The 8th Ann Mtg (2005) – International Symposium – The West Syndrome & Other Infantile Epileptic Encephalopathies
Date April 29 – May 1, 2005 Venue The Yayoi Memorial Hall 8-1, Kawada-cho, Shinjuku-ku, Tokyo 162-8666, Japan
Day 1 Friday, April 29 ☛ Day 2 ☛ Day 3 08:15-18:00 REGISTRATION 09:00 Opening Address Yukio FUKUYAMA Child Neurology Institute, Tokyo, Japan 09:10 ࠘Seizures in the Neonatal Period࠙
Chairpersons : Moshé SL (New York, USA) Watanabe K (Nagoya, Japan) 09:10-09:50 Classification of Neonatal Seizures: 1 A Fetal Neurology Perspective
Mark S. SCHER Devision of Pediatric Neurology, Department of Pediatrics, Rainbow Babies and Children’s Hospital, Cleveland, OH, USA 09:50-10:20 Benign Familial and Non-Familial Neonatal Seizures 2 Perrine PLOUIN Clinical Neurophysiology Department, Hôpital Necker Enfants Malades, Paris, France
Chairpersons : Nordli DR Jr (Chicago, USA) Eto Y (Tokyo, Japan) 10:20-11:00 Glut1 Deficiency: A Metabolic Model for Infantile Seizures 3 Darryl C. DE VIVO, Dong WANG, Juan M. PASCUAL The Colleen Giblin Research Laboratories for Pediatric Neurology, Neurological Institute, Columbia University Medical Center, New York, NY, USA
11:00-11:15 Clinical and Neurophysiological Manifestations of Glucose 4 Transporter-1 Deficiency Syndrome in Japan
Keiko YANAGIHARA, Noriko KAMIO, Toshisaburo NAGAI, Yasuhiro SUZUKI, Hiroshi ARAI, Shihoko OHBA, Shin NABATAME, Takeshi OKINAGA, Keiichi OZONO, Minoru YAMADA, Itaru YANAGIHARA, Katsumi IMAI
Research Institute and Divisionof Pediatric Neurology, Osaka Medical Center for Maternal and Child Health, Osaka, Japan Department of Pediatrics, and Allied Health Sciences, Osaka University Graduate School of Medicine, Osaka, Japan Department of Pediatric Neurology, Bobath Memorial Hospital, Osaka, Japan
Chairpersons : Scher MS (Cleveland, USA) Iinuma K (Sendai, Japan)
11:15-11:45 Ohtahara Syndrome: With Special Reference to its Developmental 5 Aspects for Differentiating from the Early Myoclonic Encephalopathy
Shunsuke OHTAHARA Department of Child Neurology, Okayama University Medical School, Okayama, Japan 11:45-12:15 Are Early Myoclonic Encephalopathy (EME) and the Ohtahara 6 Syndrome Independent of Each Other ?
Solomon L.MOSHÉ, Aleksandra DJUKIC, Shlomo SHINNAR
Department of Neurology, Neuroscience and Pediatrics, Albert Einstein Collegeof Medicine, Montefiore Medical Center, Bronx, New York, NY, USA 12:15-12:30 GENERAL DISCUSSION 7 12:30-13:30 LUNCH AND POSTER VISIT 12:40-13:20 Luncheon Seminar (1) Chairpersons : Yagi K (Shizuoka, Japan) Co-sponsored by Nikken Chemicals Co., Ltd, Japan
【Visual Sensitivity and Photosensitivity】
Kasteleijn-Nolst Trenité DGA (Utrecht, Netherlands) 13:30 【Epileptic Encephalopathies】
Chairpersons : Specchio N (Rome, Italy) Ohtahara S (Okayama, Japan)
13:30-14:05 Epileptic Encephalopathies - An Overview 8 Natalio FEJERMAN Honorary consultant. Service of Neurology. “Juan P Garrahan”Pediatric Hospital. Buenos Aires, Argentina 14:05-14:40 How Does Etiology Influence Ictal Semiology, Treatment Choice 9 and Long-Term Outcomes in Infantile Spasms and West Syndrome?
