Encefalopatia toxica secundaria a vigabatrina: Reporte de caso / Toxic encephalopathy secondary to vigabatrin: Case report

El síndrome de West es una encefalopatía epiléptica caracterizada por espasmos en flexión, hipsarritmia en el electroencefalograma y retraso en el neurodesarrollo. Reportamos el caso de una paciente de 11 meses con diagnóstico de Síndrome de West y encefalopatía tóxica secundaria al uso de vigabatrina

West syndrome is an epileptic encephalopathy characterized by flexing spasms, hypsarritmia in the electroencephalogram and delayed neurodevelopment. We report an 11-month-old patient with a diagnosis of West syndrome and toxic encephalopathy secondary to the use of vigabatrin

Vigabatrina comparado con hormona adrenocorticotrópica para el tratamiento de espasmos infantiles y Síndrome de West / Vigabatrin compared with adrenocorticotropic hormone for the treatment of infantile spasms and West Syndrome

OBJETIVO: Determinar los riesgos y beneficios del uso de vigabatrina comparada con hormona adrenocorticotrópica (ACTH) para el tratamiento de espasmos infantiles. MÉTODO: Se realizó una búsqueda en Epistemonikos. Se extrajeron datos desde las revisiones identificadas. Se realizó un metaanálisis a partir de estudios primarios y se utilizó el método GRADE para la presentación de resultados. RESULTADOS: Se identificaron nueve revisiones sistemáticas. Se observó que el uso de vigabatrina en comparación con ACTH disminuye la resolución de espasmos (RR 0,8, IC 95% 0,65 - 0,98) y podría disminuir la resolución de hipsarritmia (RR 0,71, IC 95% 0,48 - 1,05). No fue posible determinar si el uso de vigabatrina disminuye el riesgo de desarrollar efectos adversos (RR 0,75, IC 95% 0,23 - 2,45) por certeza de evidencia muy baja. CONCLUSIONES: La evidencia parece inclinarse a favor del uso de ACTH. Sin embargo debe considerarse la necesidad de nuevas investigaciones para esclarecer su seguridad.

OBJECTIVE: To determine the risks and benefits of the use of vigabatrin compared to ACTH for the treatment of infantile spasms. METHOD: A search in Epistemonikos was performed. Data were extracted from the identified reviews. A meta-analysis was performed from primary studies and the GRADE method was used to present the results. RESULTS: Nine systematic reviews were identified. Vigabatrin use compared to ACTH was found to decrease resolution of spasms (RR 0.8, 95% CI 0.65 - 0.98) and might decrease resolution of hypsarrhythmia (RR 0.71, 95% CI 0 .48 - 1.05). It was not possible to determine whether the use of vigabatrin reduces the risk of developing adverse effects (RR 0.75, 95% CI 0.23 - 2.45) due to very low certainty of evidence. CONCLUSIONS: The evidence seems to lean in favor of the use of ACTH. However, the need for new research should be considered to clarify its safety.

Síndrome transicional: del síndrome de West al síndrome de Lennox-Gastaut / Transional syndrome: from West to Lennox-Gastaut syndromes

El síndrome de West o espasmos infantiles, es una encefalopatía epiléptica clasificada como epilepsias y síndromes generalizados. Hay múltiples informes de la evolución de síndrome de West a síndrome de Lennox-Gastaut de un 25 hasta 60%, sin reconocerse una causa específica. Se ha comunicado que pueden ser solo una entidad epiléptica dependiente de la edad y que estaría en relación con el grado de inmadurez cerebral. En esta revisión retrospectiva de 130 casos de espasmos infantiles, solo 14 (10.7%) evolucionaron a Lennox-Gastaut. El haber recibido en todos los casos vigabatrina como tratamiento nos hace suponer que la baja incidencia podría estar relacionada con el uso de este fármaco. Dado que la vigabatrina tiene una acción gabaérgica y aumenta los niveles de ACTH podría explicar esta relación, pero esto deberá confirmarse con el mejor conocimiento de los mecanismos íntimos de estas graves encefalopatías.

