[Post-operative progress of patients with Rasmussen's encephalitis who have undergone hemispherotomy surgery]. / Evolucion posquirurgica en pacientes con encefalitis de Rasmussen operados por hemisferotomia.

INTRODUCTION: Rasmussen's encephalitis (RE) is a progressive pathology affecting the brain that causes unilateral hemispheric atrophy, neurological dysfunction and refractory epilepsy. Hemispherotomy is considered the most effective treatment today, but some cases present certain peculiarities that can seriously affect the decision to go ahead with this procedure. AIMS: To evaluate the post-operative progress made by children with RE who have undergone hemispherotomy surgery, and who, in the pre-operative assessment, presented certain characteristics that complicated the decision to perform surgery. PATIENTS AND METHODS: The sample selected for study consisted of the cases of RE attended in the Hospital Sao Paulo between 2003 and 2012 who, in the pre-surgery evaluation, presented clinical, electroencephalographic or neuroimaging evidence of involvement of both brain hemispheres, compromise of the dominant brain hemisphere, absence of severe neurological deficit and absence of criteria for refractory epilepsy and atypical crises. The post-operative assessment of the epileptic seizures was evaluated using the Engel scale; motor function was analysed with the Gross Motor Function Classification System and Manual Ability Classification System scales, and language was evaluated clinically. RESULTS: Six cases were selected (four girls), with a mean age at clinical onset of 3.3 ± 1.2 years (range: 2-7 years) and a mean age at hemispherotomy of 6.7 years (range: 2.3-16.5 years). The mean post-surgery follow-up time was three years (range: 0.5-7.2 years). In the post-surgery evaluation of the epileptic seizures, four cases were classified as Engel class I (66%); there was some improvement in motor functioning in five of them, and language improved in all cases. CONCLUSIONS: Hemispherotomy must be considered an efficient option for treatment in children with RE.

Evolución posquirúrgica en pacientes con encefalitis de Rasmussen operados por hemisferotomía / Post-operative progress of patients with Rasmussen’s encephalitis who have undergone hemispherotomy surgery

Introducción. La encefalitis de Rasmussen (ER) es una afección cerebral progresiva que provoca una atrofia hemisférica unilateral, disfunción neurológica y epilepsia refractaria. La hemisferotomía se considera actualmente el tratamiento más efectivo, pero algunos casos presentan particularidades que dificultan la decisión de realizarla. Objetivo. Evaluar la evolución posquirúrgica de niños con ER operados por hemisferotomía, que en la evaluación prequirúrgica presentaron algunas características que dificultaron la decisión quirúrgica. Pacientes y métodos. Se seleccionaron los casos con ER, atendidos en el Hospital São Paulo entre 2003 y 2012, que en la evaluación prequirúrgica presentaran evidencias clínicas, electroencefalográficas o en la neuroimagen de participación de ambos hemisferios cerebrales, afectación del hemisferio cerebral dominante, ausencia de déficit neurológico grave y ausencia de criterios de epilepsia refractaria y crisis atípicas. Para la evaluación posquirúrgica de las crisis epilépticas se utilizó la escala de Engel; para analizar la función motora, las escalas Gross Motor Function Classification System y Manual Ability Classification System, y el lenguaje se evaluó clínicamente. Resultados. Se seleccionaron seis casos (cuatro niñas), con edad media de inicio clínico de 3,3 ± 1,2 años (rango: 2-7 años) y edad media de hemisferotomía de 6,7 años (rango: 2,3-16,5 años). El tiempo medio de seguimiento posquirúrgico fue de tres años (rango: 0,5-7,2 años). En la evaluación posquirúrgica de las crisis epilépticas, cuatro casos se clasificaron como Engel clase I (66%); hubo mejoría en la función motora en cinco, y mejoría en el lenguaje, en todos. Conclusión. La hemisferotomía debe considerarse una opción eficiente de tratamiento en niños con ER (AU)

Introduction. Rasmussen’s encephalitis (RE) is a progressive pathology affecting the brain that causes unilateral hemispheric atrophy, neurological dysfunction and refractory epilepsy. Hemispherotomy is considered the most effective treatment today, but some cases present certain peculiarities that can seriously affect the decision to go ahead with this procedure. Aims. To evaluate the post-operative progress made by children with RE who have undergone hemispherotomy surgery, and who, in the pre-operative assessment, presented certain characteristics that complicated the decision to perform surgery. Patients and methods. The sample selected for study consisted of the cases of RE attended in the Hospital São Paulo between 2003 and 2012 who, in the pre-surgery evaluation, presented clinical, electroencephalographic or neuroimaging evidence of involvement of both brain hemispheres, compromise of the dominant brain hemisphere, absence of severe neurological deficit and absence of criteria for refractory epilepsy and atypical crises. The post-operative assessment of the epileptic seizures was evaluated using the Engel scale; motor function was analysed with the Gross Motor Function Classification System and Manual Ability Classification System scales, and language was evaluated clinically. Results. Six cases were selected (four girls), with a mean age at clinical onset of 3.3 ± 1.2 years (range: 2-7 years) and a mean age at hemispherotomy of 6.7 years (range: 2.3-16.5 years). The mean post-surgery follow-up time was three years (range: 0.5-7.2 years). In the post-surgery evaluation of the epileptic seizures, four cases were classified as Engel class I (66%); there was some improvement in motor functioning in five of them, and language improved in all cases. Conclusions. Hemispherotomy must be considered an efficient option for treatment in children with RE (AU)

Atypical presentation in Rasmussen encephalitis: delayed late-onset periodic epileptic spasms.

A five-and-a-half-year-old girl started experiencing progressive left hemiparesis at age two and a half years. At age five years and four months she started presenting clusters of asymmetric periodic epileptic spasms with no hypsarrhythmia. The ictal EEG showed periodic, constant and stereotyped complexes. Serial brain imaging revealed progressive atrophy of the right hemisphere with increased T2 signal on MRI. She underwent a right hemispherotomy, and histological examination showed signs of inflammation and features of focal cortical dysplasia (FCD). She has been seizure-free for 16 months. This case is unique in the following aspects: the presence of typical Rasmussen encephalitis features of progressive unilateral brain involvement without seizures, a delay of almost three years prior to seizure onset; an atypical seizure type presentation with periodic epileptic spasms and the presence of FCD associated with inflammatory changes. [Published with video sequences].