Stiripentol open study in Japanese patients with Dravet syndrome

Epilepsia

Volumn 50, Issue 11, 2009, Pages 2362-2368

  Inoue, Yushi ^a   Ohtsuka, Yoko ^b   Oguni, Hirokazu ^c   Tohyama, Jun ^d   Baba, Hiroshi ^e   Fukushima, Katsuyuki ^f   Ohtani, Hideyuki ^a   Takahashi, Yukitoshi ^a   Ikeda, Shunya ^g  

^a SHIZUOKA INSTITUTE OF EPILEPSY AND NEUROLOGICAL DISORDERS   (Japan)

^b OKAYAMA UNIVERSITY   (Japan)

^c TOKYO WOMEN'S MEDICAL UNIVERSITY   (Japan)

^d NISHI NIIGATA CHUO NATIONAL HOSPITAL   (Japan)

^e NATIONAL HOSPITAL ORGANIZATION NAGASAKI MEDICAL CENTER   (Japan)

^f National Hospital Organization   (Japan)

^g INTERNATIONAL UNIVERSITY OF HEALTH AND WELFARE   (Japan)

Author keywords

Clobazam; Cytochrome P450; Dravet syndrome; Severe myoclonic epilepsy in infancy; Stiripentol

Indexed keywords

ACETAZOLAMIDE; BROMIDE; CARBAMAZEPINE; CLOBAZAM; CLONAZEPAM; CLORAZEPATE; CYTOCHROME P450 2C19; DIAZEPAM; ETHOSUXIMIDE; NITRAZEPAM; PHENOBARBITAL; PHENYTOIN; PRIMIDONE; STIRIPENTOL; VALPROIC ACID; ZONISAMIDE;

ADD ON THERAPY; ADOLESCENT; ADULT; ANOREXIA; ARTICLE; ATAXIA; CHILD; CLINICAL ARTICLE; CLINICAL TRIAL; CLONIC SEIZURE; CONTROLLED STUDY; DISEASE DURATION; DOSE RESPONSE; DRAVET SYNDROME; DROWSINESS; DRUG EFFICACY; DRUG WITHDRAWAL; EPILEPTIC STATE; FEMALE; GENE MUTATION; GENERALIZED EPILEPSY; HUMAN; HYPERACTIVITY; INSOMNIA; IRRITABILITY; JAPANESE; LONG TERM CARE; MALE; MULTICENTER STUDY; NAUSEA; PRIORITY JOURNAL; SEIZURE; SIDE EFFECT; SLEEP DISORDER; TONIC CLONIC SEIZURE;

ADOLESCENT; ADULT; ANTICONVULSANTS; ARYL HYDROCARBON HYDROXYLASES; BENZODIAZEPINES; CHILD; CHILD, PRESCHOOL; DIOXOLANES; DRUG ADMINISTRATION SCHEDULE; DRUG THERAPY, COMBINATION; EPILEPSIES, MYOCLONIC; EPILEPSY, TONIC-CLONIC; FEMALE; HUMANS; INFANT; MALE; MEDICAL RECORDS; MIDDLE AGED; RETROSPECTIVE STUDIES; SYNDROME; TREATMENT OUTCOME;

EID: 70350401140     PISSN: 00139580     EISSN: 15281167     Source Type: Journal    
DOI: 10.1111/j.1528-1167.2009.02179.x     Document Type: Article

References (22)

