Rasmussen脑炎

Rasmussen encephalitis (RE) is a rare but severe immune-mediated brain disorder leading to unilateral hemispheric atrophy, associated progressive neurological dysfunction and …

Rasmussen's encephalitis (RE) is a severe, rare, chronic inflammatory brain disease resulting in drug-resistant epilepsy and progressive destruction of one hemisphere with loss of neurological function. RE is associated with a deterioration of background electroencephalography (EEG) activity, a progressive atrophy on magnetic resonance imaging (MRI) imaging and an extensive positron emission tomography hypometabolism over the affected hemisphere. RE is an immune-mediated disease, with a predominant role of CD8+ T cytotoxic cells, microglial cells, and activation of inflammasome pathway. The diagnosis of RE is based on clinical (intractable epilepsy and neurological deterioration), electrophysiological (unilateral EEG slowing) and MRI (hemiatrophy) criteria. Antiseizure medications are generally unable to stop seizures. The most effective procedure is hemispherotomy (surgical disconnection of one cerebral hemisphere), but this is associated with permanent motor and neurological deficits. Treatments targeting the immune system are recommended especially in the early stages of the disease or in patients with slow disease progression and mild deficits and/or not eligible for surgery. Based on the pathophysiology, several immunotherapies have been tried in RE (none exhaustively: corticosteroid, intravenous immunoglobulins, tacrolimus, azathioprine, adalimumab, mycophenolate mofetil, natalizumab). However, only small cohorts have been reported without comparative study. In this review, we will summarise some pathophysiological mechanisms of RE, before reporting the literature data concerning immunotherapies. We then discuss the limitations of these studies and the prospects for further research.

Rasmussen encephalitis (RE) is a progressive and destructive inflammatory disease of one hemisphere. Its cause is unknown. We investigated comorbidity and laterality factors that might predispose to RE.

Rasmussen encephalitis (RE) is a rare neuroinflammatory disease characterized by intractable seizures and progressive brain atrophy that is usually confined to one cerebral hemisphere. Disease management involves anti-seizure medications and immunotherapy, although surgical resection remains the only effective treatment option to achieve seizure freedom. The presence of clonally expanded resident memory T cells in brain tissue removed to control seizures suggests the involvement of an autoimmune response in the etiology of the disease. Blocks of fresh brain tissue were obtained from three RE surgery cases (ages 5, 8, and 26 years at the time of surgery) and immune cells were isolated. Single cell RNA sequencing was used to define the types of immune cells present in the affected brain tissue and potential crosstalk between them, along with multiplex immunofluorescence immunostaining of sections from the same specimens. We matched T cell receptor sequences to T cell phenotypes and used ViralTrack software to search for evidence of activation of latent viruses in the immune cells. The immune cells isolated from the three RE cases comprised primarily activated microglia and resident memory CD8 T cells with fewer CD4 T cells, NK cells and monocyte-derived macrophages and dendritic cells. The majority of CD8 T cells expressed killer cell lectin-like receptors, and a virus responsive gene signature that included XCL1, TNFRSF9 and CRTAM, but also the exhaustion markers LAG-3 and TIM-3. Microglia expressed transcripts found in disease-associated microglia and transcripts associated with NLRP3 inflammasomes. We found no evidence for active latent viruses; however, we found endogenous HERV-K retrovirus sequences that were transcribed from multiple provirus insertion sites.

Rasmussen encephalitis (RE) is a rare progressive disorder causing drug‐resistant epilepsy. Hemispheric surgery is an established treatment, but comprehensive data on postoperative seizure, motor, and cognitive outcomes are limited. We aimed to evaluate these outcomes and identify associated prognostic factors.

Abstract Rasmussen et al. described a syndrome characterized by intractable focal seizures in childhood, leading to focal motor seizures, progressive hemiparesis, and cognitive impairment. Rasmussen's encephalitis (RE) is now defined as an autoimmune disorder that determines progressive multifocal encephalopathy associated to unilateral cortical atrophy and progressive deterioration. Histologically, RE is characterized by an inflammatory lesion compromising the gray and white matters, with frequent lymphocytes arising in sanguineous vessels and respective perivascular tissues, glial nodules, hydropic degeneration, and atrophy. Currently, conventional antiepileptic therapy drugs remain unhelpful in treating the seizures, but the process appeared to be halted by hemispherectomy. We herein report three cases of RE treated by surgical resection and discuss the pathogenesis and diagnostic histopathological findings of this uncommon process.

