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Rasmussen encephalitis: long-term outcome after surgery

Child's Nervous System volume 25, pages 583–589 (2009)Cite this article

Abstract

Background and purpose

Rasmussen encephalitis (RE) is characterized by intractable epilepsy, progressive hemiparesis, and unilateral hemispheric atrophy. The progression of the symptoms to significant neurological impairment usually occurs within months to a few years. RE causes are unknown, although evidence of an autoimmune process has been extensively described in the literature. Antiepileptic drugs are usually not effective to control seizures or cerebral atrophy; despite data supporting a beneficial effect of early immunosuppressive and immunomodulatory interventions, for intractable seizures in RE patients with advanced disease, 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.

Materials 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), taking variables such as gender; age at epilepsy onset; seizure semiology; seizure frequency; interictal and ictal electroencephalographic (EEG) findings; age at surgery, when done; duration of epilepsy; surgery complications; follow-up duration; anatomo-pathological findings; post-surgery seizure; language and cognitive outcome; and anti-epileptic drug treatment after surgery into account.

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). Immunomodulatory therapy was tried in 12 patients (48%). Twenty-three patients underwent surgery. The mean follow-up was 63.3 months. Eleven patients had total seizure control. Twelve individuals persisted with seizures consisting of mild facial jerks (six patients), occasional hemigeneralized tonic–clonic seizures (three patients), and frequent tonic–clonic seizures (three patients). Mental and language impairment was observed in 15 and 12 patients, after surgery, respectively. Eight patients presented post-operative cognitive decline, while only two patients had cognitive improvement. Comparing pre- and post-operative language deficits, 66.7% of the 12 patients with language disturbance did not improve after surgery.

Conclusions

This retrospective study reported the clinical and electrographic analysis, as well as the evolution of 23 patients with RE. Patients were divided into two groups: fast evolution and slow evolution to hemiparesis and epilepsia partialis continua. These groups may represent different RE substrates. 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 disturbance, which did not improve after surgery in 66.6% of patients. Cognitive evaluation showed that the majority of the patients did not have any significant improvement, and 38.1% had cognitive deterioration after surgery.

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Acknowledgments

The authors thank the epilepsy fellows, neurology residents, and staff of the Epilepsy Outpatient Clinics and Video-EEG Monitoring Unit of the Hospital das Clínicas da Faculdade de Medicina de Ribeirão Preto/USP. This work was supported by FAPESP, CNPq, CInAPCe-FAPESP, and FAEPA.

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Authors and Affiliations

  1. Department of Neurology, Ribeirão Preto School of Medicine, University of São Paulo, Ribeirão Preto, Brazil

    Vera C. Terra-Bustamante, Cecília Souza-Oliveira, Sara Escorsi-Rosset & Américo C. Sakamoto

  2. Department of Neurosurgery, Ribeirão Preto School of Medicine, University of São Paulo, Ribeirão Preto, Brazil

    Helio R. Machado & Ricardo dos Santos Oliveira

  3. Department of Pathology, Ribeirão Preto School of Medicine, University of São Paulo, Ribeirão Preto, Brazil

    Luciano N. Serafini

  4. Department of Neurology and Neurosurgery, Universidade Federal de São Paulo/Escola Paulista de Medicina, São Paulo, Brazil

    Elza Márcia Targas Yacubian, Maria da Graça Naffah-Mazzacoratti, Carla A. Scorza, Esper A. Cavalheiro, Fulvio A. Scorza & Américo C. Sakamoto

  5. Department of Neurology—CIREP, Campus Universitário, Ribeirão Preto, SP, CEP 14048-900, Brazil

    Vera C. Terra-Bustamante

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  1. Vera C. Terra-Bustamante
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  2. Helio R. Machado
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  3. Ricardo dos Santos Oliveira
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  5. Cecília Souza-Oliveira
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  7. Elza Márcia Targas Yacubian
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  8. Maria da Graça Naffah-Mazzacoratti
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  11. Fulvio A. Scorza
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  12. Américo C. Sakamoto
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Correspondence to Vera C. Terra-Bustamante.

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Terra-Bustamante, V.C., Machado, H.R., dos Santos Oliveira, R. et al. Rasmussen encephalitis: long-term outcome after surgery. Childs Nerv Syst 25, 583–589 (2009). https://doi.org/10.1007/s00381-008-0795-1

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