Regular Article| Volume 9, ISSUE 8, P590-594, December 2000

Topiramate in the treatment of severe myoclonic epilepsy in infancy

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      The aim of this study was to assess the effectiveness of topiramate (TPM) as an add-on regimen in reducing seizure rate in a population sample of patients diagnosed with severe myoclonic epilepsy in infancy (SME). Eighteen patients were evaluated. The mean observation time was 10.5 months (range, 6–18 months). Seizure frequency and type were recorded. Topiramate was administered as an add-on regimen at a starting dose of 1 mg kg−1and titrated to a maximum of 6–8 mg per kg per day. Different escalation rates were used, mainly weekly or fortnightly increments of dose. Three patients (16.6%) became seizure free, and 10 (55.6%) had a >50% reduction in seizure frequency: six of them (22.2%) achieved a reduction greater than 75%. Side-effects were observed in nine patients , eight with a weekly titration schedule and one with a fortnightly schedule. TPM is effective as adjunctive therapy for SME. Side-effects were mild and transient, generally related to rapid dosage titration.




        • Dravet C
        • Roger J
        Bureau M, Dalla Bernardina B. Myoclonic epilepsies in childhood.
        in: Akimoto H Kazamatsu M Seino M Ward A Advances in Epileptology: The XIIIth Epilepsy International Symposium. Raven Press, New York1982: 135-140
        • Commission on Classification and Terminology of the International League Against Epilepsy
        Proposal for revised classification of epilepsies and epileptic syndromes.
        Epilepsia. 1989; 30: 389-399
        • Nieto M
        • Candau R
        • Rufo M
        • Ruiz del Portal L
        Epilepsia mioclonica severa de la infancia. Tratamiento con gammaglobulina humana.
        Revista de Neurologia. 1995; 23: 1265
        • Rogawski M.A
        • Porter R.J
        Antiepileptic drugs: Pharmacological mechanisms and clinical efficacy with consideration of promising developmental stage compounds.
        Pharmacological Review. 1990; 42: 223-286
        • Coulter D.A
        • Sombati S
        • De Lorenzo R.J
        Selective effects of topiramate on sustained repetitive firing and spontaneous bursting in cultured hippocampal neurons.
        Epilepsia. 1993; 34 (Suppl. 2): 123
        • Brown S.D
        • Wolf H.H
        • Swinyard E.A
        • Twyman R.E
        • White H.S
        The novel anticonvulsivant topiramate enhances GABA-mediated chloride flux.
        Epilepsia. 1993; 34 (Suppl. 2): 122-123
        • Marson A.G
        • Kadir Z.A
        • Chadwick D.W
        New antiepileptic drugs: a systematic review of their efficacy and tolerability.
        British Medical Journal. 1996; 313: 1169-1174
        • Faught E
        • Wilder B.J
        • Ramsay R.E
        • et al.
        Topiramate placebo-controlled dose-ranging trial in refractory partial epilepsy using 200, 400, and 600 mg daily dosages.
        Neurology. 1996; 46: 1684-1690
        • Sharief M
        • Viteri C
        • Ben-Menachem E
        • et al.
        Double-blind, placebo-controlled study of topiramate in patients with refractory partial epilepsy.
        Epilepsy Research. 1996; 25: 217-224
        • Glauser T.A
        Seminars in Pediatric Neurology. 1997; 4: 34-42
        • Sachdeo R.C
        • Glauser T.A
        • Ritter F
        • et al.
        A double-blind, randomized trial of topiramate in Lennox–Gastaut syndrome.
        Neurology. 1999; 52: 1882-1887
        • Herranz J.L
        • Arteaga R
        • De las Cuevas I
        Topiramate in the treatment of children with Lennox–Gastaut syndrome.
        Epilepsia. 1997; 38 (Suppl. 3): S37
        • Glauser T.A
        • Clark P.O
        • Strawsburg R
        A pilot study of topiramate in the treatment of infantile spasms.
        Epilepsia. 1998; 39: 1324-1328
        • Dravet C
        • Bureau M
        • Guerrini R
        • Giraud N
        • Roger J
        Severe myoclonic epilepsy in infancy.
        in: Roger J Bureau M Dravet C Dreiffus F Peret A Wolf P Epileptic Syndromes in Infancy, Childhood, and Adolescence. John Libbey Eurotext, London1992: 103-114
        • Guerrini H
        • Dravet C
        • Genton C
        • et al.
        Lamotrigine and seizure aggravation in severe myoclonic epilepsy.
        Epilepsia. 1998; 39: 508-512
        • Lambarri I
        • Garaizar C
        • Zuazo E
        • Prats J.M
        Epilepsia polimorfa de la infancia. Revision de 12 casos.
        Anales Españoles de Pediatria. 1997; 46: 571-575
        • Burkart P
        • Schneble H
        • Ziegler G
        Topiramate in severe myoclonic convulsive epilepsy of infancy.
        Epilepsia. 1999; 40 (Suppl. 2): 179