Rasmussen encephalitis (RE), also called Rasmussen syndrome, is a rare, progressive, chronic encephalitis affecting one hemisphere of the brain. It occurs mainly in children. However, around 10% of all cases are adolescents and adults. RE occurs usually in healthy individuals. It is estimated that no more than two new cases per year are identified in large epilepsy centers. Rasmussen’s encephalitis is characterized by intractable focal seizures, often in the form of epilepsia partialis continua (EPC) with motor and cognitive deterioration. Neuroimaging shows the progressive damage of the affected hemisphere, and histopathology is consistent with a T-cell dominated encephalitis with activated microglial cells and reactive astrogliosis. Cerebral hemispherectomy remains the only cure for seizures, but there are inevitable functional compromises. We report the rare case of an 8-year-old girl without any clinical history presented with recent left hemiparesis and seizures, and whose investigations based on the brain MRI and Electroencephalogram data, conducted to the diagnosis of Rasmussen’s encephalitis. Our management was exclusively medical, with a good outcome.

Rasmussen encephalitis (RE) is a rare epileptic disorder that is characterized by the presence of unihemispheric seizures coinciding with inflammation. The disease mostly presents in children. Clinically, patients often reach a residual stage with drug resistant seizures and severe neurological deficits. Pathologically, at this stage, the brain shows variable neuronal loss. The etiology is unknown but the infiltration of large numbers of CD8 positive T lymphocytes suggests that this is an autoimmune disease. Treatment consists of anti-inflammatory therapy (IVIG or tacrolimus), which, however, does not reduce the drug-resistant seizures. Therefore, hemisperectomy is the most effective treatment of RE.

Rasmussen’s encephalitis usually presents in childhood. The clinical picture in Rasmussen’s disease is protean, and the findings from direct brain imaging help to explain the variations seen in this syndrome. The role and involvement of the basal ganglia in this condition is clarified by comparing the changes seen on computerised tomography and magnetic resonance imaging with the clinical details of 21 patients described in this retrospective study.

Rasmussen encephalitis (RE) is a severe pediatric inflammatory brain disease characterized by unilateral inflammation and atrophy of the cerebral cortex, drug‐resistant focal epilepsy and progressive neurological and cognitive deterioration. The etiology and pathogenesis of RE remain unclear. Our previous results demonstrated that the adenosine A1 receptor (A1R) and the major adenosine‐removing enzyme adenosine kinase play an important role in the etiology of RE. Because the downstream pathways of inhibitory A1R signaling are modulated by stimulatory A2AR signaling, which by itself controls neuro‐inflammation, glial activation and glial glutamate homeostasis through interaction with glutamate transporter GLT‐1, we hypothesized that maladaptive changes in adenosine A2A receptor (A2AR) expression are associated with RE. We used immunohistochemistry and Western blot analysis to examine the expression of A2ARs, glutamate transporter‐I (GLT‐1) and the apoptotic marker Bcl‐2 in surgically resected cortical specimens from RE patients (n = 18) in comparison with control cortical tissue. In lesions of the RE specimen we found upregulation of A2ARs, downregulation of GLT‐1 and increased apoptosis of both neurons and astroglia. Double staining revealed colocalization of A2ARs and Bcl‐2 in RE lesions. These results suggest that maladaptive changes in A2AR expression are associated with a decrease in GLT‐I expression as a possible precipitator for apoptotic cell loss in RE. Because A2AR antagonists are already under clinical evaluation for Parkinson's disease, the A2AR might likewise be a tractable target for the treatment of RE.