West syndrome or infantile spasms is an epileptic encephalopathy, classified as generalized epilepsies and syndromes. There are multiple reports of the evolution from West to Lennox-Gastaut syndrome of 25 up to 60%, without a specific cause is determined. It has been reported that they may be only an epileptic entity age dependent that it would be in relation to the degree of brain immaturity. In this retrospective review of 130 cases of West syndrome, only 14 (10.7%) evolved to Lennox-Gastaut. Having received in all cases vigabatrin as a treatment, makes us suppose that the low incidence could be related to the use of this drug. Given that vigabatrin has a gabaergic action and increased levels of ACTH, may explain this relationship but this must be confirmed with the best knowledge of the intimate mechanisms of these serious epileptic encephalopathies.

Lacosamida como terapia aditiva em pacientes com epilepsia focal refratários aos tratamentos prévios com os fármacos antiepilépticos disponíveis no SUS / Lacosamide as additive therapy in patients with focal epilepsy refractory to previous treatments with antiepileptic drugs available in SUS

CONTEXTO: A epilepsia é uma doença cerebral crônica caracterizada pela recorrência de crises epilépticas não provocadas. De acordo com o Protocolo Clínico e Diretrizes Terapêuticas (PCDT) do Ministério da Saúde (MS) aproximadamente 30% dos pacientes, tratados adequadamente, continuam a ter crises, sem remissão. O tratamento disponível no SUS inclui os agentes antiepilépticos fenobarbital, fenitoína, primidona, topiramato, lamotrigina, carbamazepina e valproato de sódio. Recentemente foi avaliado e incorporado o levetiracetam. TECNOLOGIA: Lacosamida (Vimpat®). INDICAÇÃO: Terapia aditiva para o tratamento da epilepsia focal em pacientes refratários aos tratamentos prévios já disponíveis pelo Sistema Único de Saúde (SUS). PERGUNTAS: 1) A lacosamida oral como terapia adjuvante é tão segura e eficaz quanto a lamotrigina, topiramato, vigabatrina e gabapentina no tratamento da epilepsia focal em pacientes já submetidos a duas linhas de monoterapia? 2) Qual a efetividade da lacosamida como tratamento adjuvante na epilepsia focal não controlada, em pacientes adultos, em comparação ao uso de esquemas terapêuticos convencionais, quanto à redução da frequência de crises epilépticas, eventos adversos e custos? EVIDÊNCIAS CIENTÍFICAS: Não existem estudos de comparação direta entre a lacosamida e outro antiepiléptico. As evidências apresentaram comparações indiretas que apontam similaridade de eficácia e segurança entre a lacosamida e os medicamentos antiepilépticos disponíveis no SUS para o tratamento aditivo de pacientes com epilepsia focal, refratários a monoterapia. AVALIAÇÃO ECONÔMICA: Foi apresentada análise de custo-minimização para o tratamento aditivo da epilepsia focal refratária com lacosamida, porém o custo do tratamento com lacosamida, por paciente, só foi inferior ao custo do tratamento utilizando a vigabatrina. Os demais medicamentos já disponíveis para esta condição apresentam menor custo ao sistema. AVALIAÇÃO DE IMPACTO ORÇAMENTÁRIO: A incorporação da lacosamida proporcionaria economia ao sistema de saúde apenas se comparado ao tratamento com vigabatrina. EXPERIÊNCIA INTERNACIONAL: Em outros países a lacosamida é fornecida para o tratamento da epilepsia focal refratária, em similaridade de condições à carbamazepina, clobazam, gabapentina, lamotrigina, levetiracetam, oxcarbazepina, valproato de sódio ou topiramato, se ocorrer refratariedade ou intolerância ao tratamento em primeira linha. MONITORAMENTO DO HORIZONTE TECNOLÓGICO: Há medicamentos em fase de desenvolvimento clínico para o tratamento da epilepsia focal, com diferentes mecanismos de ação, em estágios avançados de pesquisa clínica. RECOMENDAÇÃO PRELIMINAR: De acordo com o exposto, a CONITEC em sua 62ª reunião, no dia 7 de dezembro de 2017, recomendou a não incorporação da lacosamida no SUS como terapia aditiva em pacientes com epilepsia focal, refratários aos tratamentos prévios com os fármacos antiepilépticos disponíveis. A matéria será disponibilizada em consulta pública. CONSULTA PÚBLICA: Foram recebidas 13 contribuições técnico-científicas e 23 contribuições de experiência ou opinião, sendo quase a totalidade discordante da recomendação preliminar da CONITEC. As contribuições técnico-científicas embasaram-se no fato das comparações indiretas se tratarem da melhor qualidade de evidência disponível, sobre a efetividade da lacosamida em resposta à pergunta de pesquisa realizada. As contribuições de experiência e opinião expressaram o desejo dos participantes em agregar mais um medicamento ao tratamento disponibilizado pelo SUS como uma alternativa a mais para os pacientes refratários. A CONITEC entendeu que não houve evidência adicional e/ou argumentação suficientes para alterar sua recomendação inicial. RECOMENDAÇÃO FINAL: Os membros da CONITEC em 07/03/2018 deliberaram por não recomendar a lacosamida como terapia aditiva em pacientes com epilepsia focal, refratários aos tratamentos prévios com os fármacos antiepilépticos disponíveis no SUS. Foi assinado o Registro de Deliberação nº 335/2018. DECISÃO FINAL: O Secretário de Ciência, Tecnologia e Insumos Estratégicos do Ministério da Saúde, por meio da Portaria SCTIE/MS nº 20, de 27 de abril de 2018, publicada no DOU nº 82 de 30 de abril de 2018, Seção I, tornou pública a decisão de não incorporar a lacosamida como terapia aditiva em pacientes com epilepsia focal refratários aos tratamentos prévios com os fármacos antiepilépticos disponíveis no SUS no âmbito do Sistema Único de Saúde - SUS.(AU)

