Functional hemispherectomy for adult rasmussen encephalitis: a case report and literature review

Wang, Qun; Zhu, Zhanpeng; Wang, Guangming; Sun, Lichao; Qiu, Jiqing

doi:10.5137/1019-5149.jtn.21188-17.1

Cited by 6 publications

(7 citation statements)

References 8 publications

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“…20,38 Esta reorganização cerebral parece ocorrer também após hemisferectomia do hemisfério esquerdo em doentes com SR, alcançando relativo bom prognóstico linguístico a longo prazo mesmo sem afasia prévia à cirurgia. 18,19,39,40 Apesar de tudo, não há ainda evidência suficiente sobre o melhor timing para propor cirurgia, especialmente nos casos com afeção do hemisfério dominante para a linguagem.…”

Section: Discussionunclassified

Rasmussen’s Syndrome: Case-Series Study from a Refractory Epilepsy Reference Center and Revision of Literature

Serôdio,

Pires,

Azurara

et al. 2024

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Introduction: Rasmussen’s syndrome (RS) is a rare immunomediated disease of childhood-predominant onset, presenting with refractory epilepsy and neurological deficits associated to progressive brain hemiatrophy. Its pathophysiological mechanisms are not well understood, and the effect of immunotherapy is not ascertained. Hemispheric surgery is an efficacious therapy for epilepsy, despite focal and cognitive sequelae. Curative treatment and ideal timing for surgery are still not defined. Our study aim is to describe a pediatric case-series with RS evaluated in a Reference Center for Refractory Epilepsy and compare our results with the available literature. Methods: Review of clinical, neurophysiological, radiological, therapeutics and prognosis characteristics of pediatric patients evaluated in our center with RS since 2006. Results: Eight children were included (median age of onset 8.0 years), seven with left hemisphere dysfunction. Up until surgery/present moment, four presented epilepsia partialis continua, six had focal deficits and four had cognitive decline. Most patients revealed unilateral atrophy and T2/FLAIR hyperintensity with frontal predominance in the last magnetic resonance imaging (MRI). In the last video-EEG, all patients had ipsilateral interictal epileptiform activity and one had contralateral epileptiform activity as well. PET/SPECT was used in three patients and functional MRI in one patient for language lateralization. Four were submitted to surgery (three hemispherectomies, one frontal resection), having all previously done immunoglobulin, three corticosteroids and two tacrolimus. In the first year post-surgery, two patients were seizure-free, with the others with a significant improvement of their seizures; the patient submitted to frontal resection did not present surgical sequelae, with the remaining having motor and cognitive sequelae (albeit one with language improvement). Among patients not submitted to surgery, all maintain refractory epilepsy with at least four antiseizure drugs, with immunoglobulin been used in three patients, corticosteroids in two and tacrolimus in one. Conclusion: The management of RS is challenging regarding the balance between seizure control and the neurological surgical deficits. Our sample reflects the literature data, according to which hemispherectomy is the most effective therapy for seizure control, despite causing significant morbidity, rising questions about its timing.

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Section: Discussionunclassified

Rasmussen’s Syndrome: Case-Series Study from a Refractory Epilepsy Reference Center and Revision of Literature

Serôdio,

Pires,

Azurara

et al. 2024

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“…EEG and MRI are diagnostic in patients with Rasmussen's encephalitis often showing abnormal slow-wave discharges and delta activity with irregular spike and wave phenomenon, and extensive inflammation and white matter atrophy with ventricular enlargement consolidated to a single hemisphere of the brain [5]. Rasmussen's encephalitis is unique as these symptoms tend to be refractory to typically highly effective anti-epileptic medications, and an effective treatment option is usually surgical intervention via hemispherectomy, which has its own associated benefits, risks, and contraindications [2,7,11]. A study done following the clinical course of 10 atypical cases of Rasmussen's encephalitis showed hemispherectomy being effective in controlling seizures and motor and mental deterioration in over 80% of patients with partial recovery of neurological deficits [2,3,7,11].…”

Section: Discussionmentioning

confidence: 99%

“…These changes were highly suggestive of Rasmussen's encephalitis and had also caused lateral ventricle asymmetry leading to a leftward 8mm interventricular septum deviation due to diffuse volume loss. Our patient and family were informed of the results and instructed to follow-up with another children's hospital capable of providing a wider variety of treatment options including hemispherectomy as Rasmussen's encephalitis is a progressive disorder that is frequently refractory to medical therapy alone [2,7,11].…”