We studied the clinical, electrophysiological, imaging and pathological features of 18 patients with Rasmussen’s encephalitis (RE). This descriptive study included 18 patients (six males…

BACKGROUND: Rasmussen encephalitis (RE) is a rare inflammatory disease affecting one hemisphere, causing progressive neurological deficits and intractable seizures. OBJECTIVE: To report long-term seizure outcomes, reoperations, and functional outcomes in patients with RE who underwent hemispherectomy at our institution. METHODS: Retrospective review was performed for all patients with RE who had surgery between 1998 and 2020. We collected seizure history, postoperative outcomes, and functional data. Imaging was independently reviewed in a blinded fashion by 2 neurosurgeons and a neuroradiologist. RESULTS: We analyzed 30 patients with RE who underwent 35 hemispherectomies (5 reoperations). Using Kaplan-Meier analysis, seizure-freedom rate was 81.5%, 63.6%, and 55.6% at 1, 5, and 10 years after surgery, respectively. Patients with shorter duration of hemiparesis preoperatively were less likely to be seizure-free at follow-up (P = .011) and more likely to undergo reoperation (P = .004). Shorter duration of epilepsy (P = .026) and preoperative bilateral MRI abnormalities (P = .011) were associated with increased risk of reoperation. Complete disconnection of diseased hemisphere on postoperative MRI after the first operation improved seizure-freedom (P = .021) and resulted in fewer reoperations (P = .034), and reoperation resulted in seizure freedom in every case. CONCLUSION: Obtaining complete disconnection is critical for favorable seizure outcomes from hemispherectomy, and neurosurgeons should have a low threshold to reoperate in patients with RE with recurrent seizures. Rapid progression of motor deficits and bilateral MRI abnormalities may indicate a subpopulation of patients with RE with increased risk of needing reoperation. Overall, we believe that hemispherectomy is a curative surgery for the majority of patients with RE, with excellent long-term seizure outcome.

Background and Purpose. Rasmussen's encephalitis (RE) is a rare condition of unknown cause characterized by intractable seizures, progressive hemiparesis, mental impairment, and …

… Rasmussen’s encephalitis is a rare clinical syndrome of chronic encephalitis characterized by refractory partial seizures, epilepsia partialis … Rasmussen encephalitis in childhood …

Rasmussen encephalitis (RE) is a rare, chronic, idiopathic, progressive, inflammatory, neurodegenerative disease process and typically seen in pediatric cohort. Although primarily a disease affecting children, adult cases with RE have also been reported. It manifests as drug refractory epilepsia partialis continua (EPC). Immunomodulation, although delays progression of disease, seldom influences outcome. Imaging is crucial for early diagnosis, and monitoring disease progression. Magnetic resonance imaging (MRI) is mainstay of imaging with nuclear imaging being a complimentary tool for diagnosing RE. Typical imaging features of RE on MRI are hemispherical atrophy, caudate nucleus atrophy, ex vacuo dilatation of the ventricular system and sulci. We review 5 cases of RE who fulfilled diagnostic criteria proposed by Bien et al. in 2005. One patient had typical imaging pattern of RE while other four patients had atypical imaging features of RE on PET-MRI.

Rasmussen's encephalitis is a rare syndrome characterized by intractable seizures, often associated with epilepsia partialis continua and symptoms of progressive hemispheric dysfunction. Seizures are usually the hallmark of presentation, but antiepileptic drug treatment fails in most patients and is ineffective against epilepsia partialis continua, which often requires surgical intervention. Co-occurrence of focal cortical dysplasia has only rarely been described and may have implications regarding pathophysiology and management. We describe a rare case of dual pathology of Rasmussen's encephalitis presenting as a focal cortical dysplasia (FCD) and discuss the literature on this topic.

Rasmussen encephalitis (RE) is a rare paediatric epilepsy with uni-hemispheric inflammation and progressive neurological deficits. To elucidate RE immunopathology, we applied T-cell receptor (TCR) sequencing to blood (n=23), cerebrospinal fluid (n=2) and brain biopsies (n=5) of RE patients, and paediatric controls. RE patients present with peripheral CD8+ T-cell expansion and its strength correlates with disease severity. In addition, RE is the only paediatric epilepsy with prominent T-cell expansions in the CNS. Consistently, common clones are shared between RE patients, who also share MHC-I alleles. Public RE clones share Vβ genes and length of the CDR3. Rituximab/natalizumab/basiliximab treatment does not change TCR diversity, stem cell transplantation replaces the TCR repertoire with minimal overlap between donor and recipient, as observed in individual cases. Our study supports the hypothesis of an antigen-specific attack of peripherally expanded CD8+ lymphocytes against CNS structures in RE, which might be ameliorated by restricting access to the CNS. Rasmussen Encephalitis is a rare neurological disease accompanied by inflammation and T cell infiltration in the brain. Here the authors show that the severity of this disease correlates with clonal CD8 T cell expansion.

