A Case Series of Adult-Onset Rasmussen’s Encephalitis: Diagnostic and Therapeutic Challenges

Castellano, James F.; Meyer, Jenny A.; Lado, Fred A.

doi:10.3389/fneur.2017.00564

Cited by 20 publications

(23 citation statements)

References 74 publications

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“…The pathognomonic radiological images are not always presented in the adult as described in the child, these could even be bilateral or accompanied by cortical dysplasia and the time of appearance in the MRI is variable. 14 As in the case of our patient, the delayed of the appearance of the image last around two years; as well as the hemispheric atrophy progressed slowly.…”

Section: Discussionsupporting

confidence: 68%

Adult-Onset Rasmussen’s Encephalitis: Response after Steroid Pulses

Navarro-Flores¹,

Villarrealinos²

2020

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Introduction: Rasmussen's encephalitis (RE) is an infrequent chronic neurological disorder characterized by unihemispheric inflammation of the brain cortex, refractory epilepsy, and progressive motor and cognitive decline. The presentation in adulthood is uncommon and represents around 10% of the cases.Case Report: A 54-year-old woman developed focal myoclonic seizures involving the right face and upper limb. The seizures were recurrent and progressed to Epilepsia partialis continua. Gradually, she developed cognitive impairment. The magnetic resonance imaging showed left unihemispheric frontoparietal moderate atrophy compared to the baseline magnetic resonance imaging. Rasmussen's encephalitis was diagnosed according to the Bien criteria. The antiepileptic drug combination treatment partially controlled the seizures. After pulses of methylprednisolone, the patient was discharged seizure-free.Conclusion: Rasmussen's encephalitis is rare in adults; however, it should be considered as a differential diagnosis in patients with a history of pharmacoresistant focal seizures with progressive involvement of higher functions and imaging studies showing cerebral hemiatrophy. Current pharmacological treatment for this condition does not include the use of steroids. Nevertheless, in this case, methylprednisolone represented a treatment option in a limited-resource context. ResumoIntrodução: A encefalite de Rasmussen (ER) é uma doença neurológica crónica pouco frequente, caracterizada por inflamação unihemisférica do córtex cerebral, epilepsia refratária e declínio motor e cognitivo progressivo. A apresentação na idade adulta é incomum e representa cerca de 10% dos casos.Caso Clínico: Uma mulher de 54 anos desenvolveu convulsões mioclónicas focais envolvendo a face direita e o membro superior. As crises foram recorrentes e evoluíram para epilepsia partialis continua. Gradualmente, a doente desenvolveu comprometimento cognitivo. A ressonância magnética mostrou atrofia frontoparietal unihemisférica moderada em comparação com a ressonância magnética basal. A encefalite de Rasmussen foi diagnosticada de acordo com os critérios de Bien. O tratamento combinado com fármacos antiepilépticos controlou parcialmente as Informações/Informations:

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Section: Discussionsupporting

confidence: 68%

Adult-Onset Rasmussen’s Encephalitis: Response after Steroid Pulses

Navarro-Flores¹,

Villarrealinos²

2020

Sinapse

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“…There was no prodromal phase, and he had already seemingly entered the acute phase with worsening deficits albeit no seizures. Studies suggest that these phases may last a longer period in adults than pediatric patients [2].…”

Section: Discussionmentioning

confidence: 99%

“…Semiology in adult-onset cases can be more characteristic of temporal lobe epilepsy. Perisylvian and fronto-insular regions are the usual sites of involvement, leading to hemiparesis and hemianopia [1][2][3][4]. Hemiathetosis and hemidystonia are rarely described.…”

Section: Discussionmentioning

confidence: 99%

“…Rasmussen's encephalitis (RE) is a rare immune-mediated condition that classically presents in children with focal epilepsy or epilepsia partialis continua, progressive hemiplegia associated with cognitive deterioration and chronic unilateral cortical inflammation and hemiatrophy [1]. Adults may have atypical manifestations including a prolonged prodromal phase, poorly defined residual period and slower progression [2]. Adults have more frequent occipital lobe involvement, bilateral hemispheric involvement or presentation as temporal lobe epilepsy or movement disorders [3].…”

Section: Introductionmentioning

confidence: 99%

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Rasmussen’s encephalitis presenting as progressive parietal dysfunction sans seizures

Garg

Agarwal

Dash

et al. 2019

Seizure

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“…Among biologic agents, the anti-CD20 antibody rituximab has been used in a few published case series and reports [92,93], for its efficacy in the reduction of the B cell activation and, therefore, of the antibody levels. As previously discussed, the reported efficacy is variable [56,94], as the drug does not adequately target the T cells.…”

Section: Biologic Drugsmentioning

confidence: 99%

Rasmussen's encephalitis: From immune pathogenesis towards targeted-therapy

Orsini

Foiadelli

Carli

et al. 2020

Seizure

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Rasmussen encephalitis (RE) is a unilateral hemispheric encephalitis whose main clinical features include refractory focal epilepsy or epilepsia partialis continua, hemiparesis, and progressive cognitive decline. Despite the autoimmune pathogenesis of RE, the only definitive therapeutic option is currently represented by surgery.We review the clinical features, the immune pathogenesis, and the available therapeutic options for RE, with special focus on immunosuppressive agents. The research includes systematic reviews, meta-analyses, observational studies, clinical trials, cases series and reports, until 2020.The use of immunosuppressive agents in RE is supported by the evidence of an autoimmune involvement of the central nervous system in this condition. Although often insufficient to modify the disease course and to achieve symptomatic control, immune therapy can be effective in patients with slow disease progression or in patients in which surgery is not applicable. Moreover, the documentation of T-cell involvement in the pathogenesis of RE, with a specific cytokine pattern, opens a window of opportunity for the use of T-targeted therapies and biologic drugs (i.e. anti-TNFα agents) in the treatment of this disease.

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A Case Series of Adult-Onset Rasmussen’s Encephalitis: Diagnostic and Therapeutic Challenges

Cited by 20 publications

References 74 publications

Adult-Onset Rasmussen’s Encephalitis: Response after Steroid Pulses

Adult-Onset Rasmussen’s Encephalitis: Response after Steroid Pulses

Rasmussen’s encephalitis presenting as progressive parietal dysfunction sans seizures

Rasmussen's encephalitis: From immune pathogenesis towards targeted-therapy

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