are the Treatments?

The treatment is based on controlling the seizures. Focal seizures with identified focal cortical dysplasia can be very difficult to control with antiepileptic medications. Surgical removal of the FCD and surrounding epileptogenic (seizure-causing) zone may lead to a seizure-free life for the patient. Scalp EEG often shows prolonged or continuous runs of spikes usually localized to the scalp over the dysplastic lesion. Invasive EEG, which records from electrodes placed directly in or on the cortex by a neurosurgeon, is necessary to localize the epileptogenic zone prior to the surgical removal.

Where can I learn more?

An article, “Focal dysplasia of the cerebral cortex in epilepsy” was published in 1971 after lobectomy brain specimens of ten patients with epilepsy were noted as having “unusual microscopic abnormality”. Since then, developments in the recognition, understanding, and treatment of epilepsy caused by focal cortical dysplasia has greatly expanded. Today, the Cortical Foundation is an excellent resource dedicated to providing services to educate, advocate, support, and improve awareness of cortical malformations.   

 

READER POLL: Do you know how many types of focal cortical dysplasia there are?

Take our Quick Poll

 

Author: Lucy Sullivan, R. EEG T., CLTM

Lucy_SullivanLucy has more than 42 years of EEG experience, having served as Program Director of an accredited EEG school, Supervisor of an EEG Department at Children’s Hospital in Pittsburgh, and Director of Publications for ASET which included Managing Editor of The Neurodiagnostic Journal. She has expertise in EEG and long-term monitoring for epilepsy including invasive EEG and accomplishments have included receiving the Maureen Berkeley Award for the outstanding technologist article in The Neurodiagnostic Journal and the Theda Sannit Award for outstanding neurodiagnostic educator. Lucy was trained in EEG at the University of Wisconsin – Madison.  Lucy joined RSC's team in February 2016 and we are delighted to have her join RSC efforts to Redefine Seizure Care.

Sources: 

Taylor DC, Falconer MA, Burton CJ, Corsellis JA. Focal dysplasia of the cerebral cortex in epilepsy. J Neurol Neurosurg Psychiatry 1971:34:369–387

http://www.ncbi.nlm.nih.gov/pmc/articles/PMC493805/pdf/jnnpsyc00214-0001.pdf

http://hemifoundation.homestead.com/hemimegalencephaly.html

http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3403799/

http://cortfoundation.org/cms/

Topics: Causes of Seizures