Late-onset Rasmussen encephalitis (LoRE) is a rare unihemispheric progressive inflammatory disorder causing neurological deficits and epilepsy. The long-term radiological evolution has never been fully described. We retrospectively analyzed the MR images of 13 LoRE patients from a total of 136 studies, and searched for focal areas of volume loss or signal intensity abnormality in grey matter or white matter. Each subject had a median of nine MRI studies (IQR 7–13). Frontal and temporal lobes were the most affected regions (13/13 and 8/13, respectively) and showed the greatest worsening over time in terms of atrophic changes (9/13 and 5/8, respectively). A milder cortical atrophy was found in the insular and parietal lobes. The caudate nucleus was affected in seven patients. Hyperintensities of grey matter and white matter on T2-WI and FLAIR images were observed in all patients, and transiently in eight patients. In two cases out of the latter patients, these transient alterations evolved into atrophy of the same region. Disease duration was significantly associated with signal abnormalities in the grey matter at last follow-up. LoRE MRI alterations are milder, and their progression is markedly slower compared to radiological findings described in the childhood form.

… Rasmussen encephalitis is a disease consisting of chronic encephalitis with progressive … The etiology is unknown, but pathologic specimens revealed changes consistent with viral …

… Rasmussen encephalitis (RE) is a rare devastating disease of childhood causing progressive neurological deficits and intractable seizures, typically affecting one hemisphere. …

This study was designed to explore the feasibility of PET using [11C](R)-PK11195 as an in vivo marker of activated microglia/brain macrophages for the assessment of neuroinflammation in Rasmussen's encephalitis (RE). [11C](R)-PK11195 PET was carried out in four normal subjects, two patients with histologically confirmed RE, and three patients with clinically stable hippocampal sclerosis and low seizure frequency. Binding potential maps showing specific binding of [11C](R)-PK11195 were generated for each subject. Regional binding potential values were calculated for anatomically defined regions of interest after coregistration to and spatial transformation into the subjects' own MRI. In one patient with RE who underwent hemispherectomy, the resected, paraffin-embedded brain tissue was stained with an antibody (CR3/43) that labels activated human microglia. Whereas specific binding of [11C](R)-PK11195 in clinically stable hippocampal sclerosis was similar to that in normal brain, patients with RE showed a focal and diffuse increase in binding throughout the affected hemisphere. In RE, [11C](R)-PK11195 PET can reveal in vivo the characteristic, unilateral pattern known from postmortem neuropathologic study. PET imaging of activated microglia/brain macrophages offers a tool for investigation of a range of brain diseases where neuroinflammation is a component and in which conventional MRI does not unequivocally indicate an inflammatory tissue reaction. [11C](R)-PK11195 PET may help in the choice of appropriate biopsy sites and, further, may allow assessment of the efficacy of antiinflammatory disease-modifying treatment.

The glymphatic system (GS) facilitates perivascular clearance of interstitial solutes and is modulated in part by neuronal activity. However, its relationship to cortical excitability in epilepsy remains unclear. We aim to clarify the mechanistic link between GS function and cortical excitation–inhibition (E–I) balance in patients with epilepsy.

