Subacute sclerosing panencephalitis resembling Rasmussen's encephalitis on magnetic resonance imaging

Subacute sclerosing panencephalitis (SSPE) is a rare, slowly progressing but invariably fatal disease that is related to a prior measles virus infection and most commonly affects paediatric patients. Magnetic resonance (MR) imaging is the modality of choice for determining such changes in white matter. SSPE typically demonstrates bilateral but asymmetric periventricular and subcortical white matter involvement. We herein report a rare case of unilateral white matter involvement in a 13-year-old boy with SSPE that closely simulated Rasmussen's encephalitis. To the best of our knowledge, this is the first report of an atypical presentation on MR imaging in which SSPE was a rare cause of unilateral brain parenchymal involvement in a patient with intractable seizures.

Electroencephalographic Features of Rasmussen's Encephalitis by Stage

PURPOSE: The purpose of this study was to describe the characteristic electroencephalographic features in Rasmussen's encephalitis by stage. METHODS: Patients diagnosed with Rasmussen's encephalitis at Seoul National University Children's Hospital were retrospectively assessed. We analyzed the background activities and epileptiform discharges from electroencephalography (EEG) findings to identify the characteristic EEG features by stage. RESULTS: Seven patients were included in the study. The mean age of first seizure onset was 6.7 years, and the mean duration of the prodromal phase was 21.4 months. During disease course, background activities, such as slow waves, were more prominent and diffuse, and contralateral slow waves were observed. In most patients, focal epileptiform discharges were observed during all stages without change. CONCLUSION: As Rasmussen's encephalitis progresses, background abnormalities in the affected hemisphere increased, and contralateral slow waves occurred. However, characteristic EEG findings that were distinguishable at each stage were not observed.

Findings of Transcranial Doppler during Simple Partial Seizures with Rasmussen Encephalitis: 8 Cases Report / 中国康复理论与实践

@#Objective To analyze the blood flow velocities in middle cerebral artery with Rasmussen encephalitis. Methods8 patients with Rasmussen encephalitis were detected bilateral middle cerebral artery in both period of onset and intermission during simple partial seizures with Transcranial Doppler. ResultsThe mean velocity variance between focus side and non-focus side was minimums among the 3 indexes observed. The mean velocity of MCA displayed uneven increase during seizures. ConclusionThe mean velocity was the most stable index for judging.

Changes of Blood Flow Velocities in Middle Cerebral Artery with Rasmussen Encephalitis: 8 Cases Report / 中国康复理论与实践

@#ObjectiveTo discuss clinical significance of changes in cerebral blood flow velocities with Rasmussen encephalitis. Methods8 cases diagnosed as Rasmussen encephalitis were measured the blood flow velocities in middle cerebral artery (MCA) with transcranial Doppler in ictal and interictal seizures. ResultsIctal mean flow velocity in the ipsilateral MCA to the epileptogenic foci increased 14.02%～48.14% to interictal one, while it was -0.74%～22.63% in the contralateral MCA. ConclusionAn increased flow velocity has been found in the ipsilateral MCA to the epileptogenic foci during seizure in Rasmussen encephalitis patients.

Rasmussen Encephalitis: longterm outcome after surgery / Encefalite de Rasmussen: avaliação de resultados depois da cirurgia

BACKGROUND AND PURPOSE: Rasmussen Encephalitis (RE) is characterized by intractable epilepsy, progressive hemiparesis and unilateral hemispheric atrophy. The progression of the symptoms usually occurs within months to few years. Antiepileptic drugs are usually not effective to control disease progression and epilepsy surgery in the form of hemispheric disconnection has been considered the treatment of choice. This work describes the clinical and electrographic analyses, as well as the post-operative evolution of patients with RE. PATIENTS AND METHODS: This work includes all the patients with RE evaluated from January 1995 to January 2008 by the Ribeirão Preto Epilepsy Surgery Program (CIREP) considering demographic data, interictal and ictal electroencephalographic (EEG) findings; anatomo-pathological findings and clinical outcome. RESULTS: Twenty-five patients were evaluated, thirteen were female. Mean age of epilepsy onset was 4.4±2.0 years. There were no differences between patients with slow and fast evolution with respect to age of epilepsy onset (p=0.79), age at surgery (p=0.24), duration of epilepsy (0.06), and follow-up (p=0.40). There were no correlations between the presence of bilateral EEG abnormalities or the absence of spikes and post-operative seizure outcome (p=0.06). Twenty-three patients underwent surgery. The mean follow-up was 75.3 months. Eleven patients had total seizure control. Twelve individuals persisted with seizures consisting of mild facial jerks (6 patients), occasional hemigeneralized tonic-clonic seizures (3 patients), and frequent tonic-clonic seizures (3 patients). Mental and language impairment was observed in 15 and 12 patients, after surgery, respectively. CONCLUSIONS: This retrospective study reported the clinical and electrographic analysis, as well as the evolution of 23 patients with RE. Fourteen patients achieved satisfactory seizure control, three patients had partial response to surgery, and five patients had maintenance of the pre-operative condition. All patients with left side involvement presented with some language and cognitive disturbance.

