C. Dravet, M. Bureau, H. Oguni, M. Bureau, C. Dravet et al., Severe Myoclonic Epilepsy in Infancy (Dravet Syndrome) In Epileptic Syndromes in Infancy, Childhood and Adolescence, pp.75-88

C. Dravet, M. Bureau, H. Oguni, Y. Fukuyama, and O. Cokar, Dravet syndrome (severe myoclonic epilepsy in infancy), Adv Neurol, vol.95, pp.71-102, 2005.
DOI : 10.1016/B978-0-444-52891-9.00065-8

L. Claes, J. Del-favero, B. Ceulemans, L. Lagae, C. Van-broeckhoven et al., De Novo Mutations in the Sodium-Channel Gene SCN1A Cause Severe Myoclonic Epilepsy of Infancy, The American Journal of Human Genetics, vol.68, issue.6, pp.1327-1332, 2001.
DOI : 10.1086/320609

C. Depienne, D. Bouteiller, K. B. Cheuret, E. Poirier, K. Trouillard et al., Sporadic Infantile Epileptic Encephalopathy Caused by Mutations in PCDH19 Resembles Dravet Syndrome but Mainly Affects Females, PLoS Genetics, vol.295, issue.2, p.1000381, 2009.
DOI : 10.1371/journal.pgen.1000381.t001

R. Nabbout and O. Dulac, Epileptic syndromes in infancy and childhood, Current Opinion in Neurology, vol.21, issue.2, pp.161-166, 2008.
DOI : 10.1097/WCO.0b013e3282f7007e

C. Catarino, J. Liu, I. Liagkouras, V. Gibbons, R. Labrum et al., Dravet syndrome as epileptic encephalopathy: evidence from long-term course and neuropathology, Brain, vol.134, issue.10, pp.2982-3010, 2011.
DOI : 10.1093/brain/awr129

URL : http://doi.org/10.1093/brain/awr129

M. Wolff, C. Casse-perrot, and C. Dravet, Severe Myoclonic Epilepsy of Infants (Dravet Syndrome): Natural History and Neuropsychological Findings, Epilepsia, vol.28, issue.s2, pp.4745-4793, 2006.
DOI : 10.1002/ana.410140407

A. Brunklaus, R. Ellis, E. Reavey, G. Forbes, and S. Zuberi, Prognostic, clinical and demographic features in SCN1A mutation-positive Dravet syndrome, Brain, vol.135, issue.8, pp.2329-2336, 2012.
DOI : 10.1093/brain/aws151

D. Riva, C. Vago, C. Pantaleoni, S. Bulgheroni, M. Mantegazza et al., Progressive neurocognitive decline in two children with Dravet syndrome, de novo SCN1A truncations and different epileptic phenotypes, American Journal of Medical Genetics Part A, vol.15, issue.10, pp.2339-2345, 2009.
DOI : 10.1002/ajmg.a.33029

F. Ragona, D. Brazzo, D. Giorgi, I. Morbi, M. Freri et al., Dravet syndrome: Early clinical manifestations and cognitive outcome in 37 Italian patients, Brain and Development, vol.32, issue.1, pp.71-77, 2010.
DOI : 10.1016/j.braindev.2009.09.014

F. Ragona, T. Granata, D. Bernardina, B. Offredi, F. Darra et al., Cognitive development in Dravet syndrome: A retrospective, multicenter study of 26 patients, Epilepsia, vol.47, issue.suppl 2, pp.386-392, 2011.
DOI : 10.1111/j.1528-1167.2010.02925.x

R. Caraballo and N. Fejerman, Dravet syndrome: A study of 53 patients, Epilepsy Research, vol.70, issue.1, pp.231-238, 2006.
DOI : 10.1016/j.eplepsyres.2005.11.026

C. Marini, D. Mei, T. Temudo, A. Ferrari, D. Buti et al., Abnormalities, Epilepsia, vol.61, issue.9, pp.1678-1685, 2007.
DOI : 10.1111/j.1528-1167.2007.01122.x

D. Chieffo, D. Battaglia, D. Lettori, D. Re, M. Brogna et al., Neuropsychological development in children with Dravet syndrome, Epilepsy Research, vol.95, issue.1-2, pp.86-93, 2011.
DOI : 10.1016/j.eplepsyres.2011.03.005

F. Guzzetta, Cognitive and behavioral characteristics of children with Dravet syndrome: An overview, Epilepsia, vol.9, issue.suppl 2, pp.35-38
DOI : 10.1111/j.1528-1167.2011.02999.x

A. Berg, S. Berkovic, M. Brodie, J. Buchhalter, J. Cross et al., Revised terminology and concepts for organization of seizures and epilepsies: report of the ILAE Commission on Classification and Terminology, Epilepsia, vol.51, issue.4, pp.2005-2009676, 2010.