Background: Rasmussen encephalitis (RE) is a chronic, progressive encephalitis affecting one hemisphere of the brain. Intractable focal seizures, progressive neurological & cognitive decline and hemispheric atrophy are common clinical and radiological presentations of the disease. Objective: To see the clinical spectrum & short-term treatment outcome of Rasmussen Encephalitis. Method: It was a prospective interventional study, conducted at Department of Pediatric Neurology, Bangabandhu Sheikh Mujib Medical University, Dhaka from July 2022 to July 2023. Total 15 patients with Rasmussen encephalitis were evaluated after IV Methylprednisolone therapy at the doses of 20-30 mg/kg/day. Results: Among 15 patients, 8 (53.3%) were aged 5–10 years and 7 (46.7%) were <5 years; males predominated (11, 73.3%). All presented with seizures, hemiparesis, neuroregression, and cognitive impairment. Dysarthria was observed in 10 (66.7%) and facial nerve palsy in 4 (26.7%). Focal seizures were most common (7, 46.7%), followed by generalized tonic–clonic seizures (3, 20%). EEG showed unihemispheric slowing in 12 (80%) and generalized slowing in 3 (20%). Neuroimaging revealed unihemispheric insular–perisylvian atrophy with basal ganglia involvement in all cases. Following IV methylprednisolone, seizure frequency improved in 13 (86.7%) and EEG improved in 8 (53.3%). Conclusion: All patients with Rasmussen encephalitis presented with seizure, hemiparesis, neuroregression & cognitive impairment. IV Pulse methylprednisolone therapy were effective where seizure frequency reduced more than three-fourth cases & electroencephalographical improvement occured more than half of the cases of all Rasmussen encephalitis.

Abstract Rasmussen encephalitis (RE) is a rare neurologic disorder of childhood characterized by unihemispheric inflammation, progressive neurologic deficits, and intractable focal epilepsy. The pathogenesis of RE is still enigmatic. Adenosine is a key endogenous signaling molecule with anticonvulsive and anti-inflammatory effects, and our previous work demonstrated that dysfunction of the adenosine kinase (ADK)–adenosine system and astrogliosis are the hallmarks of epilepsy. We hypothesized that the epileptogenic mechanisms underlying RE are related to changes in ADK expression and that those changes might be associated with the development of epilepsy in RE patients. Immunohistochemistry was used to examine the expression of ADK and glial fibrillary acidic protein in surgically resected human epileptic cortical specimens from RE patients (n = 12) and compared with control cortical tissues (n = 6). Adenosine kinase expression using Western blot and enzymatic activity for ADK were assessed in RE versus control samples. Focal astrogliosis and marked expression of ADK were observed in the lesions of RE. Significantly greater ADK expression in RE versus controls was demonstrated by Western blot, and greater enzymatic activity for ADK was demonstrated using an enzyme-coupled bioluminescent assay. These results suggest that upregulation of ADK is a common pathologic hallmark of RE and that ADK might be a target in the treatment of epilepsy associated with RE.

Rasmussen encephalitis (RE) is a very rare chronic neurological disorder of unilateral inflammation of the cerebral cortex. Hemispherotomy provides the best chance at achieving seizure freedom in RE patients, but with significant risks and variable long-term outcomes. The goal of this study is to utilize our multicenter pediatric cohort to characterize if differences in pathology and/or imaging characterization of RE may provide a window into post-operative seizure outcomes, which in turn could guide decision-making for parents and healthcare providers. This multi-institutional retrospective review of medical record, imaging, and pathology samples was approved by each individual institution’s review board. Data was collected from all known pediatric cases of peri-insular functional hemispherotomy from the earliest available electronic medical records. Mean follow-up time was 4.9 years. Clinical outcomes were measured by last follow-up visit using both Engel and ILAE scoring systems. Relationships between categorical and continuous variables were analyzed with Pearson correlation values. Twenty-seven patients met study criteria. No statistically significant correlations existed between patient imaging and pathology data. Pathology stage, MRI brain imaging stages, and a combined assessment of pathology and imaging stages showed no statistically significant correlation to post-operative seizure freedom rates. Hemispherectomy Outcome Prediction Scale scoring demonstrated seizure freedom in only 71% of patients receiving a score of 1 and 36% of patients receiving a score of 2 which were substantially lower than predicted. Our analysis did not find evidence for either independent or combined analysis of imaging and pathology staging being predictive for post peri-insular hemispherotomy seizure outcomes, prompting the need for other biomarkers to be explored. Our data stands in contrast to the recently proposed Hemispherectomy Outcome Prediction Scale and does not externally validate this metric for an RE cohort.

