title: Clinical characteristics in focal cortical dysplasia: a retrospective evaluation in a series of 120 patients.
abstract: 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 (> or =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 approximately 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.
authors.affiliation: Department of Epilepsy Centre, University of Freiburg Kehl-Kork, Germany. fauser@nz11.ukl.uni-freiburg.de
authors.forename: Susanne
authors.initials: S
authors.lastname: Fauser
authors.forename: Hans-Juergen
authors.initials: HJ
authors.lastname: Huppertz
authors.forename: Thomas
authors.initials: T
authors.lastname: Bast
authors.forename: Karl
authors.initials: K
authors.lastname: Strobl
authors.forename: Georgios
authors.initials: G
authors.lastname: Pantazis
authors.forename: Dirk-Matthias
authors.initials: DM
authors.lastname: Altenmueller
authors.forename: Bertram
authors.initials: B
authors.lastname: Feil
authors.forename: Sabine
authors.initials: S
authors.lastname: Rona
authors.forename: Christoph
authors.initials: C
authors.lastname: Kurth
authors.forename: Dietz
authors.initials: D
authors.lastname: Rating
authors.forename: Rudolf
authors.initials: R
authors.lastname: Korinthenberg
authors.forename: Bernhard J
authors.initials: BJ
authors.lastname: Steinhoff
authors.forename: Benedikt
authors.initials: B
authors.lastname: Volk
authors.forename: Andreas
authors.initials: A
authors.lastname: Schulze-Bonhage
mesh_terms: D000293:Adolescent; D000328:Adult; D017668:Age of Onset; D000927:Anticonvulsants / Q000627:therapeutic use; D002540:Cerebral Cortex / Q000002:abnormalities*; D002648:Child; D002675:Child, Preschool; D018450:Disease Progression; D004351:Drug Resistance; D004828:Epilepsies, Partial / Q000188:drug therapy / Q000209:etiology* / Q000473:pathology; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D008279:Magnetic Resonance Imaging; D008875:Middle Aged; D012189:Retrospective Studies; D003294:Seizures, Febrile / Q000209:etiology / Q000473:pathology; D012720:Severity of Illness Index; D013226:Status Epilepticus / Q000209:etiology / Q000473:pathology; D016896:Treatment Outcome
chemical_list: D000927:Anticonvulsants