Epileptic Spasms, a Journey to Find Therapeutics Based on Pathophysiology / 대한소아신경학회지

After the first description of infantile spasms (IS) in 1841, extensive clinical and laboratory investigations have been done to find the pathophysiology and the optimal treatments. The concept of the “infantile spasms” has been evolved to the “epileptic spasms”, which includes the spasms outside the infancy the pathophysiology of IS, however, is still unknown. There have been a few randomized trials that proved the efficacy of the anecdotally used drugs in IS including hormonal therapy and vigabatrin. Due to its relative low incidence (1/2000) and the variable etiologies, clinical studies have difficulties in making a clear conclusion. Thus, animal models were eagerly sought to find the pathophysiology based treatments with definite efficacy and several models are now available. In this paper, the current understandings of the epileptic spasms as well as the translational researches using the animal models of IS are reviewed. The latest evidences of therapeutics in IS are discussed shortly.

Recent advances in the pharmacotherapy of psychiatric disorders

Psychopharmacology has developed over approximately the past five decades. The remarkable proliferation of information in this area has made it difficult for clinicians to understand the characteristics of various psychotropic agents. Atypical antipsychotics including amisulpride, asenapine, aripiprazole, blonanserin, clozapine, iloperidone, lurasidone, olanzapine, paliperidone, quetiapine, risperidone, ziprasidone, and zotepine cause fewer extrapyramidal problems and have many clinical applications, but they can cause metabolic disturbances. Mood stabilizers and lamotrigine are widely used for bipolar disorder. Other novel anticonvulsants such as topiramate, oxcarbazepine, gabapentin, tiagabine, pregabalin, vigabatrin, levetiracetam, and riulzole have also been tested with diverging or inconclusive results. Antidepressants are commonly used in the clinical treatment of depression and anxiety disorder. However, the mechanism of action of medications used in the treatment of psychiatric disorders remains unclear. Understanding the mechanisms of action and clarifying the diagnosis may enhance the treatment outcome in psychiatry. In this review, we analyzed clinical pharmacology data for each drug within a class and discussed clinical strategies for administering currently available antipsychotics, mood stabilizer/anticonvulsants, and antidepressants widely used for various psychiatric indications.