Andrew L. LUX, John P. OSBORNE Department of Paediatric Neurology, British Royal Hospital for Children, Bristol, UK School for Health, University of Bath, UK Bath Unit for Research in Paediatrics, Royal United Hospital, Bath, UK ࠈ Chairpersons : Sankar R (Los Angeles, USA) Niijima S (Tokyo, Japan) ࠈࠈࠈࠈࠈ ࠈ ࠈࠈࠈࠈࠈࠈࠈࠈࠈࠈࠈ 14:40-15:00 Mutation Analysis of the ARX Gene in Patients with West 10 Syndrome
Mitsuhiro KATO, Takashi SHIIHARA, Jun TOHYAMA, Noriyuki AKASAKA, Yasuhiro SUZUKI, Shinji FUJIMOTO, Kiyoshi HAYASAKA Department of Pediatrics, Yamagata University School of Medicine, Yamagata, Japan Epilepsy Center, Department of Pediatrics, Nishi-Niigata Chuo National Hospital, Niigata, Japan Division of Pediatric Neurology, Osaka Medical Center and Research Institute for Maternal and Child Health, Osaka, Japan Department of Pediatrics, Neonatalogy and Congenital Disorders, Nagoya City University Graduate School of Medical Sciences, Nagoya, Aichi, Japan
15:00-15:20 Migrating Partial Seizures in Infancy 11 PerrinePLOUIN, Olivier DULAC
Clinical Neurophysiology Department, Hôpital Necker Enfants Malades, Paris, France Paediatric Neurology Department, Hôpital Necker Enfants Malades, Paris, France
15:20-15:40 Severe Epilepsy with Multiple Independent Spike Foci 12 Yasuko YAMATOGI, Shunsuke OHTAHARA
Faculty of Health and Welfare Science, Okayama Prefectural University, Soja and Okayama, Japan Department of Child Neurology, Okayama University Medical School, Okayama, Japan 16:10 ࠘How to Classify ? ࠙
Chairpersons : Andermann F (Montreal, Canada) Chang K-P (Taipei, Taiwan) 16:10-16:30 Unclassified Infantile Epilepsies : Infants with Both Generalized 13 and Focal Features
Douglas R. NORDLI, Jr.., Kent R. KELLEY, Linda LAUX, Joshua GOLDSTEIN, Christian M. KORFF, Laurel BUCHANAN, Pulio SILVA
Children’s Memorial Epilepsy Center, Chicago, IL, USA 16:30-16:50 Diagnostic Issues of Cryptogenic or Symptomatic Generalized 14 Epilepsies
Yoko OHTSUKA, Harumi YOSHINAGA, Katsuhiro KOBAYASHI, Tatsuya OGINO
Department of Child Neurology, Okayama University Graduate School of Medicine and Dentistry, Okayama, Japan 16:50-17:10 Are Absences Truly Generalized Seizures? 15 Dana CRAIU, Sanda MAGUREANU
Pediatric Neurology Clinic, National Reference Center Against Epilepsy, Alexandru Obregia Hospital, Bucharest, Romania ࠈࠈ Chairpersons : Lux A (Bristol, UK) Visudtibhan A (Bangkok, Thailand) 17:10-17:30 Benign Myoclonic Epilepsy: Is the Outcome so Benign? 16 Mary E. O’REGAN, Sameer M. ZUBERI Fraser of Allander Neuroscience Unit, Royal Hospital for Sick Children, Glasgow, UK 17:30-17:50 Electro-clinical Characterization of Infantile Seizures 17 Douglas R. NORDLI Jr., Christian M. KORFF Children’s Memorial Epilepsy Center, Chicago, IL, USA 17:50-18:00 ࠘General Discussion࠙ 18 18:30-22:00 WELCOME RECEPETION Venue: Restaurant “Kansui”, Rokubancho, Chiyoda-ku
☛ Day 1 ☛ Day 3
Day 2 Saturday, April 30 08:15- 18:00 REGISTRATION 08:30 ࠘Epilepsy Classification by ILAE Task Force࠙
Chairpersons : Engel J Jr (Los Angeles, USA) Seino M (Shizuoka, Japan)
08:30- 09:00 ILAE Classification of Epileptic Syndromes : Progress on Revising 19 the ILAE Classification of Seizures and Epilepsy
Jerome ENGEL, Jr. Department of Neurology and Neurobiology, David Geffen School of Medicine at UCLA, Los Angeles, CA,USA 09:00- 09:30 Concepts in Classification and Their Relevance to Epilepsy 20 Anne T. BERG Department of Biology, Northern Illinois University, DeKalb, IL, USA 09:30- 10:00 Epileptic Seizures, Syndromes and Epilepsies - A Conceptual Issue 21 Masakazu SEINO National Epilepsy Center, Shizuoka Institute of Epilepsy and Neurological Disorders, Shizuoka, Japan 10:00- 10:30 The Classification of Idiopathic Generalized Epilepsies: Time for 22 Re-evaluation?