Objective: To determine the relationship between the severity of pathology and seizure outcomes in patients who underwent hemispherectomy for Rasmussen Encephalitis, and to investigate which clinical factors correlated with severity of pathology. Methods: In this retrospective cohort study, we collected and reviewed pathology and clinical variables. We ascertained seizure outcomes using Engel’s classification, and Pardo stages were used to grade pathology. Results: We included 29 unique patients who underwent 34 hemispherectomy procedures for analysis. There was no statistically significant correlation between Pardo stage and seizure outcome (p = 1). Increasing duration of epilepsy (β = .011, p = .02) and duration of hemiparesis (β = .024, p = .01) were significantly associated with a more severe Pardo stage. In contrast, the presence of EPC had a negative relationship with Pardo stage (β = −.49, p = .04). 26 (89.75%) patients were Engel class I at last follow up, including all five patients who underwent redo hemispherectomy in our cohort. Conclusions: Consistent with the progressive nature of RE, more severe pathology was associated with a longer duration of epilepsy and longer duration of hemiparesis while the presence of EPC was associated with less severe pathology. Results from this series suggest the degree of cortical involvement with RE as assessed on surgical histopathology does not correlate with seizure outcome after hemispherectomy, which appears to be more dependent on surgical technique/complete disconnection.

Rasmussen encephalitis (RE), initially described half a century ago, is an inflammatory unihemispheric brain disorder. Its two clinical key facets are the progressive tissue and function …

Rasmussen encephalitis (RE) is a rare and progressive form of chronic encephalitis that typically affects one hemisphere of the brain and primarily occurs in pediatric individuals. The current study aims to narratively review the literature about RE, including historical information, pathophysiology, and management of this condition. RE often occurs in individuals with normal development, and it is estimated that only a few new cases are identified each year in epilepsy centers. Approximately 10% of cases also occur in adolescents and adults. The hallmark feature of RE is drug-resistant focal seizures that can manifest as epilepsia partialis continua. Also, patients with RE usually develop motor and cognitive impairment throughout the years. Neuroimaging studies show progressive damage to the affected hemisphere, while histopathological examination reveals T-cell-dominated encephalitis with activated microglial cells and reactive astrogliosis. The current therapy guidelines suggest cerebral hemispherotomy is the most recommended treatment for seizures in RE, although significant neurological dysfunction can occur. Another option is pharmacological management with antiseizure medications and immunomodulatory agents. No significant progress has been made in understanding the pathophysiology of this condition in the last decades, especially regarding genetics. Notably, RE diagnosis still depends on the criteria established by Bien et al., and the accuracy can be limited and include genetically different individuals, leading to unexpected responses to management.

… Rasmussen’s encephalitis constituted 1 per cent of all patients admitted for investigation of epilepsy, and in our material, 6.8 per cent of all resections for the treatment of epilepsy. Our …

OBJECTIVE Rasmussen encephalitis (RE) is a rare inflammatory neurological disorder typically involving one hemisphere and resulting in drug-resistant epilepsy and progressive neurological decline. Here, the authors present seizure outcomes in children who underwent epilepsy surgery for RE at a single institution. METHODS The records of consecutive patients who had undergone epilepsy surgery for RE at the UCLA Mattel Children's Hospital between 1982 and 2018 were retrospectively reviewed. Basic demographic information, seizure history, procedural notes, and postoperative seizure and functional outcome data were analyzed. RESULTS The cohort included 44 patients, 41 of whom had sufficient data for analysis. Seizure freedom was achieved in 68%, 48%, and 22% of the patients at 1, 5, and 10 years, respectively. The median time to the first seizure for those who experienced seizure recurrence after surgery was 39 weeks (IQR 11-355 weeks). Anatomical hemispherectomy, as compared to functional hemispherectomy, was independently associated with a longer time to postoperative seizure recurrence (HR 0.078, p = 0.03). There was no statistically significant difference in postoperative seizure recurrence between patients with complete hemispherectomy and those who had less-than-hemispheric surgery. Following surgery, 68% of the patients could ambulate and 84% could speak regardless of operative intervention. CONCLUSIONS A large proportion of RE patients will have seizure relapse after surgery, though patients with anatomical hemispherectomies may have a longer time to postoperative seizure recurrence. Overall, the long-term data in this study suggest that hemispheric surgery can be seen as palliative treatment for seizures rather than a cure for RE.