Vigabatrín en el tratamiento de epilepsia causada por trastornos del desarrollo cortical / Vigabatrin in the treatment of epilepsy caused by cortical developmental disorders

Introducción. Se presenta una serie de pacientes que recibieron vigabatrín como tratamiento farmacológico para manejo de epilepsia causada por trastornos del desarrollo cortical. Objetivos. Evaluar la efectividad en cuanto a control de crisis en estos pacientes, teniendo en cuenta los hallazgos previos de la semejanza histológica y funcional de los trastornos del desarrollo cortical con las lesiones de la esclerosis tuberosa. Material y métodos. Se revisaron las historias de los pacientes con diagnóstico de trastorno del desarrollo cortical que recibieron este tratamiento como pacientes de la Liga Central Contra la Epilepsia, en el periodo comprendido entre los años 2005 a 2008. Se registraron datos sobre tipo de trastorno del desarrollo cortical, clasificación de la epilepsia, control de crisis antes y después del tratamiento con vigabatrín y presencia de efectos adversos. Resultados. Se revisaron 14 historias, el 57,11 % varones, con edades comprendidas entre 4 y 28 años. La forma de epilepsia más común fue la focal de diferente localización, y el tipo de malformación cortical más común fue la displasia cortical focal. El 50% de los pacientes mostró control superior al 90% respecto a las crisis registradas antes del inicio de tratamiento con Vigabatrin. Sólo un paciente mostró clínicamente disminución del campo visual y mejoró con la suspensión del tratamiento. Conclusión. En nuestra experiencia en pacientes pediátricos y adultos Vigabatrín es un tratamiento efectivo y seguro para el manejo de pacientes con epilepsia secundaria a trastornos del desarrollo cortical. Debe vigilarse estrechamente la posible aparición de los efectos adversos.

Introduction. We present a case series diagnosed with cortical developments disorders on vigabatrin treatment. Objetive. The study aims to evaluate the effectiveness in terms of crisis control these patients, according to histological findings and functional disorders caused by tuberous sclerosis.. Materials and methods. We reviewed records of patients diagnosed with cortical developmental disorder treated with Vigabatrin, in the period between 2005 to 2008 at the Liga central contra la epilepsia Bogotá, Colombia. Data about type of disturbance of cortical development, classification of epilepsy, treatment before and after treatment with vigabatrin and presence of adverse effects. Results. We reviewed 14 records, being 57.11% males, aged between 4 and 28 years. The most common form of epilepsy was the focal of a different location and type of cortical malformation was the most common focal cortical dysplasia. The response obtained in terms of seizure control in 50% of the patients was over 90% with respect to seizure frequency prior to the start of vigabatrin. Only one patient showed clinically decreased visual field and improved with the cessation of treatment. Conclusion. Vigabatrin is an effective and safe treatment for the management of patients with epilepsy secondary to cortical development disorders. Should closely monitor the possible occurrence of adverse effects.

Uso clínico de vigabatrin en epilepsia: [revisión] / Clinical use of vigabatrin in epilepsy: [review]

El Vigabatrin (VGB) es un medicamento anticonvulsivante cuyo uso en epilepsia se conoce desde hace tres décadas, está indicado en el control de crisis parciales complejas en epilepsias focales refractarias y en espasmos infantiles, especialmente los asociados a Esclerosis tuberosa, pero la descripción de efectos secundarios con la parición de defectos de los campos visuales (DCV) comenzó a limitar su uso en particular en Estados Unidos y otros países; sin embargo, en Europa, especialmente en el Reino Unido y Francia y en Asia principalmente en Japón, asi como en Latinoamérica, se continuó con su formulación, con buenos resultados en cuanto a control de crisis y manejo farmacocinético y se continuó acumulando experiencia en la aparición de efectos adversos especialmente el DCV. En Agosto de 2009, la Food and Drug Administration (FDA) aprueba su uso en Estados Unidos, como terapia adjunta para el tratamiento de crisis parciales complejas en adultos y en espasmos infantiles. La FDA implementa y recomienda métodos y estrategias de evaluación y control por los posibles efectos secundarios. En este artículo se revisan generalidades sobre las propiedades farmacológicas y farmacocinéticas, modo de acción, indicaciones en epilepsia y la relevancia de la limitación de su uso por los efectos colaterales en particular en los trastornos de la visión.