Frederick ANDERMANN, Samuel F. BERKOVIC Department of Neurology and Pediatrics,McGill University; Epilepsy Service, Montreal Neurological Hospital and Institute, Montreal, Quebec, Canada Austin and Repatriation Medical Center, Epilepsy Research Institute, Melbourne, Victoria, Australia 10:30- 11:00 Basic Principles of the ILAE Syndrome Classification 23 Peter WOLF The Danish Epilepsy Centre, Dianalund, Denmark, and The National Hospitals Neurocentre, Copenhagen, Denmark 11:00- 12:00 GENERAL DISCUSSION 24 12:00- 13:05 LUNCH AND POSTER VISIT 12:15-12:55 Luncheon Seminar (2)
ࠈ Chairperson: Takahashi T (Tokyo, Japan)
࠘Epilepsies in Developing Animals and Humans࠙
Solomon L. MOSHÉ ( New York, USA)
13:05 ࠘Epilepsy Prognosis in Children࠙
Chairpersons : Salonga AM (Manila, Philippines) Osawa M (Tokyo, Japan) 13:05- 13:40 The Prognosis of Childhood-Onset Epilepsy 25 Anne T. BERG Department of Biology,Northern Illinois University, DeKalb,IL, USA 13:40 ࠘Benign Infantile Seizures Complex - Part 1࠙
Chairpersons : Fejerman N (Buenos Aires, Argentina) Yamano T (Osaka, Japan) 13:40- 14:15 Benign Familial Infantile Seizures 26 Nicola SPECCHIO, Federico VIGEVANO Head of Neurology Division, Bambino Gesù Children Hospital, Rome, Italy 14:15- 14:40 Benign Partial Epilepsy in Infancy (Watanabe Syndrome) and its 27 Marginal Syndromes. Aspects from a Long-Term Follow-up
Akihisa OKUMURA, Kazuyoshi WATANABE, Tamiko NEGORO, Fumio HAYAKAWA, Toru KATO, Kazuya ITOMI, Seiko ITOMI, Taketo IKUTA Department of Pediatrics, Nagoya University Graduate School of Medicine, Nagoya, Aichi, Japan Faculty of Medical Welfare,Aichi Shukutoku University, Nagakute, Aichi, Japan 14:40- 15:00 Benign Infantile Seizures in Japan : Clinical Manifestations and 28 Good Efficacy of Low Dose CBZ
Keiko YANAGIHARA, Kazumasa OTANI, Katsumi IMAI, Collaborate Clinical Research Group for Pediatric Neurology, Osaka University Graduate School of Medicine Research Institute, Osaka Medical Center for Maternal and Child Health, Osaka, Japan Otani Clinic, Wakayama, Japan Department of Pediatrics, Osaka University Graduate School of Medicine, Osaka, Japan 15:00- 15:20 General Discussion 29
15:20- 15:50 INTERMISSION AND POSTER VISIT 15:50 ࠘Benign Infantile Seizures Complex - Part 2࠙
