TY - JOUR
T1 - Idiopathic West Syndrome followed by childhood absence epilepsy
AU - Specchio, Nicola
AU - Trivisano, Marina
AU - Vigevano, Federico
AU - Fusco, Lucia
PY - 2010/11
Y1 - 2010/11
N2 - West Syndrome (WS) is a severe epileptic encephalopathy occurring in the first year of life. According the ILAE classification of epileptic seizures and epilepsy the etiology could be symptomatic or cryptogenic. Some authors identified a small group of patients (5%) with a particular good outcome, a complete recovery from seizures and a normal cognitive development within the cryptogenic group that they suggested to be idiopathic. Between 1996 and 2007, at the Neurology Division of the Bambino Ges Children's Hospital in Rome, we collected 241 patients with WS. Sixteen (6.6%) were considered with idiopathic aetiology. All clinical notes of these patients were reviewed in order to evaluate the prevalence of other epileptic syndrome after WS. Two of them had at the age of 8 and 3 months idiopathic WS, and at the age of 6 and 4 years respectively, they presented with childhood absence epilepsy (CAE) successfully treated with valproate. The favorable evolution of the WS and the later occurrence of an idiopathic form of epilepsy, such as CAE, confirm the possibility of an idiopathic aetiology for WS that, although rare, can represent one of the etiologies of otherwise severe syndrome. Even if a common physiophatogenetic role, probably related to a genetic predisposition, could be hypothesized and appears to be intriguing, no data are available and more studies are needed to confirm this hypothesis.
AB - West Syndrome (WS) is a severe epileptic encephalopathy occurring in the first year of life. According the ILAE classification of epileptic seizures and epilepsy the etiology could be symptomatic or cryptogenic. Some authors identified a small group of patients (5%) with a particular good outcome, a complete recovery from seizures and a normal cognitive development within the cryptogenic group that they suggested to be idiopathic. Between 1996 and 2007, at the Neurology Division of the Bambino Ges Children's Hospital in Rome, we collected 241 patients with WS. Sixteen (6.6%) were considered with idiopathic aetiology. All clinical notes of these patients were reviewed in order to evaluate the prevalence of other epileptic syndrome after WS. Two of them had at the age of 8 and 3 months idiopathic WS, and at the age of 6 and 4 years respectively, they presented with childhood absence epilepsy (CAE) successfully treated with valproate. The favorable evolution of the WS and the later occurrence of an idiopathic form of epilepsy, such as CAE, confirm the possibility of an idiopathic aetiology for WS that, although rare, can represent one of the etiologies of otherwise severe syndrome. Even if a common physiophatogenetic role, probably related to a genetic predisposition, could be hypothesized and appears to be intriguing, no data are available and more studies are needed to confirm this hypothesis.
KW - Absences
KW - Childhood absence epilepsy
KW - Idiopathic epilepsy
KW - Infantile spasms
KW - West Syndrome
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U2 - 10.1016/j.seizure.2010.07.016
DO - 10.1016/j.seizure.2010.07.016
M3 - Article
C2 - 20729098
AN - SCOPUS:77958001706
VL - 19
SP - 597
EP - 601
JO - Seizure : the journal of the British Epilepsy Association
JF - Seizure : the journal of the British Epilepsy Association
SN - 1059-1311
IS - 9
ER -