Vigabatrin is an antiepileptic drug, used in epilepsy since three decades ago, is indicated in the treatment of refractory complex partial seizures and infantile spasms, specially associated to tuberous sclerosis, but the Visual Fields Defects (VFD), has limited its use in some countries. Nevertheless in others such as United Kingdom, France, in Asia like Japan and Latinamerica, its use has continue, and now VGB was approved in the USA in August 2009 by the Food and Drug administration (FDA) as adjunctive therapy for the treatment of refractory complex partial seizures in adults and for the infantile spasm. The FDA implemented a Risk Evaluation and Mitigation Strategy to control for the possibility of adverse drug events. In this paper we review generalities, pharmacokinetic and pharmacologic properties and its role like antiepileptic drug, indications according to type of seizures and use limitations due to visual field defects.

Antiepilépticos de tercera generación:[revisión] / Third-generation antiepileptic: [review]

Aunque se ha descrito una alta probabilidad de control de pacientes recientemente diagnosticados con medicamentos de primera clase, se ha mostrado también como esta probabilidad se reduce al requerir tratamientos adjuntos de segunda línea. Por esta razón el descubrimiento y entendimiento de nuevos mecanismos farmacocinéticos y farmacodinámicos se ha convertido en uno de los puntos de mayor relevancia al diagnosticar y tratar pacientes con epilepsia. Estos argumentos han permitido desarrollar nuevos medicamentos que aumentan las opciones de tratamiento en pacientes epilépticos. Aunque estas opciones muestran resultados prometedores, la evidencia que los soporta se basa en la mayoría de los casos en estudios fase III de aprobación, por tal razón se hace necesario el diseño de nuevas pruebas que soporten su eficacia y seguridad en escenarios clínicos más próximos a la realidad. Presentamos una revisión no sistemática que discute los principales aspectos de estos medicamentos.

Although there is a high probability to control new onset epileptic patients with first line drugs, this probability is considerably lesser when add on therapy is needed. For this reason the understanding and discovering of new pharmacodynamic and pharmacokinetic mechanisms has became an important topic when diagnosing and treating epileptic patients. These arguments have allowed developing new anti epileptic drugs, options that improve the therapeutic options in these patients. Although these options have promising results on clinical setting, available evidence is limited to phase III studies, being necessary to design new trials that prove efficacy and safety in real clinical circumstances. We present a non systematic review that shows the main clinical and basic aspects about new antiepileptic drugs.

Efectividad del vigabatrín en niños: estado del arte: [revisión] / Effectiveness of vigabatrin in children: state of the art: [review]

Síndrome de West y epilepsia focal. En el primer caso la evidencia es comparable con la descrita para esteroides, sin embargo la incidencia de efectos colaterales es menor. Es de elección, con adecuado nivel de evidencia, en el manejo de epilepsia causada por esclerosis tuberosa. Se puede recomendar en el manejo de epilepsia focal, vigilando la aparición de crisis paradójicas.

We present a review regard the effectiveness of vigabatrine in the treatment of childhood epilepsy, emphasizing on West syndrome and focal epilepsy. In the first case, evidence is comparable with corticosteroids but with lesser side effects. In epilepsy caused by tuberous sclerosis Vigabatrine is the drug of choice. Vigabatrine can be recommended in the treatment of focal epilepsy monitoring the occurrence of paradoxical crisis.

Vigabatrín y compromiso de los campos visuales: ¿qué sabemos?: [revisión] / Vigabatrin and visual field compromise: ¿what do we know?: [review]

Se presenta una revisión de la literatura acerca de la presencia de alteración del campo visual asociada al uso de vigabatrín como antiepiléptico. Considerando las precauciones de prescripción derivadas de los efectos visuales de vigabatrin, presentamos una revisión no sistemática que muestra los principales datos respecto a prevalencia, factores de riesgo, fisiopatología y seguimiento de esta alteración. La prevalencia de aparición de la retinopatía es variable según las series revisadas, y los factores de riesgo como uso de dosis máxima, dosis acumulada y edad no están claramente demostrados, por lo que se considera a éste un efecto idiosincrático. Se recomienda la vigilancia oftalmológica y campimétrica periódica.