Chairpersons : Plouin P (Paris, France) Matsuishi T (Kurume, Japan) 15:50- 16:15 Benign Infantile Focal Epilepsy with Midline Spikes and Waves 30 During Sleep (BIMSE)
Giuseppe CAPOVILLA Epilepsy Center, C. Poma Hospital, Mantova, Italy 16:15- 16:40 First Afebrile Seizures Associated with Non-CNS Infections 31 Comprise a Distinct Seizure Syndrome
Wei-Ling LEE, Hian-Tat ONG Department of Neurology, National Neuroscience Institute, Singapore and Department of Paediatrics, National University of Singapore, Singapore Department of Paediatrics, National University of Singapore, Singapore 16:40- 17:00 Clinical and Genetic Analysis of Paroxysmal Kinesigenic 32 Choreoathetosis - A Possible Association with Benign Familial Neonatal Convulsion and Infantile Convulsions and Choreoathetosis –
Nobumasa KATO, Taeko KIKUCHI, Miyuki SADAMATSU, Tatsuya FUJII, Kayoko SAITO, Norio NIIKAWA, Yukio FUKUYAMA Department of Neuropsychiatry, Graduate School of Medicine, University of Tokyo, Tokyo, Japan Department of Human Genetics, Nagasaki University, Graduate School of Biomedical Sciences, Nagasaki, Japan Department of Psychiatry, Shiga University of Medical Science, Otsu, Shiga, Japan Department of Pediatrics, Shiga Medical Center for Children, Moriyama, Shiga, Japan Department of Pediatrics, Tokyo Women’s Medical University, School of Medicine, Tokyo, Japan
Chairpersons : Hwang YS (Seoul, Korea) Abe T (Tokyo, Japan)
17:00- 17:20 Classification of Benign Infantile Seizures; Does Seizure Type Play 33 an Important Role in Diagnosis?
Takuya TANABE, Keita HARA, Mitsuru KASHIWAGI, Hiroshi TAMAI Division of Pediatrics, Hirakata City Hospital, Osaka, Japan Department of Pediatrics, Osaka Medical College, Osaka, Japan 17:20- 17:40 Benign Convulsions with Mild Gastroenteritis in Infancy: Clinical 34 Features, Laboratory Findings, and Follow-up Results
Dong Wook KIM, Jung Il CHO Department of Pediatrics, Clinical Research Center, Inje University Ilsan Paik Hospital, Goyang, Korea 17:40- 18:00 GENERAL DISCUSSION 35
18:30- 22:00 SOCIAL PARTY - TOKYO EVENING Venue: Arcadia Ichigaya - Daisetsu Room (5th Floor) ☛ Day 1 ☛ Day 2
Day 3 Sunday, May 1 08:15- 13:10 REGISTRATION
08:30 【Landau-Kleffner Syndrome】 Chairpersons : Kasteleijn-Nolst Trenite DGA (Utrecht, Netherlands) Segawa M (Tokyo, Japan) 08:30-09:05 Landau-Kleffner Syndrome and CSWS. Are not Eponymic Badge 36 of Ignorance ?