Section: Case Presentationmentioning

confidence: 99%

“…They presented the first case of what we now understand as Rasmussen's encephalitis. While the pathobiology of this disease process is still incompletely understood, some proposed hypotheses to explain its etiology include T-cell mediated cytotoxicity, antibody-mediated central nervous system (CNS) inflammation, and cellular anomalies related to aberrant gene expression [1][2][3][4][5][6][7][8][9][10][11]. Classically considered a childhood disease with a median age of onset of six years, it consists of three stages in the presentation: a prodromal phase that could last a few years and includes isolated seizures and mild hemiplegia, an acute phase that follows with simple partial seizures and often epilepsa partialis continua (EPC) mainly arising from a single hemisphere, and lastly a residual phase with often pharmaco-refractory epilepsy as well as permanent motor and cognitive deficits [4].…”

Section: Introductionmentioning

confidence: 99%

“…MRI often shows marked atrophy as well as increased signal intensity from the affected unilateral hemisphere [5]. Treatment of this neurological malady often involves a combination of immunotherapy, antiepileptic medications, and then hemispherectomy in cases of treatment-resistant epilepsy [2,7,11].…”

Section: Introductionmentioning

confidence: 99%

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Atypical Presentation of Cerebral Palsy and Seizures: A Case Report on Rasmussen’s Encephalitis in an Adolescent

Noordin

Deyo

Ryon

et al. 2021

Cureus

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Rasmussen's encephalitis is a rare neurological disease first described in 1958 that is characterized by medico-refractory seizures, focal unilateral cerebral inflammation, and deficits such as hemiparesis. While we still do not have a full understanding of this disease, proposed theories behind its etiology include autoimmune manifestations, immune attack by T cells, and malfunctional alterations in genetic expression. It is classically considered a rare childhood malady with a median age of onset of six years, and cases in adolescents and adults are even rarer, representing up to 10% of all cases to date. In this report, we would like to share a rare case of Rasmussen's encephalitis that occurred in an adolescent. Our 17-year-old male patient presented with signs and symptoms beginning at age 14 and was initially diagnosed with cerebral palsy only to later present with additional symptoms and characteristic EEG and MRI findings that ultimately led to a diagnosis of Rasmussen's encephalitis. Thus, with this case report, our intent is twofold: to shed light on an atypical presentation of an already rare disease, even rarer in adolescents and adults, and to underscore the importance of keeping a broad differential when it comes to evaluating a patient with seizures.

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Rasmussen’s encephalitis: mechanisms update and potential therapy target

Tang

Luan

2020

Therapeutic Advances in Chronic Disease

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Rasmussen’s encephalitis (RE) is rare neurological diseases characterized as epilepsia partialis continua, invariably hemiparesis, and cognitive impairment. This disease is encountered frequently in childhood and presents with progressive atrophy of the unilateral hemisphere, and there are also sustained neurological complications. Owing to uncertain pathogenesis, the most effective way to limit the influence of seizures currently is cerebral hemispherectomy. In this review, we focus on four main lines of pathogenesis: virus infection, antibody-mediated, cell-mediated immunity, and microglia activation. Although one or more antigenic epitopes may give rise to infiltrating T cell responses in RE brain tissue, no exact antigen was confirmed as the definite cause of the disease. On the other hand, the appearance of antibodies related with RE seem to be a secondary pathological process. Synthetic studies have suggested an adaptive immune mechanism mediated by CD8+ T cells and an innate immune mechanism mediated by activated microglia and neuroglia. Accordingly, opinions have been raised that immunomodulatory treatments aimed at initial damage to the brain that are induced by cytotoxic CD8+ T cell lymphocytes and microglia in the early stage of RE slow down disease progression. However, systematic exploration of the theory behind these therapeutic effects based on multicenter and large sample studies are needed. In addition, dysfunction of the adenosine system, including the main adenosine removing enzyme adenosine kinase and adenosine receptors, has been demonstrated in RE, which might provide a novel therapeutic target for treatment of RE in future.

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Functional hemispherectomy for adult rasmussen encephalitis: a case report and literature review

Abstract: In an adult patient with RE, a favorable prognosis was achieved after functional hemispherectomy. The safety and efficacy of functional hemispherectomy in patients with RE is highlighted.

Cited by 6 publications

References 8 publications

Rasmussen’s Syndrome: Case-Series Study from a Refractory Epilepsy Reference Center and Revision of Literature

Rasmussen’s Syndrome: Case-Series Study from a Refractory Epilepsy Reference Center and Revision of Literature

Atypical Presentation of Cerebral Palsy and Seizures: A Case Report on Rasmussen’s Encephalitis in an Adolescent

Rasmussen’s encephalitis: mechanisms update and potential therapy target

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