A review of the literature about the presence of visual field disturbance associated with the use of vigabatrin as an antiepileptic. Taking into account that there exists warnings to prescribe vigabatrine derived from visual defects, we present a non - systematic review that describe the main aspects regard to prevalence, risk factors, pathophisiology and following of this side effect. Prevalence of occurrence of retinopathy varies according to differentseries, and risk factors such as use of maximum dose, cumulative dose and age, so that this effect is considered idiosyncratic. Periodic ophthalmologic supervision is recommended.

Vigabatrina aumenta atividade da superóxido dismutase no corpo estriado de ratos após crises convulsivas induzidas pela pilocarpina / Vigabratine increases superoxide dismutase activity in striatum of rat after pilocarpine-induced seizures

CONTEXTO: Tem sido sugerido que durante as crises convulsivas induzidas pela pilocarpina pode ser observado aumento no estresse oxidativo cerebral. Estudos sugerem que compostos com atividade antioxidante podem fornecer certo grau de proteção contra a neurotoxicidade induzida pelas crises convulsivas. OBJETIVOS: O presente estudo investigou as ações farmacológicas da vigabatrina nas alterações comportamentais e na atividade enzimática da superóxido dismutase (SOD) no corpo estriado de ratos adultos. MÉTODOS: Ratos Wistar adultos (2 meses de idade) foram usados nos experimentos e divididos em quatro grupos. O primeiro foi tratado com solução salina 0,9 por cento (grupo controle). O segundo grupo foi tratado com pilocarpina (400 mg/kg, i.p., grupo P400). O terceiro grupo foi tratado com vigabatrina (500 mg/kg, i.p., grupo VGB) e o quarto grupo foi tratado com vigabatrina (500 mg/kg, i.p.) e 30 minutos depois com pilocarpina (400 mg/kg, i.p., grupo VGB + P400). Os animais que apresentaram crises convulsivas, estado de mal epiléptico e não morreram durante o período de 24 horas de observação foram sacrificados para dissecação do corpo estriado para realização da determinação da atividade da SOD. RESULTADOS: Os estudos comportamentais revelaram que, após administração de pilocarpina, todos os animais apresentaram sinais colinérgicos periféricos, movimentos estereotipados e tremores. No mesmo grupo, foram observados, em 75 por cento dos animais, crises convulsivas e o estado de mal epiléptico. Por sua vez, o pré-tratamento com vigabatrina produziu redução significativa de 50 por cento nas crises convulsivas. Com relação aos estudos neuroquímicos, não foram observadas alterações na atividade da SOD no corpo estriado do grupo P400, em comparação aos valores do grupo controle. No entanto, no grupo VGB + P400 foi visto aumento significativo na atividade da SOD de 34 por cento e 35 por cento, quando comparado aos grupos controle e P400, respectivamente. ...

BACKGROUND: Pilocarpine-induced seizures have been suggested to be mediated by increases in oxidative stress. Current studies have suggested that antioxidant compounds may afford some level of neuroprotection against the neurotoxicity of seizures. OBJECTIVES: This study investigated the pharmacological actions of vigabatrin on behavioral changes and superoxide dismutase (SOD) activity in striatum of adult rats. METHODS: Adult rats (2 months old) were used in the experiments and divided into four groups. The first was treated with 0.9 percent saline (control group). The second group was treated with pilocarpine (400 mg/kg, i.p., P400 group). The third group received vigabatrin alone (500 mg/kg, i.p., VGB group) and the fourth group was treated with vigabatrin (500 mg/kg, i.p.) and 30 minutes later received pilocarpine (400 mg/kg, i.p., VGB + P400 group). The animals which had seizures and status epilepticus (SE) and did not die within 24 hours of observation were sacrificed to perform the neurochemical studies. RESULTS: Behavioral studies showed that the administration of pilocarpine produces peripheral cholinergic signs, tremors and stereotyped movements in all animals. An amount of 75 percent of those rats developed to seizures and SE. In turn, the pre-treatment with vigabatrin produced a 50 percent reduction in the rate of seizures and SE. Regarding the neurochemical studies, there were no changes in the striatal SOD activity in P400 group as compared to the control group. However, in the VGB + P400 group it was verified significant increases in SOD activity of 34 percent and 35 percent as compared to control and P400 group, respectively. DISCUSSION: Our results indicate that behavioral changes occur during seizures, but SOD activity remained unaltered during the acute phase of the convulsive crisis. Our findings suggest that the anticonvulsant effect of vigabatrin may be the result of modulation of this enzyme, in an attempt to protect ...