Edouard HIRSCH Department of Neurology, Hôspitaux Universitaires, Strasbourg,, France 09:05-09:40 Children with CSWS - Variability in the Syndrome 37 Raman SANKAR, Michele VAN HIRTUM-DAS, Eliot LICHT, Susan KOH, Joyce WU Department of Pediatrics and Neurology, David Geffen School of Medicine at UCLA, Los Angeles, CA, USA 09:40 【Fever-Sensitive Seizures】
Chairpersons : De Vivo DC (New York, USA) Oguni H (Tokyo, Japan) 09:40-10:00 Clinical Study of Childhood Epilepsies with Predominantly Fever- 38 sensitive Seizures and Onset in the First Year of Life
Kitami HAYASHI, Hirokazu OGUNI, Makiko OSAWA, Yukio FUKUYAMA Department of Pediatrics, Tokyo Women’s Medical University, Tokyo, Japan Child Neurology Institute, Tokyo, Japan 10:00-10:25 Complex Genetics of Febrile Seizures 39 Junko NAKAYAMA Department of Neurology, University of California San Francisco, San Francisco, CA, USA Department of Medical Genetics, University of Tsukuba, Ibaraki, Japan 10:25-10:45 Phenotypes and Genotypes in Epilepsy with Febrile Seizures Plus 40 Masatoshi ITO, Kazuhiro YAMAKAWA, Takashi SUGAWARA, Shinichi HIROSE, Goryu FUKUMA, Sunao KANEKO Department of Pediatrics, Shiga Medical Center for Children, Moriyama, Shiga, Japan Laboratory for Neurogenetics, RIKEN, Brain Science Institute, Wako, Saitama, Japan Department of Pediatrics, School of Medicine, Fukuoka University, Fukuoka, Japan Department of Neuropsychiatry, School of Medicine, Hirosaki University, Hirosaki, Aomori, Japan 10:45 【Dravet Syndrome / SMEI and Related Conditions】
Chairpersons : Hirsch E (Strasbourg, France) Kim HD (Seoul, Korea) 10:45-11:25 SCNA1 Mutations : From Benign Seizure Disorders to Epileptic 41 Encephalopathies
Ingrid SCHEFFER, John C. MULLEY, Jacinta MACMAHON, Samuel F. BERKOVIC, Louise HARKIN University of Melbourne, Austin Health, Royal Children’s Hospital, Monash Medical Centre, Melbourne, Australia Women’s and Children’s Hospital, University of Adelaide, Adelaide, Australia 11:25-11:50 Study of Genotypes and Phenotypes of Severe Myoclonic Epilepsy 42 in Infancy and Related Epilepsies
Tateki FUJIWARA National Epilepsy Center, Shizuoka Institute of Epilepsy and Neurological Disorder, Shizuoka, Japan 11:50-12:10 GENERAL DISCUSSION 43 12:10-13:10 LUNCH AND POSTER VISIT 12:20-13:00 Luncheon Seminar (3) Chairperson : Hori T (Tokyo, Japan) Co-sponsored by Kyowa Hakko Kogyo Co., Ltd, Japan 【Surgical Treatment of Intractable Epilepsies in Children】
Engel J Jr (Los Angeles, USA) 13:10 【Dravet Syndrome / SMEI - Molecular Genetics】
Chairpersons : Scheffer I (Melbourne, Australia) Ito M (Moriyama, Japan) 13:10-13:30 Genetic Analysis of Neuronal Voltage-gated Na+-channel Subunits 44 in Core Severe Myoclonic Epilepsy in Infancy (SMEI) and in Borderline SMEI (SMEB)
Shinichi HIROSE, Goryu FUKUMA, Hirokazu OGUNI, Yukiyoshi SHIRASAKA, Kazuyoshi WATANABE, Tasuku MIYAJIMA, Sawa YASUMOTO, Masaharu OHFU, Takahito INOUE, Aruchalean WATANACHAI, Ryutaro KIRA, Muneaki MATSUO, Hideki MURANAKA, Fumiko SOFUE, Sunao KANEKO, Akihisa MITSUDOME Department of Pediatrics, Fukuoka University, Fukuoka, Japan Department of Pediatrics, Tokyo Women’s Medical University,Tokyo, Japan Department of Pediatrics, Kobe City General Hospital, Kobe, Hyogo, Japan Department of Pediatrics, Nagoya University, Nagoya, Aichi, Japan Department of Pediatrics, Tokyo Medical University, Tokyo, Japan Department of Pediatrics, Kyushu University, Fukuoka, Japan Department of Pediatrics, Saga Medical School, Saga, Japan Muranaka Clinic, Hirosaki, Aomori, Japan Department of Neuropsychiatry, Hirosaki University, Hirosaki, Aomori, Japan
