title: Management of and prophylaxis against status epilepticus in children with severe myoclonic epilepsy in infancy (SMEI; Dravet syndrome)--a nationwide questionnaire survey in Japan.
abstract: The aim of this study was to establish strategies for prophylaxis against status epilepticus (SE) associated with high fever and for management of ongoing SE in children with severe myoclonic epilepsy in infancy (SMEI).
  
  
  METHODS
  The investigation was performed retrospectively using a questionnaire, asking about medications, which was distributed to epilepsy specialists throughout Japan. All respondents were members of the Japan Epilepsy Society (JES) and/or the Japanese Society of Child Neurology (JSCN). Data from 109 SMEI patients (51 males and 58 females), 1-37 (M+/-SD, 10.7+/-6.53) years old, were used for this study. Of these 109 patients, 10 were excluded because they had not experienced SE, such that data from 99 patients were analyzed.
  
  
  RESULTS
  Among the anti-epileptic drugs (AEDs) used daily, excellent efficacy against SE evolution was obtained with the following: potassium bromide (KBr) (41.7%), zonisamide (ZNS) (13.5%), clobazam (CLB) (10.0%), valproate (VPA) (8.0%), phenobarbital (PB) (6.7%), and phenytoin (PHT) (2.6%). Excellent efficacy was not obtained with either clonazepam (CZP) or carbamazepine (CBZ). The diazepam (DZP) suppository was the most frequently given drug for prophylaxis against SE triggered by fever, but only 2 (2.4%) cases showed excellent results. Excellent efficacy in terminating ongoing SE was obtained with the following medications; intravenous barbiturates (75-100%), intravenous midazolam (MDZ) (68.8%), intravenous DZP (54.3%), intravenous lidocaine (Lid) (21.4%), and intravenous PHT (15.4%).
  
  
  CONCLUSIONS
  Daily KBr was most efficacious for controlling seizures in SMEI patients. Early use of intravenous barbiturates is the most effective strategy in stopping SE in a subset of patients. Reliable efficacy in SE was not obtained with prophylactic DZP, intravenous benzodiazepines (BZPs), PHT and Lid.
authors.affiliation: Division of Pediatrics, Hirakata City Hospital, 2-14-1 Kinyahonmachi, Hirakata City, Osaka 573-1013, Japan. tanabemapa@pop01.odn.ne.jp
authors.forename: Takuya
authors.initials: T
authors.lastname: Tanabe
authors.forename: Yutaka
authors.initials: Y
authors.lastname: Awaya
authors.forename: Toyojiro
authors.initials: T
authors.lastname: Matsuishi
authors.forename: Kuniaki
authors.initials: K
authors.lastname: Iyoda
authors.forename: Toshisaburo
authors.initials: T
authors.lastname: Nagai
authors.forename: Mana
authors.initials: M
authors.lastname: Kurihara
authors.forename: Kazuya
authors.initials: K
authors.lastname: Yamamoto
authors.forename: Kimio
authors.initials: K
authors.lastname: Minagawa
authors.forename: Kihei
authors.initials: K
authors.lastname: Maekawa
mesh_terms: D000293:Adolescent; D000927:Anticonvulsants / Q000627:therapeutic use*; D002648:Child; D002675:Child, Preschool; D003625:Data Collection; D004831:Epilepsies, Myoclonic / Q000150:complications / Q000188:drug therapy*; D005260:Female; D005334:Fever / Q000150:complications; D006801:Humans; D007223:Infant; D007564:Japan; D008297:Male; D012189:Retrospective Studies; D013226:Status Epilepticus / Q000188:drug therapy* / Q000209:etiology / Q000517:prevention & control*; D011795:Surveys and Questionnaires
chemical_list: D000927:Anticonvulsants