Local experience of management of infantile spasms and its treatment: a retrospective study of ten cases

To evaluate and assess the efficacy of ACTH and Vigabatrin in Symptomatic variety of Infantile Spasm in Pakistani patients to document local experience. This is a retrospective study carried out at Neuro Diagnostic Centre, Hamdard University Hospital [Taj Medical Complex], Karachi, from January 2006 to December 2008. Patients registered with Infantile Spasm to the Neuro Diagnostic Centre, Karachi, Pakistan between January 2006 to December 2008 were included in the study. The total number of patients was ten; six male and four female. Age ranged from four months to three and a half years. All patients demonstrated electroencephalographic evidence of hypsarrhythmia and a variety of jerks representing Infantile Spasm. ACTH was administered in nine out of ten patients but always in combination. Six out of ten patients had Vigabatrin used in tandem with ACTH initially and later Vigabatrin alone [was continued in five of these patients]. In one patient Vigabatrin was continued with ACTH and Valproate. ACTH was combined with Valproate in three of the patients while Valproate alone was tried in one patient. All patients on ACTH in whatever combination responded adequately and some of them dramatically becoming seizure-free. The patient on Valproate alone did not respond early and took almost 20 days from start of treatment to become seizure-free. ACTH was highly effective especially in patients with symptomatic form of Infantile Spasm. Although prednisolone [oral] has not been tried in this study but in affording patients ACTH should remain the first-line treatment for Infantile Spasm especially the symptomatic variety

Seizure characteristics in Aicardi syndrome

We are describing a one year old Bahraini girl with Aicardi syndrome; she has agenesis of the corpus callosum, inter hemispheric cyst, neuronal migration disorder, ocular abnormalities and infantile spasm. She presented initially with focal seizure which later evolved into infantile spasm, her seizures were intractable to different anticonvulsant medications except for Vigabatrin which reduced her seizure episodes dramatically

Study of Clinical Course of Epilepsy in Children with Tuberous Sclerosis Complex / 대한간질학회지

PURPOSE: The aim of this study is to investigate the incidence, clinical features and outcome in pediatric tuberous sclerosis complex(TSC) patients with epilepsy. METHODS: Fifty seven of 74 patients (77.0%) were included in the study, who were diagnosed with epilepsy associated with TSC from 1991 to 2008. Clinical data were obtained from medical records retrospectively. RESULTS: Of the 57 patients, initial seizure types were infantile spasms (n=25, 43.8%), complex partial seizure (n=24, 42.1%), generalized tonic seizure (n=5, 8.7%), simple partial seizure (n=2, 3.4%), and atonic seizure (n=1, 1.7%), respectively. Seventeen patients (29.8%) had changes of their seizure types during the clinical course. Excluding the five patients with insufficient data, 52 patients were treated with antiepileptic drugs and four of them underwent epilepsy surgery. Twenty-six of 52 patients (50.0%) with medical treatment and two patients (50.0%) with epilepsy surgery became seizure free. Among the patients with infantile spasms, vigabatrin induced seizure freedom in 13 of 14 patients (92.8%) within four weeks, and five of them maintained seizure remission with vigabatrin monotherapy. CONCLUSIONS: Half of epileptic patients showed good responses to medical treatment, especially vigabatrin for infantile spasms. Epilepsy surgery can be treatment option for selected patients.