D. Wechsler, Echelle d'Intelligence de Wechsler pour la période Préscolaire et Primaire, Les Editions du Centre de Psychologie Appliquée, 2004.

D. Wechsler, Echelle d'Intelligence de Wechsler pour Enfants. Paris: Les Editions du Centre de Psychologie Appliquée, 2005.

T. Achenbach, Manual for the Child Behavior Checklist/4?18 and 1991 Profile, 1991.

C. Conners, . Conners-'rating, and . Scales, RevisedMulti Health Systems, 1997.

S. Sparrow, N. Carrey, D. Wiggins, R. Millin, and S. Hosendocus, American Guidance Service, Vineland Adaptive Behavior Scales. Circle Pines, 1984.

M. Chipaux, N. Villeneuve, P. Sabouraud, I. Desguerre, N. Boddaert et al., Unusual consequences of status epilepticus in Dravet syndrome, Seizure, vol.19, issue.3, pp.190-194, 2010.
DOI : 10.1016/j.seizure.2010.01.007

M. Tsuji, E. Mazaki, I. Ogiwara, T. Wada, M. Iai et al., Acute Encephalopathy in a Patient with Dravet Syndrome, Neuropediatrics, vol.42, issue.02, pp.78-81, 2011.
DOI : 10.1055/s-0031-1279725

P. Parisi, A. Spalice, F. Nicita, L. Papetti, F. Ursitti et al., “Epileptic Encephalopathy” of Infancy and Childhood: Electro-Clinical Pictures and Recent Understandings, Current Neuropharmacology, vol.8, issue.4, pp.409-421, 2010.
DOI : 10.2174/157015910793358196

D. Chieffo, D. Ricci, G. Baranello, D. Martinelli, C. Veredice et al., Early development in Dravet syndrome; visual function impairment precedes cognitive decline, Epilepsy Research, vol.93, issue.1, pp.73-79, 2011.
DOI : 10.1016/j.eplepsyres.2010.10.015

C. Chiron, I. Jambaque, R. Nabbout, R. Lounes, A. Syrota et al., The right brain hemisphere is dominant in human infants, Brain, vol.120, issue.6, pp.1057-1065, 1997.
DOI : 10.1093/brain/120.6.1057

H. Ishino, S. Higashi, M. Chuta, and H. Ohta, Juvenile Alzheimer's disease with myoclonus: amyloid plaques and grumose alteration in the cerebellum, Clin Neuropathol, vol.3, pp.193-198, 1984.

K. Bhatia, P. Brown, R. Gregory, G. Lennox, H. Manji et al., Progressive myoclonic ataxia associated with coeliac disease, Brain, vol.118, issue.5, pp.1087-1093, 1995.
DOI : 10.1093/brain/118.5.1087

URL : http://doi.org/10.1093/brain/118.5.1087

L. Abbott, M. Bump, A. Brandl, and S. De-laune, Investigation of the role of the cerebellum in the myoclonic-like movement disorder exhibited by tottering mice, Movement Disorders, vol.15, issue.S1, pp.53-59, 2000.
DOI : 10.1002/mds.870150710

K. Koh, B. Lim, H. Hwang, J. Park, J. Chae et al., Cerebellum Can Be a Possible Generator of Progressive Myoclonus, Journal of Child Neurology, vol.25, issue.6, pp.728-731, 2010.
DOI : 10.1177/0883073809342273

A. Lorincz and Z. Nusser, Cell-Type-Dependent Molecular Composition of the Axon Initial Segment, Journal of Neuroscience, vol.28, issue.53, pp.14329-14340, 2008.
DOI : 10.1523/JNEUROSCI.4833-08.2008