Dados do Trabalho

Título

RASMUSSEN ENCEPHALITIS: DRUG TREATMENTS AND RESULTS AFTER SURGERY FOLLOWED UP IN A LARGE MEDICAL CENTER IN BRAZIL

Introdução

Rasmussen Encephalitis is characterized by epilepsia partialis continua, hemiparesis, cognitive decline and progressive cerebral hemiatrophy1. The typical form begins before age 10 and is divided into three phases: prodromal, acute, and residual2. The most accepted cause is autoimmunity3.

Objetivo

The present study aims to evaluate the epidemiological profile of patients with Rasmussen Encephalitis undergoing hemispherectomy surgery and the outcome of epileptic seizures.

Métodos

Eighteen patients' medical records were evaluated between the years 2014 and 2022. Children treated at the Hospital das Clínicas da Universidade de São Paulo who met the criteria for Rasmussen Encephalitis were included, totaling 12 children who underwent hemispherectomy surgery.

Resultados

The disease started at age 5.9. Epilepsy was the first symptom in 91% (n.11) except for hemiparesis in one participant. Progressively all developed severe and refractory seizures.
Epilepsia partialis continua were present at 50% (n. 6). All children had focal motor seizures (between tonic and clonic seizures). Second generalized seizures occurred in 25% and segmental myoclonic seizures in 8.3% (n.1). Evolutionarily, cognitive decline was observed 83.3% (n.10), hemiparesis 75% (n.9), behavioral changes in 16.7% (n.2), language alterations 16.7% (n.2) and psychiatric symptoms in 8.3% (n.1).l
On resonance, progressive brain hemiatrophy was observed (100%). In the electroencephalogram, focal epileptiform activity was unanimous, multifocal activities were progressively confined to one hemisphere in 16.7% (n.2), and in one patient (8.3%) had bilateral activity. Half of the participants underwent cerebrospinal fluid analysis, being normal in 41.7% (n.5), oligoclonal bands were observed in one (8.3%).
Immunotherapies were the primary strategy, being Intravenous Immunoglobulin isolated in 25% (n.3) and 33.3% (n.4) pulse therapy with Methylprednisolone, and dual therapy 25%(n.3). Two did not use any modality 16.7%.
After surgery, 75% (n. 9) had seizure resolution, 16.7% (n. 2) had reduction, and 1 (8.3%) maintained electrographic seizures.
Postoperatively, Topiramate, Clobazam (58.3%), and Carbamazepine (33,3%) were maintained, and 25% (n.3) were not taking any medication.

Conclusões

The data obtained in this study are similar to the literature1,4 on the development of the epilepsy5 and the symptoms of the various stages of the disease4. Surgery is a curative treatment for seizures1, and children who have undergone surgery show a good response.

Palavras chave

Rasmussen Encephalitis, seizures, hemispherectomy.

Referências (se houver)

1. Varadkar, Sophia et al. Rasmussen’s encephalitis: clinical features, pathobiology, and treatment advances. Lancet Neurol 2014;13:195–205. http://dx.doi.org/10.1016/S1474- 4422(13)70260-6.
2. Lagarde, et ali. Medical treatment of Rasmussen’s Encephalitis: A systematic review. Revue Neurologique, Elsevier Masson, 2022, ff10.1016/j.neurol.2022.01.007ff. ffhal03563010f.
3. Granata, Tiziana; Andermann, Frederick. Rasmussen encephalitis. Handbook Of Clinical Neurology, [s.l.], p.511-519, 2013. Elsevier. http://dx.doi.org/10.1016/b978-0-444-52891-9.00054-3.
4 Longaretti. Evolution of the EEG in children with Rasmussen’s syndrome. Epilepsia, 53(9):1539–1545, 2012.
5. Tang e Tianfu Li. Rasmussen’s encephalitis: mechanisms update and potential therapy target. 2020.

Declaração de conflito de interesses de TODOS os autores

Sem conflito de Interesses.