Late‐onset Rasmussen's encephalitis and long‐term remission

Jutila, Leena; Immonen, Arto; Vanninen, Ritva; Mervaala, Esa; Pateau, Anders; Luostarinen, Liisa; Kälviäinen, Reetta

doi:10.1684/epd.2011.0412

Cited by 12 publications

(8 citation statements)

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“…A detailed discussion of these studies is beyond the scope of this paper and a near complete review may be found in Varadkar et al ( 68 ). As might be expected a priori given the clinical variability of RE, response to immune therapy has ranged from slowing of progression ( 11 , 59 – 61 , 69 ) to essentially curative ( 7 , 62 , 63 , 67 ). One fundamental concept of many immune-mediated encephalitides that appears to be true in RE as well is the benefit of early therapy ( 58 , 69 , 70 ).…”

Section: Discussionmentioning

confidence: 98%

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

2017

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Rasmussen’s encephalitis (RE) is a rare neurologic disorder characterized by progressive cerebral hemiatrophy and medically refractory epilepsy. The majority of current literature on this topic is focused on the pediatric population. In this case series, we will review three cases of adult-onset RE, as defined by fulfillment of the 2005 Bien criteria. The diagnostic challenge of characterizing this rare disease will be highlighted by the extensive serum, CSF, and pathologic sampling in all three patients. MR imaging and EEG data will be examined over time to characterize hallmark findings as well as progression. In addition, we will review the various forms of therapy attempted in these three patients, namely anti-epileptic drug therapy and immunomodulatory therapy. We will also utilize this case series to critically evaluate the broader context of atypical presentations of this disease and the value of current diagnostic criteria.

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Section: Discussionmentioning

confidence: 98%

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

2017

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“…[103–106] Patients develop frequent, unilateral motor seizures that evolve to intractable epilepsia partialis continua, hemiplegia, and cognitive decline, accompanied by progressive unilateral hemispheric atrophy. [107–109] Younger patients are more likely to develop more severe disease and a higher degree of hemispheric atrophy.…”

Section: Introductionmentioning

confidence: 99%

Seizures and risk of epilepsy in autoimmune and other inflammatory encephalitis

Spatola

Dalmau

2017

Current Opinion in Neurology

148

137

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Purpose of review To assess the seizure manifestations and risk of epilepsy in encephalitis associated to antibodies against neuronal cell-surface (AE) or myelin-associated antigens, and to review several chronic epileptic disorders including, Rasmussen’s encephalitis (RE), fever-induced refractory epileptic syndromes (FIRES), and new-onset refractory status epilepticus (NORSE). Recent findings Seizures are a frequent manifestation of AE. Some AE may associate with characteristic features: faciobrachial dystonic seizures (anti-LGI1 encephalitis), EEG extreme delta brush (anti-NMDAR), or multifocal FLAIR-MRI abnormalities (anti-GABAAR). In anti-LGI1 encephalitis, cortical, limbic, and basal ganglia dysfunction results in different types of seizures. AE or myelin-antibody associated syndromes are often immunotherapy-responsive and appear to have a low risk for chronic epilepsy. In contrast patients with seizures related to GAD65-antibodies (an intracellular antigen) frequently develop epilepsy and have suboptimal response to treatment (including surgery). RE or FIRES may occur with autoantibodies of unclear significance and rarely respond to immunotherapy. A study of patients with NORSE showed that 30% developed chronic epilepsy. Summary Although seizures are frequent in all types of AE, the risk for chronic epilepsy is dependent on the antigen: lower if located on the cell-surface, and higher if intracellular. For other disorders (RE, FIRES, NORSE) the prognosis remains poor.

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“…The patient remained seizure-free for three years (time of publication) with AED combined treatment, oral prednisone and monthly pulses of IVIG. 16 Poloni et al reported the case of a 29-year-old patient with RE, who meets the diagnostic criteria of Bien et al, with polymorphic crises (focal, motor and sensory), with infection screening tests negative, who improved with courses of methylprednisolone. However, three years later, he presented hemiparesis, cognitive symptoms, and depression, and a CSF examination suggestive of CMV infection, which when treated with high-dose intravenous polyvalent immunoglobulins (HDIV), achieved remarkable improvement.…”

Section: Discussionmentioning

confidence: 99%

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

Navarro-Flores¹,

Villarrealinos²

2020

Sinapse

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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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Late‐onset Rasmussen's encephalitis and long‐term remission

Cited by 12 publications

References 10 publications

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

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

Seizures and risk of epilepsy in autoimmune and other inflammatory encephalitis

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

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