Brain Advance Access originally published online on May 19, 2006
Brain 2006 129(7):1907-1916; doi:10.1093/brain/awl133
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Clinical characteristics in focal cortical dysplasia: a retrospective evaluation in a series of 120 patients
1 Department of Epilepsy Centre, University of Freiburg Kehl-Kork, Germany 2 Department of Neuropathology, University of Freiburg Kehl-Kork, Germany 3 Department of Neuropediatrics and Muscular Diseases, University of Freiburg Kehl-Kork, Germany 4 Department of Pediatric Neurology, University of Heidelberg Kehl-Kork, Germany 5 Epilepsy Centre Kork Kehl-Kork, Germany
Correspondence to: Susanne Fauser, Epilepsy Center, University of Freiburg, Breisacher Street 64, 79106 Freiburg, Germany E-mail: fauser{at}nz11.ukl.uni-freiburg.de
Focal cortical dysplasias (FCDs) are increasingly diagnosed as a cause of symptomatic focal epilepsy in paediatric and adult patients. However, little is known about the clinical characteristics of epilepsy in these patients. In order to elucidate the clinical characteristics of their epilepsy, 120 pharmacoresistant patients including children and adults with histologically proven FCD were studied retrospectively. Age at seizure onset was analysed in the total group and compared between subgroups with different localization and different histological subtypes of FCD. The role of febrile seizures with respect to dual pathology was investigated. Seizure semiology was analysed focusing on initial seizure type and change of seizure semiology during the course of disease. Finally, transient responsiveness to antiepileptic drug therapy was studied. In the majority of patients, epilepsy began in the first 5 years of life. However, onset of epilepsy could also occur in the second or third decade until the age of 60. Age at epilepsy onset was not significantly different between temporal, extratemporal and multilobar localization of FCD. Patients without cytoarchitectural abnormalities (mild malformations of cortical development, FCD 1a according to Palmini) had significantly later epilepsy onset (P= 0.001) compared with patients with cytoarchitectural abnormalities (FCD 1b, 2a and 2b according to Palmini). In patients with additional hippocampal sclerosis (dual pathology) febrile seizures were significantly more frequently reported (P = 0.02) than in patients without dual pathology. Moreover, patients with dual pathology and febrile seizures significantly more frequently presented with severe hippocampal sclerosis (Wyler Grade 3–4) as compared with patients with dual pathology in the absence of febrile seizures (P = 0.03). First observed seizures were mainly tonic or generalized tonic–clonic. A change of seizure semiology seemed to be age-dependent and occurred between the age of >1 and 14 years. About 15.8% of the patients presented with status epilepticus during the course of disease. About 17% of the patients showed transient responsiveness (
1 year seizure freedom) to antiepileptic drug therapy either after initial therapy (50%) or later in the course of epilepsy (50%). Patients with FCD represent a heterogeneous group. Different age at epilepsy onset and transient responsiveness to antiepileptic drugs in 
17% of patients may reflect different dynamics in epileptogenicity of the underlying FCD. Dual pathology may be associated with different pathomechanisms in patients with and without febrile seizures.
Key Words: focal cortical dysplasia; clinical characteristics; semiology; status epilepticus
Abbreviations: FCD, focal cortical dysplasia; mMCD, mild malformations of cortical development; MTD, mesial temporal damage
Received December 29, 2005. Revised March 17, 2006. Accepted April 20, 2006.
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