Pathogenesis, diagnosis and treatment of Rasmussen encephalitis: A European consensus statement

Bien, Christian G.; Granata, Tiziana; Antozzi, Carlo; Cross, JH; Dulac, Olivier; Kurthen, Martin; Lassmann, Hans; Mantegazza, Renato; Villemure, Jean‐Guy; Spreafico, Roberto; Elger, Christian E.

doi:10.1093/brain/awh415

Cited by 539 publications

(820 citation statements)

References 168 publications

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“…Additional spectratyping revealed the clonal expansion of a subset of CD8 + T cells. Further, an accumulation of immunoglobulins and of C3 and C1q within the vessel wall can be found [2,7]. In Rasmussen encephalitis, MRI findings evolve with time.…”

Section: Discussionmentioning

confidence: 99%

Overlap between linear scleroderma, progressive facial hemiatrophy and immune-inflammatory encephalitis in a paediatric cohort

Somer¹,

Morren

Müller

et al. 2015

Eur J Pediatr

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Linear scleroderma en coup the sabre (LSCS), progressive facial hemiatrophy (PFH) and autoimmune encephalitis are distinct clinical entities, although patients with overlapping features have been reported. We performed a multicenter retrospective review of a series of children with LSCS and/or PFH to explore the relation between these entities. The files of 16 children were reviewed, 11 presented with LSCS, 5 with PFH, with time overlapping cutaneous features were seen. Extracutaneous signs were found in both groups. ANA were present in more than 50 % of patients. Almost half of our patients presented with CNS manifestations comprising unilateral headache, migraine and epilepsy with or without abnormalities on MRI. Brain biopsy in one patient was consistent with Rasmussen encephalitis. In two other children, associated autoimmune manifestations were present.Conclusion: Our patient cohort brings more arguments to consider LSCS and PFH as a single disease entity with LSCS and superficial skin involvement at one end of the spectrum and PFH with involvement of subcutaneous deep tissue at the other end. In both entities, encephalitis can be observed. Our findings of circulating ANA, intradermal lymphocytes and IgG, intrathecal IgG production and clinical improvement with immunosuppressive therapy endorse the concept of a possible common immune-inflammatory pathogenesis.

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Section: Discussionmentioning

confidence: 99%

Overlap between linear scleroderma, progressive facial hemiatrophy and immune-inflammatory encephalitis in a paediatric cohort

Somer¹,

Morren

Müller

et al. 2015

Eur J Pediatr

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“…The clinical diagnosis was made according to the European diagnostic criteria (Bien et al, 2005). The control group consisted of 20 patients who underwent surgical treatment for temporal lobe epilepsy, generally matched with the RE patient group for age at the time of surgery and seizure onset.…”

Section: Methodsmentioning

confidence: 99%

Expression of human cytomegalovirus components in the brain tissues of patients with Rasmussen’s encephalitis

et al. 2017

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Rasmussen's encephalitis (RE) is a rare and severe progressive epileptic syndrome with unknown etiology. Infection by viruses, including human cytomegalovirus (HCMV), has been speculated to be a potential trigger for RE. However, no viral antigens have been detected in the brains of patients with RE; thus, a possible clinical linkage between viral infections and RE has not been firmly established. In this study, we evaluated the expression of HCMV pp65 antigen in brain sections from 26 patients with RE and 20 non-RE patients by immunohistochemistry and in situ hybridization, and assessed the associations between HCMV infection and clinical parameters. Elevated expression of HCMV pp65 protein and DNA was observed in 88.5% (23/26) and 69.2% (18/26) of RE cases, respectively. In the non-RE group, HCMV pp65 antigen was detected only in two cases (10%), both of which were negative for DNA staining. Additionally, the intensity of HCMV pp65 staining was correlated with a shorter duration of the prodromal stage, younger age of seizure onset, and more severe unilateral cortical atrophy. Elevated expression of HCMV pp65 was observed in RE brain tissue and was correlated with the clinical features of RE disease. In summary, our results suggested that HCMV infection may be involved in the occurrence and progression of RE disease. Thus, further studies are needed to determine whether early treatment with anti-HCMV antibodies could modulate the course of RE.

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“…Glutamat reseptörü subünitesi 3'e karşı oluşan antikor (anti-Glu3R) saptanabilir, ancak bu hastalık için spesifik değildir. Bu ağır tabloya yönelik en etkili müdahalenin hemisferektomi ya da hemisferotomi olduğu bildirilse de [10,11] özellikle motor fonksiyonların henüz bozulmadığı hastaların başta IVIG ve plazmaferez olmak üzere steroid ya da takrolimus gibi immün tedavi seçeneklerinden faydalandığı gösterilmiştir. [11][12][13] …”

Section: Rasmussen Ensefalitiunclassified

Immunotherapy Responsive Epilepsies: General Clinical Features, Commonly Detected Antibodies and Treatment Studies

Eki̇zoğlu¹

2015

Epilepsi

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SummaryThe role of immunity and inflammation in epilepsy has long been considered and in recent years, discovery of some antibodies against to various neuronal antigens especially in cases refractory to conventional anti-epileptic drugs raised the concept of "autoimmune epilepsy". Immunotherapy may lead to significant benefits in cases with suspicion of autoimmune etiology. In this paper, epileptic pictures with a known autoimmune aspect as well as those pictures with recent increased awareness due to the discovery of neuronal auto-antibodies responding to immunotherapies and their management options are summarized.Keywords: Epilepsy; immunotherapy; autoantibodies; autoimmunity. ÖzetEpilepside immünite ve enflamasyonun rolü üzerinde uzun zamandır durulmakta olup, son yıllarda özellikle geleneksel antiepileptik ilaçlara dirençli olgularda birtakım nöronal antijenlere karşı gelişen antikorların saptanması ile "otoimmün epilepsi" kavramı doğmuştur. Otoimmün etiyolojiden şüphelenilen olgularda immün tedavinin başarılı olduğu görülmüştür. Bu yazıda otoimmün boyutu bilinen epilepsi tablolarının yanısıra, son yıllarda önemi artan immüntedaviye yanıtlı nöronal otoantikorlarla ilişkili tablolar ve tedavi seçenekleri özetlenmiştir.

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Pathogenesis, diagnosis and treatment of Rasmussen encephalitis: A European consensus statement

Cited by 539 publications

References 168 publications

Overlap between linear scleroderma, progressive facial hemiatrophy and immune-inflammatory encephalitis in a paediatric cohort

Overlap between linear scleroderma, progressive facial hemiatrophy and immune-inflammatory encephalitis in a paediatric cohort

Expression of human cytomegalovirus components in the brain tissues of patients with Rasmussen’s encephalitis

Immunotherapy Responsive Epilepsies: General Clinical Features, Commonly Detected Antibodies and Treatment Studies

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