Management of and prophylaxis against status epilepticus in children with severe myoclonic epilepsy in infancy (SMEI; Dravet syndrome)--a nationwide questionnaire survey in Japan.
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.
Tanabe
Takuya
T
Division of Pediatrics, Hirakata City Hospital, 2-14-1 Kinyahonmachi, Hirakata City, Osaka 573-1013, Japan. tanabemapa@pop01.odn.ne.jp
Awaya
Yutaka
Y
Matsuishi
Toyojiro
T
Iyoda
Kuniaki
K
Nagai
Toshisaburo
T
Kurihara
Mana
M
Yamamoto
Kazuya
K
Minagawa
Kimio
K
Maekawa
Kihei
K
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
D000927:Anticonvulsants