Myoclonic Seizures In Symptomatic Temporal Lobe Epilepsy Of Childhood

Rationale and objective: Generalized epileptic manifestations may occur in symptomatic focal epilepsies in children, particularly in infants and pre-school children. Our aim here is to report two patients with myoclonic seizures associated with temporal lobe epilepsy. Methodology: Case report. Results: Two children with tumor in temporal lobe presented myoclonic seizures or spasms since infancy besides other types of seizures. Conclusion: Our findings support the idea that myoclonic seizures may be generalized manifestations of focal insults to the CNS, and this pattern may be an age-dependent specific response of a developing brain to a severe insult.1114547Faught, E., Clinical presentations and phenomenology of myoclonus (2003) Epilepsia, 44 (SUPPL. 11), pp. 7-12Hallet, M., Myoclonus and myoclonic syndromes (1997) Epilepsy: A comprensive textbook, pp. 2717-2723. , Engel J, Pedley, TA, editores, Philadelphia: Lippincott- Raven;Nordli, D.R., Kurada, M.M., Hirsch, L.J., The ontogeny of partial seizures in infants and young children (2001) Epilepsia, 42 (8), pp. 986-990Fogarasi, A., Jokeit, H., Feveret, E., Jansky, J., Tuxhorn, J., The effect of age on seizure semiology in childhood temporal lobe epilepsy (2002) Epilepsia, 43 (6), pp. 638-643Franzon, R.C., Montenegro, M.A., Guimarães, C.A., Guerreiro, C.A.M., Cendes, F., Guerreiro, M.M., Clinical, electroencephalographic and behavioral features of temporal lobe epilepsy in chidhood (2004) J Child Neurol, 19 (6), pp. 418-423Mimaki, T., Ono, J., Yabuuchi, H., Temporal lobe astrocytoma with infantile spasms (1983) Ann Neurol, 14, pp. 695-696Singer, W., Haller, J., Mills, C., Rabe, E., The value of neuroradiology in infantile spasms (1982) Pediatrics, 100, pp. 47-50Palm, D.G., Brandt, M., Korinthenberg, R., West syndrome and Lennox-Gastaut syndrome in children with porencephalic cysts: Long-term follow-up after neurosurgical treatment (1988) The Lennox-Gastaut syndrome, pp. 419-426. , Niedermeyer E, Degn R, editores, New York: Alan R. Liss;Chugani, H.T., Shields, D., Shewmon, D.A., Olson, D.M., Phelps, M.E., Peacock, W.J., Infantile Spasms: I. PET Identifies Focal Cortical Dysgenesis in Cryptogenic Cases for Surgical Treatment (1990) Ann Neurol, 27, pp. 406-413Chugani, H.T., Infantile Spasms (1995) Neurology, 8, pp. 139-144Miyazaki, M., Hashimoto, T., Fujii, E., Tayama, M., Kuroda, Y., Infantile spasms: Localized cerebral lesions on SPECT (1994) Epilepsia, 35, pp. 988-992Chugani, H.T., Silva, E., Chugani, D.C., Infantile Spasms: III. Prognostic Implications of Bitemporal Hypometabolism on Positron Emission Tomography (1996) Ann Neurol, 39, pp. 643-649Dalla Bernardina, B., Colamaria, V., Capoville, G., Epileptic syndromes and cerebral malformations in infancy: Multicentric study (1984) Boll Lega Ital Epil, 45-46, pp. 65-67Carrazana, E.J., Lombroso, C.T., Mikati, M., Helmers, S., Holmes, G.L., Facilitation of Infantile Spasms by partial seizures (1993) Epilepsia, 34, pp. 686-691Alvarez, L.A., Shinnar, S., Moshe, S.L., Infantile spasms due to unilateral cerebral infarcts (1987) Pediatrics, 79, pp. 1024-102

Efficacy And Tolerability Of Vigabatrin In West Syndrome [avaliaçao Da Eficácia E Tolerabilidade Da Vigabatrina Na Síndrome De West]