INTRODUÇÃO E OBJETIVOS: A Encefalite de Rasmussen (ER) é caracterizada por epilepsia intratável, hemiparesia progressiva e atrofia hemisférica unilateral. A progressão dos sintomas geralmente ocorre em meses ou poucos anos. As drogas antiepilépticas são usualmente ineficazes no controle da progressão da doença e o tratamento cirúrgico, com desconexão hemisférica tem sido considerado o tratamento de escolha. Neste trabalho descreveremos os achados clínicos e eletrográficos, assim como a evolução pós-operatória de pacientes com ER. PACIENTES E MÉTODOS: foram incluídos todos os pacientes com ER avaliados no período de janeiro de 1995 a janeiro de 2008, no Centro de Cirurgia de Epilepsia de Ribeirão Preto (CIREP), sendo considerados os dados demográficos, os achados do eletrencefalograma (EEG) interictal e ictal, resultado anatomo-patológico e o seguimento clínico. RESULTADOS: Vinte e cinco pacientes foram avaliados, 13 eram do sexo feminino. A idade média de início da epilepsia foi de 4.4±2.0 anos. Não houve diferenças significativas entre os pacientes com evolução lenta ou rápida considerando-se a idade de início da epilepsia (p=0,79), idade da cirurgia (p=0,24), duração da epilepsia (p=0,06) e tempo de seguimento (p=0,40). Não houve correlação entre a presença de alterações bilaterais ou ausência de descargas ao EEG e o seguimento pós-operatório (p=0,06). Vinte e três pacientes foram submetidos à cirurgia. O tempo médio de seguimento foi de 75,3 meses. Onze pacientes evoluíram com controle total das crises. Doze pacientes permaneceram com crises que consistiram de clonias faciais sutis (6 pacientes), crises tônico-clônicas hemigeneralizadas ocasionais (3 pacientes) ou crises tônico-clônicas frequentes (3 pacientes). Alterações cognitivas e de linguagem foram observadas em 15 e 12 pacientes após a cirurgia, respectivamente. CONCLUSÕES: este estudo retrospectivo relatou os achados clínicos, eletrográficos e a evolução de 23 pacientes. Controle satisfatório das crises foi obtido em 14 pacientes. Três pacientes tiveram resposta parcial com a cirurgia e cinco pacientes mantiveram o quadro pré-operatório. Todos os pacientes com envolvimento do hemisfério cerebral esquerdo evoluíram com distúrbio de linguagem e cognitivo.

MRI findings in the diagnosis and monitoring of rasmussen's encephalitis / Achados de RM no diagnóstico e monitorização da encefalite de Rasmussen

Rasmussen's encephalitis is a devastating syndrome of multifocal brain dysfunction and focal seizures. Magnetic resonance (MR) findings, associated with clinical data and electroencephalogram (EEG), may indicate the diagnosis and could be an indicative of prognosis. We studied 5 patients with Rasmussen's encephalitis, assessing clinical history and MR images. All patients had refractory focal seizures with a predominant motor component associated with hemispheric atrophy, that was proportional to severity of disease and neurological deficits in these patients. Gray and white matter abnormal signal on T2 MR images were found in patients who had hemiparesis. It was not related to the duration of the disease but to aggressiveness. MR proton spectroscopy in severe disease showed lactate and choline increase and decreased NAA, reflecting neuronal and axonal loss, gliosis and elevated membrane turnover and recent - crisis (not controlled). MR studies, in addition to help in diagnosis, may be useful for monitoring metabolic changes and progression of disease in Rasmussen's encephalitis.

A encefalite de Rasmussen é uma devastadora síndrome com disfunção cerebral multifocal e convulsões focais. Achados de ressonância magnética (RM), associados aos dados clínicos e de eletrencefalograma (EEG), podem indicar o diagnóstico e podem ser indicativos de prognóstico. Foram estudados 5 pacientes com encefalite de Rasmussen, avaliando a história clínica e imagens de RM. Todos os pacientes apresentavam crises epilépticas focais refratárias com componente predominantemente motor associadas à atrofia hemisférica, que foi proporcional à gravidade da doença e déficits neurológicos nestes pacientes. Alteração da intensidade de sinal nas substâncias branca e cinzenta, nas sequências ponderadas em T2, foram encontradas nos pacientes com hemiparesia. Ela não estava relacionada com a duração da doença, mas à severidade. A espectroscopia de prótons por RM na doença severa demonstrou aumento dos níveis de colina e lactato e diminuição de N-acetilaspartato, refletindo perda neuronal e axonal, gliose e aumento de turnover de membrana e crise recente (não controlada). Estudos de RM, além de ajudar no diagnóstico, podem ser úteis para acompanhar alterações metabólicas e progressão da doença na encefalite de Rasmussen.

A Case of Long-term Follow-up Rasmussen's Encephalitis / 대한간질학회지

Rasmussen encephalitis (RE) is a rare, severe, and progressive disorder characterized by focal motor seizures, epilepsia partialis continua, hemiparesis, and intellectual decline. Although clinical features of RE are heterogenous, the progression of the disease is generally divided into three stages which are prodromal, acute, residual stages. We report a 29-year-old woman who had shown typical progression of the disease but preserved cognitive function during a longterm follow-up.

A Case of Adult-onset Rasmussen's Encephalitis Responsive to High-dose Steroid and Antiepileptic Medication

Rasmussen's encephalitis is a rare autoimmune disorder characterized by intractable epilepsy and progressive unilateral hemispheric dysfunction as well as mental deterioration. Although the disorder typically begins in childhood, a few cases have been reported with symptom onset in adulthood showing variable clinical characteristics. We report a 63-year-old woman with adult-onset partial seizure with intermittent secondary generalization, progressive right hemiparesis, and aphasia, who showed positive response to high-dose steroid and antiepileptic drugs.