On behalf of The Epilepsy Genetic Study Group, Japan ( Chariperson, S.K.) 13:30-13:50 Effect of Localization of Missense Mutations in SCN1A on Epilepsy 45 Phenotype Severity
Kazuaki KANAI, Shinichi HIROSE, Hirokazu OGUNI, Goryu FUKUMA, Yukiyoshi SHIRASAKA, Tasuku MIYAJIMA, Satoshi KUWABARA, Kazumaru WADA, Hiroto IWASA, Sawa YASUMOTO, Muneaki MATSUO, Takamichi HATTORI, Masatoshi ITO, Akihisa MITSUDOME, Sunao KANEKO Department of Neuropsychiatry, Hirosaki University School of Medicine, Hirosaki, Aomori, Japan Department of Pediatrics, Fukuoka University, Fukuoka, Japan Department of Pediatrics, Tokyo Women’s Medical University, Tokyo, Japan Kobe City General Hospital, Kobe, Kyogo, Japan Department of Pediatrics, Tokyo Medical University, Tokyo, Japan Department of Occupational Therapy, School of Health Science, Hirosaki University, Hirosaki, Aomori, Japan Department of Pediatrics, Saga Medical School, Saga, Japan Department of Pediatrics, Shiga Medical Center for Children, Moriyama, Shiga, Japan Department of Neurology, Chiba University School of Medicine, Chiba, Japan 13:50-14:15 Biophysical Characterization of SMEI-associated SCN1A 46 Mutations
Iori OHMORI, Dao W. WANG, Thomas H. RHODES, Alfred L. GEORGE Jr. Division of Genetic Medicine, Vanderbilt University, Nashville, TN, USA Department of Medicine, Vanderbilt University, Nashville, TN, USA Department of Pharmacology, Vanderbilt University, Nashville, TN, USA Department of Cellular Physiology, Okayama University Graduate School of Medicine and Dentistry, Okayama, Japan 14:15-14:40 Sodium Channel Mutations - Gain or Loss of Function ? 47 Kazuhiro YAMAKAWA Laboratory for Neurogenetics, RIKEN Brain Science Institute, Wako, Saitama, Japan 14:40-15:00 GENERAL DISCUSSION 48 15:00-15:30 INTERMISSION AND POSTER VISIT 15:30 【Rasmussen Syndrome; Potential New Syndromes】
Chairpersons : Capovilla G (Mantova, Italy) Kohyama J (Tokyo, Japan) 15:30-16:05 Rasmussen Syndrome in Young Children 49 Frederick ANDERMANN Department of Neurology and Pediatrics, McGill University; Epilepsy Service, Montreal Neurological Hospital and Institute, Montreal, Quebec, Canada 16:05-16:25 A Peculiar Form of Acute Encephalitis/Encephalopathy Presenting 50 with Catastrophic Status Epilepticus - A New Clinical Entity ?
Yutaka AWAYA, Yukio FUKUYAMA, Kitami HAYASHI, Makiko OSAWA Department of Pediatrics, Seibo International Catholic Hospital, Tokyo, Japan Child Neurology Institute, Tokyo, Japan Department of Pediatrics, Tokyo Women’s Medical University, Tokyo, Japan 16:25-16:45 Acute Infantile Encephalopathy Predominantly Affecting the 51 Frontal Lobes: A Novel Subtype of Acute Encephalopathy
Hideo YAMANOUCHI, Masahi MIZUGUCHI Department of Pediatrics, Dokkyo University School of Medicine, Tochigi, Japan Department of Pediatrics, Jichi University School of Medicine, Tochigi, Japan Department of Pediatrics, University of Tokyo, Tokyo, Japan (present affiliation) 16:45-16:55 GENERAL DISUCUSSION 52
16:55 CLOSURE 16:55-17:00 Closing Remarks
Makiko OSAWA Department of Pediatrics, Tokyo Women’s Medical University, Tokyo, Japan 18:00-21:00 SATELLITE BUSINESS MEETING The 2nd Business Meeting of Pediatric Epilepsy Study Group in Asia Venue: Arcadia Ichigaya, Akagi Room (5th Floor) Participation: Semi-closed