Epileptic Disorders


Epilepsy surgery in the first months of life: a large type IIb focal cortical dysplasia causing neonatal drug-resistant epilepsy Volume 21, numéro 1, February 2019


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1 Divison of Pediatric Neurology, Developmental Neurology and Social Pediatrics, Department of Pediatrics, University Hospital LMU Munich
2 Epilepsy Center for children, adolescents and adults, University Hospital LMU Munich
3 Department of Pediatrics and Adolescent Medicine, Hospital Dritter Orden, Munich
4 Department of Neuropathology, Neuropathological Reference Center for Epilepsy Surgery, University Hospital Erlangen, Erlangen
5 Department of Neuropathology, University Hospital LMU, Munich
6 Department of Radiology, University Hospital LMU, Munich
7 Institute of Human Genetics, Friedrich-Alexander-Universität Erlangen-Nürnberg, Erlangen
8 Department of Neurology, University Hospital LMU, Munich
9 Department of Neurosurgery, Section for Pediatric Neurosurgery, University of Ulm, Germany
* Correspondence: Ingo Borggraefe Dr. von Hauner‘s Childens Hospital, University of Munich, Germany

Focal cortical dysplasia is a common cause of medically refractory epilepsy in infancy and childhood. We report a neonate with seizures occurring within the first day of life. Continuous video-EEG monitoring led to detection of left motor seizures and a right frontal EEG seizure pattern. Brain MRI revealed a lesion within the right frontal lobe without contrast enhancement. The patient was referred for epilepsy surgery due to drug resistance to vitamin B6 and four antiepileptic drugs. Lesionectomy was performed at the age of two and a half months, and histopathological evaluation confirmed the diagnosis of focal cortical dysplasia type IIb (FCD IIb). The patient is free of unprovoked seizures without medication (Engel Class I) and is normally developed at 36 months after surgery. The case study demonstrates that FCD IIb may cause seizures within the first day of life and that epilepsy surgery can be successfully performed in medically intractable patients with a clearly identifiable seizure onset zone within the first three months of life. Although radical surgery such as hemispherectomy and multi-lobar resections are over-represented in early infancy, this case also illustrates a favourable outcome with a more limited resection in this age group.