Association of lamotrigine and valproate in refractory epilepsies of children and adolescents.

OBJECTIVE: To evaluate the efficacy or eventual side-effects of the association of lamotrigine and sodium valproate in the control of refractory epilepsies. METHOD: A retrospective analysis of 37 children with a mean age of 12 years taking exclusively lamotrigine and sodium valproate. Efficacy of seizure control was considered satisfactory if there was a reduction in seizures>50% or total control. RESULTS: The association of lamotrigine and sodium valproate was considered satisfactory in 65% of the studied children, independent of seizure type. Total seizure control was obtained in 33% and 35% had an unsatisfactory response or remained unchanged. Primary generalized tonic clonic seizures were the most common type with 84% of day-time seizures having a good response to treatment. Side-effects were seen in 11% of patients and the most common was tremor. CONCLUSION: Total or satisfactory control of seizures was seen in the majority of patients and side-effects were uncommon.

Association of lamotrigine and valproate in refractory epilepsies of children and adolescents / Associação de lamotrigina e valproato de sódio no tratamento de epilepsia refratária em crianças e adolescentes

OBJECTIVE: To evaluate the efficacy or eventual side-effects of the association of lamotrigine and sodium valproate in the control of refractory epilepsies. METHOD: A retrospective analysis of 37 children with a mean age of 12 years taking exclusivelly lamotrigine and sodium valproate. Efficacy of seizure control was considered satisfactory if there was a reduction in seizures >50 percent or total control. RESULTS: The association of lamotrigine and sodium valproate was considered satisfactory in 65 percent of the studied children, independent of seizure type. Total seizure control was obtained in 33 percent and 35 percent had an unsatisfactory response or remained unchanged. Primary generalized tonic clonic seizures were the most common type with 84 percent of day-time seizures having a good response to treatment. Side-effects were seen in 11 percent of patients and the most common was tremor. CONCLUSION: Total or satisfactory control of seizures was seen in the majority of patients and side-effects were uncommon.

OBJETIVO: Avaliar a eficácia ou eventuais efeitos colaterais da associação de lamotrigina e valproato de sódio no controle de epilepsia refrataria. MÉTODO: Análise retrospectiva de 37 crianças e adolescentes com idade média de 12 anos tratadas exclusivamente com lamotrigina e valproato de sódio. A eficácia do controle de crises foi considerada satisfatória se o controle das crises foi >50 por cento ou total. RESULTADOS: A associação de lamotrigina e valproato de sódio foi considerada satisfatória em 65 por cento, independente do tipo de crise. O controle total de crises foi obtido em 33 por cento e em 35 por cento a resposta foi insatisfatória ou permaneceu inalterada. Crise generalizada primaria tônico clonica foi o mais comum, com 84 por cento das crises ocorrendo durante o dia, com boa resposta ao tratamento. Efeitos colaterais foram vistos em 11 por cento dos pacientes, sendo tremor o mais freqüente. CONCLUSÃO: Controle total ou satisfatório das crises ocorreu na maioria dos pacientes, sendo pouco freqüente os efeitos colaterais.

Unusual magnetic resonance findings in two children with sudden sensorineural hearing loss

OBJETIVO: Descrever os achados de ressonância magnética (RM) em dois pacientes pediátricos com perda auditiva neuro-sensorial súbita (PANS). RELATO DOS CASOS: Dois pacientes masculinos (dois anos e três meses e um ano e quarto meses de idade) apresentaram-se com surdez súbita. Os exames físico e neurológico foram normais, à exceção da hipoacusia. Toda a investigação laboratorial foi normal, e os potenciais auditivos evocados do tronco cerebral demonstraram surdez profunda bilateral. Os exames de RM evidenciaram estruturas normais dos ouvidos internos e áreas multifocais de discreto hipossinal em T1 e hipersinal em FLAIR na substância branca dos hemisférios cerebrais. As RMs e os exames neurológicos de controle não demonstraram alterações nos achados prévios. CONCLUSÃO: Pacientes pediátricos com PANS podem apresentar à RM alterações de sinal na substância branca dos hemisférios cerebrais como único achado. Estudos futuros com casuísticas maiores deverão ser conduzidos para a elucidação destes achados.

Unusual magnetic resonance findings in two children with sudden sensorineural hearing loss.

OBJECTIVE: To describe the MRI findings of two pediatric patients with sudden sensorineural hearing loss (SSHL). CASE REPORTS: Two male patients (two-year and three-months-old, and one year and four-months-old) presented with sudden dumbness. Physical and neurological examinations were unremarkable besides bilateral hypoacusia. All the laboratory investigation was negative, and brain stem auditory evoked potentials showed deep bilateral deafness in both cases. MRI studies revealed normal inner ears and multifocal white matter areas of slight low signal on T1-weighted images and high signal on FLAIR images. The follow-up MRI studies and neurological examinations did not demonstrate alterations in the previous findings. CONCLUSION: Pediatric patients with SSHL may present cerebral white matter signal abnormalities at the MRI as the only finding. Further studies with larger casuistics need to be conducted to elucidate these findings.

Myelomeningocele: a Brazilian University Hospital experience.

We analyzed 31 children with myelomeningocele born between July 1990 and July 2000. Follow-up median was 24 months (6-68 months). Only 2 mothers had a known etiologic factor (diabetes mellitus). Twelve had the correct prenatal diagnosis. All children were born at term; 23 by cesarean; 13 had rupture of the membrane. Surgical correction had a 4 days median (1 to 44 days). Lumbosacral lesions were the most frequent (46%). Thirty patients were hydrocephalic, shunt was placed in 27. Meningitis was 4 times more frequent in shunted patients. Seven became epileptic (19.4%). Denver II test showed significant delay in gross motor development. Neurogenic bladder was diagnosed in 12 patients. Congenital clubfoot was the main orthopedic malformation (53%). Six infants died. Nowadays, 17 patients are being followed. A multidisciplinary approach probably helps for a better quality of life.

Myelomeningocele: a Brazilian University Hospital experience

Avaliamos 31 crianças com mielomeningocele nascidas entre julho de 1990 e julho de 2000. A mediana de acompanhamento foi 24 meses (6-68m). Duas mães possuíam conhecido fator de risco (diabetes mellitus). Doze obtiveram correto diagnóstico pré-natal da patologia. Todas as crianças nasceram a termo; 23 via cesariana; 13 apresentaram ruptura de membrana. A mediana de correção cirúrgica foi 4 dias (1-44d). Lesões lombosacras foram as mais freqüentes (46%). Trinta pacientes apresentaram hidrocefalia, sendo a derivação ventrículo peritoneal (DVP) necessária em 27. Meningite foi 4 vezes mais frequente em pacientes com DVP. Sete pacientes eram epilépticos (19,4%). O teste de Denver II mostrou atraso motor significante. Bexiga neurogênica foi diagnosticada em 12 pacientes. Pé torto congênito foi a malformação ortopédica mais comum (53%). Seis pacientes morreram. Até o momento, 17 pacientes são acompanhados. Uma abordagem multidisciplinar provavelmente colabora para melhor qualidade de vida.