1. Ceulemans B, Boel M, Claes L, Dom L, Willekens H, Thiry P, Lagae L 2004) Severe myoclonic epilepsy in infancy: toward an optimal treatment. J Child Neurol 19 : 516 521.
2. Chiron C, Marchand MC, Tran A, Rey E, d'Athis P, Vincent J, Dulac O, Pons G 2000) Stiripentol in severe myoclonic epilepsy in infancy: a randomized placebo-controlled syndrome-dedicated trial. STICLO study group. Lancet 356 : 1638 1642.
3. Chiron C 2005) Stiripentol. Expert Opin Investig Drugs 14 : 905 911.
4. Chiron C 2007) Stiripentol. Neurotherapeutics 4 : 123 125.
5. Dravet C, Bureau M, Oguni H, Fukuyama Y, Cokar O 2005) Severe Myoclonic Epilepsy in Infancy (Dravet Syndrome). In Roger J, Bureau M, Dravet C, Genton P, Tassinari CA, Wolf P (Eds) Epileptic syndromes in infancy, childhood and adolescence. John Libbey, London, pp. 77 88.
6. Farwell JR, Anderson GD, Kerr BM, Tor JA, Levy RH 1993) Stiripentol in atypical absence seizures in children: an open trial. Epilepsia 34 : 305 311.
7. Fisher JL 2009) The anti-convulsant stiripentol acts directly on the GABA(A) receptor as a positive allosteric modulator. Neuropharmacology 56 : 190 197.
8. Fujiwara T, Sugawara T, Mazaki-Miyazaki E, Takahashi Y, Fukushima K, Watanabe M, Hara K, Morikawa T, Yagi K, Yamakawa K, Inoue Y 2003) Mutations of sodium channel alpha subunit type 1 (SCN1A) in intractable childhood epilepsies with frequent generalized tonic-clonic seizures. Brain 126 : 531 546.
9. Giraud C, Tran A, Rey E, Vincent J, Tréluyer JM, Pons G 2004) In vitro characterization of clobazam metabolism by recombinant cytochrome P450 enzymes: importance of CYP2C19. Drug Metab Dispos 32 : 1279 1286.
10. Giraud C, Treluyer JM, Rey E, Chiron C, Vincent J, Pons G, Tran A 2006) In vitro and in vivo inhibitory effect of stiripentol on clobazam metabolism. Drug Metab Dispos 34 : 608 611.
11. Hurst DL 1990) Epidemiology of severe myoclonic epilepsy in infancy. Epilepsia 31 : 397 400.
12. Inoue Y, Nishida T 2004) Expert consensus on treatment of epilepsy. J Jpn Epilepsy Soc 22 : 128 139.
13. Kosaki K, Tamura K, Sato R, Samejima H, Tanigawara Y, Takahashi T 2004) A major influence of CYP2C19 genotype on the steady-state concentration of N-desmethylclobazam. Brain Dev 26 : 530 534.
14. Kröll-Seger J, Portilla P, Dulac O, Chiron C 2006) Topiramate in the treatment of highly refractory patients with Dravet syndrome. Neuropediatrics 37 : 325 329.
15. Levy RH, Rettenmeier AW, Anderson GD, Wilensky AJ, Friel PN, Baillie TA, Acheampong A, Tor J, Guyot M, Loiseau P 1990) Effects of polytherapy with phenytoin, carbamazepine, and stiripentol on formation of 4-ene-valproate, a hepatotoxic metabolite of valproic acid. Clin Pharmacol Ther 48 : 225 235.
16. Oguni H, Hayashi K, Oguni M, Mukahira A, Uehara T, Fukuyama Y, Umezu R, Izumi T, Hara M 1994) Treatment of severe myoclonic epilepsy in infants with bromide and its borderline variant. Epilepsia 35 : 1140 1145.
17. Oka E, Ohtsuka Y, Yoshinaga H, Murakami N, Kobayashi K, Ogino T 2006) Prevalence of childhood epilepsy and distribution of epileptic syndrome: a population-based survey in Okayama, Japan. Epilepsia 47 : 626 630.
18. Ozawa S, Soyama A, Saeki M, Fukushima-Uesaka H, Itoda M, Koyano S, Sai K, Ohno Y, Saito Y, Sawada J 2004) Ethnic differences in genetic polymorphisms of CYP2D6, CYP2C19, CYP3As and MDR1/ABCB1. Drug Metab Pharmacokinet 19 : 83 95.
19. Quilichini PP, Chiron C, Ben-Ari Y, Gozlan H 2006) Stiripentol, a putative antiepileptic drug, enhances the duration of opening of GABA-A receptor channels. Epilepsia 47 : 704 716.
20. Tanabe T, Awaya Y, Matsuishi T, Iyoda K, Nagai T, Kurihara M, Yamamoto K, Minagawa K, Maekawa K 2008) Management of and prophylaxis against status epilepticus in children with severe myoclonic epilepsy in infancy (SMEI; Dravet syndrome): a nationwide questionnaire survey in Japan. Brain Dev 30 : 629 635.
21. Thanh TN, Chiron C, Dellatolas G, Rey E, Pons G, Vincent J, Dulac O 2002) Long-term efficacy and tolerance of stiripentol in severe myoclonic epilepsy of infancy (Dravet's syndrome). Arch Pediatr 9 : 1120 1127.
22. Trojnar MK, Wojtal K, Trojnar MP, Czuczwar SJ 2005) Stiripentol: a novel antiepileptic drug. Pharmacol Rep 57 : 154 160.