West syndrome (WS) is a severe epileptic encephalopathy of childhood, characterized by spasms, developmental deterioration and hipsarhythymia. Objective: To evaluate the safety and efficacy of vigabatrin (VGB) in the treatment of WS. Method: We evaluated every patient diagnosed with WS seen at the pediatric epilepsy clinic and exposed to VGB. Patients were interviewed according to a semistructured questionnaire and we analyzed gender, age, etiology (cryptogenic or symptomatic), associated diseases, age of seizure onset, neuroimaging findings, EEG prior and after VGB, use of other antiepileptic drugs, time for seizure control, electroretinogram, visual complaints, adverse events and family history of epilepsy. Results: Twenty-three patients were evaluated, 16 boys, ages ranging from 1.25 years to 11.5 years (mean=5y3m). Sixteen (69.5%) patients were seizure free, five (22%) had partial seizure control and in two (8.5%) there was no improvement. Only one patient presented gabaergic retinopathy. Six (26%) patients presented adverse events: somnolence, aggressivity or retinopathy. Patients with seizure onset after 6 months of age presented better results after VGB introduction (p0.05). After VGB, no patient presented hipsarrhythymia and 50% had a normal EEG. Conclusion: Although VGB may be associated with serious adverse events such as gabaergic retinopathy, our results show that it should be considered in the treatment of WS.632 B469473Appleton, R.E., West syndrome: Long-term prognosis and social aspects (2001) Brain Dev, 23, pp. 688-691Mitchell, W.G., Shah, N.S., Vigabatrin for infantile spasms (2002) Pediatr Neurol, 27, pp. 161-164Wallace, S.J., Newer antiepileptic drugs: Advantages and disadvantages (2001) Brain & Dev, 23, pp. 277-283Harding, G.F., Wild, J.M., Robertson, K.A., Rietbrock, S., Martinez, C., Separating the retinal electrophysiologic effects of vigabatrin: Treatment versus field loss (2000) Neurology, 55, pp. 347-352McDonagh, J., Stephen, L.J., Dolan, F.M., Peripheral retinal dysfunction in patients taking vigabatrin (2003) Neurology, 61, pp. 1690-1694Chiron, C., Dulac, O., Luna, D., Vigabatrin in infantile spasms (1990) Lancet, 335, pp. 363-364Chiron, C., Dulac, O., Beaumont, D., Palacios, L., Pajot, N., Mumford, J., Therapeutic trial of vigabatrin in refractory infantile spasms (1991) J Child Neurol, (SUPPL. 2), pp. S52-S59Rotta, N.T., Silva, A.R., Ohlweiler, L., Riesgo, R., Vigabatrin in the treatment of epilepsy in patients with West syndrome and tuberous sclerosis (2003) Arq Neuropsiquiatr, 61, pp. 988-990Curatolo, P., Seri, S., Verdecchia, M., Bombardieri, R., Infantile spasms in tuberous sclerosis complex (2001) Brain Dev, 23, pp. 502-507Elterman, R.D., Shields, W.D., Mansdield, K.A., Nakagawa, J., Randomized trial of vigabatrin in patients with infantile spasms (2001) Neurology, 57, pp. 1416-1421Riikonen, R.S., Steroids or vigabatrin in the treatment of infantile spasms? (2000) Pediatr Neurol, 23, pp. 403-408Antoniuk, S.A., Bruck, I., Spessatto, A., West syndrome: Clinical and electroencephalographic follow up of 70 patients and response to its treatment with adrenocorticotropic hormone, prednisone, vigabatrin, nitrazepam and valproate (2000) Arq Neuropsiquiatr, 58, pp. 683-690Fejerman, N., Cersosimo, R., Caraballo, R., Vigabatrin as a first-choice drug in the treatment of West syndrome (2000) J Child Neurol, 15, pp. 161-165Appleton, R.E., Peters, A.C., Mumford, J.P., Shaw, D.E., Randomised, placebo-controlled study of