TY - JOUR
T1 - Cognitive development in Dravet syndrome
T2 - A retrospective, multicenter study of 26 patients
AU - Ragona, Francesca
AU - Granata, Tiziana
AU - Bernardina, Bernardo Dalla
AU - Offredi, Francesca
AU - Darra, Francesca
AU - Battaglia, Domenica
AU - Morbi, Monica
AU - Brazzo, Daniela
AU - Cappelletti, Simona
AU - Chieffo, Daniela
AU - De Giorgi, Ilaria
AU - Fontana, Elena
AU - Freri, Elena
AU - Marini, Carla
AU - Toraldo, Alessio
AU - Specchio, Nicola
AU - Veggiotti, Pierangelo
AU - Vigevano, Federico
AU - Guerrini, Renzo
AU - Guzzetta, Francesco
AU - Dravet, Charlotte
PY - 2011/2
Y1 - 2011/2
N2 - Purpose: To clarify the role of epilepsy and genetic background in determining the cognitive outcome of patients with Dravet syndrome. Methods: In this retrospective study, we reviewed the clinical history and cognitive development of 26 patients who had been followed with standardized evaluations since seizure onset. The cognitive outcome was quantified as differential general quotient (dGQ) between ages 12 and 60 months. Statistical analysis correlated the dGQ with genotype and epilepsy course. Key Findings: Epilepsy started at the mean age of 5.6 months. All patients experienced prolonged convulsive seizures, whereas absences and myoclonus were reported in 17. Cognitive outcome was poor in almost all patients; the mean dGQ was 33 points, varying from 6-77 points. The analysis of individual cognitive profiles identified seven patients in whom the dGQ was
AB - Purpose: To clarify the role of epilepsy and genetic background in determining the cognitive outcome of patients with Dravet syndrome. Methods: In this retrospective study, we reviewed the clinical history and cognitive development of 26 patients who had been followed with standardized evaluations since seizure onset. The cognitive outcome was quantified as differential general quotient (dGQ) between ages 12 and 60 months. Statistical analysis correlated the dGQ with genotype and epilepsy course. Key Findings: Epilepsy started at the mean age of 5.6 months. All patients experienced prolonged convulsive seizures, whereas absences and myoclonus were reported in 17. Cognitive outcome was poor in almost all patients; the mean dGQ was 33 points, varying from 6-77 points. The analysis of individual cognitive profiles identified seven patients in whom the dGQ was
KW - Cognitive development
KW - Epileptic status
KW - Myoclonus
KW - SCN1A
KW - Severe myoclonic epilepsy in infancy
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UR - http://www.scopus.com/inward/citedby.url?scp=79951678915&partnerID=8YFLogxK
U2 - 10.1111/j.1528-1167.2010.02925.x
DO - 10.1111/j.1528-1167.2010.02925.x
M3 - Article
C2 - 21269283
AN - SCOPUS:79951678915
VL - 52
SP - 386
EP - 392
JO - Epilepsia
JF - Epilepsia
SN - 0013-9580
IS - 2
ER -