Journal of Neurological Sciences 2005;22(3) 314 Journal of Neurological Sciences [Turkish] 22:(3)#45; 314-318, 2005 http://www.jns.dergisi.org/text.php3?id=46 Case Report Catastrophic Childhood Temporal Lobe Epilepsy Giancarlo Di GENNARO 1 , Antonio SPARANO 1 , Addolorata MASCIA 1 , Fabio SEBASTIANO 1 , Vincenzo ESPOSITO 2 , Pier Paolo QUARATO 1 1 Epilepsy Surgery Unit, IRCCS Neuromed, Pozzilli (IS), Italy 2 Department of Neurosurgery, University of Rome “La Sapienza”, Rome, Italy Abstract Focal epilepsy, more frequently of extratemporal type, in children can present with a rapidly progressing course of intractable, severe epilepsy, associated to cognitive regression or stagnation. We present a typical example of such a patient with focal seizures due to a temporal lobe cortical lesion of developmental origin. Brain MRI revealed abnormalities in the right temporal lobe and Video-EEG monitoring revealed episodes clinically characterized by epileptic spasms followed by automotor seizures, with EEG findings suggestive of a right temporal lobe focus. Surgical resection resulted in excellent outcome. Keywords: presurgical evaluation, epilepsy surgery Çocukluk Çağı Katastrofik Temporal Lob Epilepsisi Özet Daha sık olarak ekstratemporal tip fokal epilepsi çocuklarda hızla progresyon gösteren direngen, ağır epilepsi ile birlikte kognitif bozukluk ve gerileme ile kendini gösterebilir. Gelişimsel kaynaklı temporal lob kortikal bir lezyona bağlı olarak gelişen fokal nöbetleri olan bir hastayı böyle tipik bir örnek olarak sunmaktayız. Beyin MRG tetkikleri sağ temporal lob anormallikleri, EEG bulguları sağ temporal lob fokusu gösterdi ve video-EEG kayıtlamasında da klinik ile uyumlu epileptik spazm olaylarını izleyen otomotor nöbetler gözlendi. Cerrahi rezeksiyon ile çok iyi sonuç elde edildi. Anahtar Kelimeler: Childhood temporal lobe epilepsy, catastrophic epilepsy Introduction The term catastrophic epilepsy is used to describe the clinical picture of severe, intractable epilepsy, usually with daily seizures and developmental stagnation or regression, typically in infancy or in the early childhood. Usually this clinical picture may be found in generalized epileptic encephalopathies such as West syndrome and Lennox- Gastaut syndrome. However, catastrophic epilepsy may be found in extra-temporal lobe focal epilepsies often due to cortical development malformations 1 . In these cases epilepsy surgery should be considered, often after invasive investigations. In the best scenario, the chance for freedom from seizures after surgery for catastrophic focal epilepsy due to malformation of cortical development appears to be in the range of 50% . Occasionally, in children with catastrophic