vigabatrin as first-line treatment of infantile spasms (1999) Epilepsia, 40, pp. 1627-1633Granstrom, M.I., Gaily, E., Liukkonen, E., Treatment of infantile spasms of a population-based study with vigabatrin as the first drug for spasms (1999) Epilepsia, 40, pp. 950-957Cossette, P., Riviello, J.J., Carmant, L., ACTH versus vigabatrin therapy in infantile spasms: A retrospective study (1999) Neurology, 52, pp. 1691-1694Villeneuve, N., Soufflet, C., Plouin, P., Chiron, C., Dulac, O., Treatment of infantile spasms with vigabatrin as first-line therapy and in monoteraphy: Apropos of 70 infants (1998) Arch Pediatr, 5, pp. 731-738Wohlrab, G., Boltshauser, E., Schmitt, B., Vigabatrin as a first-line drug in West syndrome: Clinical and electroencephalographic outcome (1998) Neuropediatrics, 29, pp. 133-136Vigevano, F., Cilio, M.R., Vigabatrin versus ACTH as first-line treatment for infantile spasms: A randomized, prospective study (1997) Epilepsia, 38, pp. 1270-1274Arndt, C.F., Derambure, P., Defoort-Dhellemmes, S., Hache, J.C., Outer retinal dysfunction in patients with vigabatrin (1990) Neurology, 52, pp. 1201-1205Krauss, G.L., Johnson, M.A., Miller, N.R., Vigabatrin-associated retinal cone system dysfunction: Electroretinogram and ophthalmologic findings (1998) Neurology, 50, pp. 614-618Banin, E., Shalev, R.S., Obolensky, A., Neis, R., Chowers, I., Gross-Tsur, V., Retinal function abnormalities in patients treated with vigabatrin (2003) Arch Ophthalmol, 121, pp. 811-816Harding, G.F., Wild, J.M., Robertson, K.A., Electro-oculography, electroretinography, visual evoked potentials, and multifocal electroretinography in patients with vigabatrin-attributed visual field constriction (2000) Epilepsia, 41, pp. 1420-1431Malmgren, K., Ben-Menachem, E., Frisen, L., Vigabatrin visual toxicity: Evolution and dose dependence (2001) Epilepsia, 42, pp. 609-615Hardus, P., Verduin, W.M., Engelsman, M., Visual field loss associated with vigabatrin: Quantification and relation to dosage (2001) Epilepsia, 42, pp. 262-267Jensen, H., Sjo, O., Uldall, P., Vigabatrin and retinal changes (2002) Doc Ophthalmol, 104, pp. 171-180Schmitz, B., Schmidt, T., Jokiel, B., Pfeiffer, S., Tiel-Wilck, K., Ruther, K., Visual field constriction in epilepsy patients treated with vigabatrin and other antiepileptic drugs: A prospective study (2002) J Neurol, 249, pp. 469-475Capovilla, G., Beccaria, F., Montagnini, A., Short-term nonhormonal and nonsteroid treatment in West syndrome (2003) Epilepsia, 44, pp. 1085-1088Koul, R., Chacko, A., Ganesh, A., Bulusu, S., Al Riyami, K., Vigabatrin associated retinal dysfunction in children with epilepsy (2001) Arch Dis Child, 85, pp. 469-473Schmidt, T., Ruther, K., Jokiel, B., Pfeiffer, S., Tiel-Wilck, Schmitz, B., Is visual field constriction in patients treated with vigabatrin reversible? (2002) J Neurol, 249, pp. 1066-1071Giordano, L., Valseriati, D., Vignoli, A., Morescalchi, F., Gandolfo, E., Another case of reversibility of visual-field defect induced by vigabatrin monotherapy: Is young age a favorable factor? (2000) Neurol Sci, 21, pp. 185-186Versino, M., Veggiotti, P., Reversibility of vigabatrin-induced visual-field defect (1999) The Lancet, 354, p. 486Vanhatalo, S., Alen, R., Riikonen, R., Reversed visual field constrictions in children after vigabatrin withdrawal - True retinal recovery or improved test performance only? (2001) Seizure, 10, pp. 508-51