Eficacia de la vigabatrina en la epilepsia refractaria del niño / Efficacy of the vigabatrina in the fireproof epilepsy of the boy

Se evaluó la respuesta terapéutica a la vigabatrina en terapia adjunta en 28 pacientes, con diagnóstico de epilepsia refractaria, hospitalizados en el servicio de neuropediatría del Hospital Pediátrico Juan M. Márquez, entre enero de 2002 y julio de 2005. Se analizaron las variables sexo, edad al ingreso y al inicio de la epilepsia, tipo de crisis, diagnóstico etiológico, fallo de drogas previas, tipo de politerapia y respuesta al mes, 3 meses, 6 meses y 1 año de tratamiento, así como las reacciones adversas a la medicación. Se concluye que la epilepsia tiene un inicio temprano, responde a daño hipóxico y lesiones estructurales del cerebro en más del 70 por ciento, fallo terapéutico previo con 2 a 4 drogas y la politerapia más aceptada es con valproato. La respuesta eficaz se obtiene en el 67,9 por ciento de los casos, con reducción de las crisis a la mitad en el 21,4 por ciento de los pacientes al año de tratamiento. Los espasmos epilépticos asociados a crisis parciales tienen una respuesta más tardía que los espasmos como única forma de crisis. La vigabatrina es bien tolerada y sus reacciones adversas son leves y transitorias.

The therapeutical response to Vigabatrin in add-on therapy was evaluated in 28 patients diagnosed with refractory epilepsy and hospitalized at Neuropediatric Service of Juan Manuel Márquez pediatric hospital from January 2002 to July, 2005. Variables such as sex, age at admission, onset of epilepsy, type of seizure, etiological diagnosis, previous drug failures, type of multitherapy and response one, three, six months and 1 year after treatment and adverse reactions to medication. It was concluded that epilepsy has an early onset, is the result of hypoxic damage and structural lesions of the brain in over 70percent of cases, failed therapy with 2 to 4 drugs and that the most accepted multi-therapy was Valproate. Positive response to treatment occured in 67,9 percent of cases, seizures were half-reduced in 21,4 percent of the patients after one year of treatment. Epileptic spams associated to partial seizures showed later response than spams as the only form of seizure. Vigabatrin was well-tolerated and its adverse effects were transient and slight.

Treatment of infantile spasms with sodium valproate followed by benzodiazepines.

OBJECTIVE: To review the result of the infantile spasms' treatment with sodium valproate followed by nitrazepam or clonazepam. STUDY DESIGN: Descriptive retrospective study. SETTING: Srinagarind Hospital, Department of Pediatrics, Faculty of Medicine, Khon Kaen University, Khon Kaen, Thailand. MATERIAL AND METHOD: Twenty-four infantile spasms admitted between January 1994 and December 2003 were analyzed. The inclusion criteria were the patients with infantile spasms clinically diagnosed by the pediatric neurologist, having hypsarrhythmic pattern EEG, and receiving sodium valproate with or without nitrazepam or clonazepam. The patients who had an uncertain diagnosis, incomplete medical record, or that were incompletely followed up were excluded. Data were collected on sex, age at onset of seizure, type of infantile spasms, associated type of seizure, predisposing etiological factor, neuroimaging study, and the result of treatment including cessation of spasms, subsequent development of other seizure types, quantitative reduction of spasms, relapse rates of spasms, psychomotor development, and adverse effects of AEDs. RESULTS: The mean age at onset was 177 days. The male-to-female ratio was 1:1.2. There were 13 cryptogenic (54.2%) and 11 symptomatic (45.8%) infantile spasms. The most common predisposing etiological factors in symptomatic cases were hypoxic ischemic encephalopathy (45.5%) and microcephaly (36.4%), respectively. Ten patients received sodium valproate (41.7%), another 10 received sodium valproate with clonazepam (41.7%), and four received sodium valproate with nitrazepam (16.7%). Both, the complete cessation rate and the 50% reduction of spasms rate were 45.8%. The duration to complete cessation was 70 days. The relapse rate was 18.2%. The rate of delayed psychomotor development was 83.3%. The mean duration of follow-up was 49.6 months. CONCLUSION: The authors propose to use sodium valproate concomitantly with benzodiazepines, especially clonazepam, in situations such as unavailability, intolerability, or adverse effects of ACTH or vigabatrin, or in a patient who does not respond to ACTH or vigabatrin.