… We describe a patient with adult-onset Rasmussen's encephalitis (RE) responsive to vagus nerve stimulation. This previously healthy woman developed RE in the right hemisphere at …

… body with increasing frequency suggests Rasmussen's syndrome. The early EEG studies demonstrate persistent temporal delta activity which is unusual for the partial epilepsy [2]. Multi…

Rasmussen encephalitis (RE) is a rare pediatric neuroinflammatory disease of unknown etiology characterized by intractable seizures, and progressive atrophy usually confined to one cerebral hemisphere. Surgical removal or disconnection of the affected cerebral hemisphere is currently the only intervention that effectively stops the seizures. Histopathological evaluation of resected brain tissue has shown that activated brain resident macrophages (microglia) and infiltrating T cells are involved in the inflammatory reaction. Here, we report that T cells isolated from seven RE brain surgery specimens express the resident memory T cell (TRM) marker CD103. CD103 was expressed by >50% of CD8+ αβ T cells and γδ T cells irrespective of the length of time from seizure onset to surgery, which ranged from 0.3 to 8.4 years. Only ~10% of CD4+ αβ were CD103+, which was consistent with the observation that few CD4+ T cells are found in RE brain parenchyma. Clusters of T cells in brain parenchyma, which are a characteristic of RE histopathology, stained for CD103. Less than 10% of T cells isolated from brain specimens from eight surgical cases of focal cortical dysplasia (FCD), a condition that is also characterized by intractable seizures, were CD103+. In contrast to the RE cases, the percent of CD103+ T cells increased with the length of time from seizure onset to surgery. In sections of brain tissue from the FCD cases, T cells were predominantly found around blood vessels, and did not stain for CD103. The presence of significant numbers of TRM cells in RE brain irrespective of the length of time between clinical presentation and surgical intervention supports the conclusion that a cellular immune response to an as yet unidentified antigen(s) occurs at an early stage of the disease. Reactivated TRM cells may contribute to disease progression.

… Rasmussen's suggestion that there may be mild and nonprogressive forms of the disease. Chronic encephalitis and epilepsy (Rasmussen … with intractable focal epilepsy, epilepsia …

… Rasmussen encephalitis who achieved seizure control following right hemispherectomy. Prior to hemispherectomy, all four children had illogical thinking, loose associations, cohesive …

… Multimodal imaging was performed in Rasmussen Encephalitis (RE) during episodes of complex-partial and focal motor status epilepticus including independent component analysis of …

Purpose To characterise clinical features of Rasmussen encephalitis (RE) and identify factors associated with preoperative neurodevelopmental status in affected children. Methods …

… of Rasmussen encephalitis. Clinically, the progressive nature of the neurological deficits and subsequent neuroimaging … , the absence of typical bilateral neuroimaging changes, and the …

Rasmussen encephalitis is a rare chronic inflammatory neurological disorder of childhood characterized by progressive unilateral cerebral atrophy and typically associated with intractable focal seizures. We report a 6-year-old boy presenting with progressive right-sided weakness in the absence of clinically evident seizures. Brain MRI performed on a 1.5 T system demonstrated marked asymmetric atrophy of the left cerebral hemisphere, predominantly involving the insular and frontotemporal regions, with associated encephalomalacia, ex-vacuo ventricular dilatation, and ipsilateral Wallerian degeneration, without diffusion restriction or contrast enhancement. In the appropriate clinical context, these findings were suggestive of chronic Rasmussen encephalitis; however, given the absence of EEG and histopathological confirmation, this remains an imaging-based presumptive diagnosis. This case highlights an atypical presentation and emphasizes the importance of MRI in evaluating progressive focal neurological deficits even in the absence of seizures.

Rasmussen′s encephalitis (RE) is a chronic neurological disorder, characterised by unilateral inflammation of the cerebral cortex leading to progressive neurological and cognitive deterioration. Advances in neuroimaging suggest that progression of the inflammatory process seen with magnetic resonance imaging (MRI) might be a good biomarker in RE. We present here a case of 8-year-old male child presented with repeated episodes of drug resistant seizures leading to intellectual impairment and cognitive deterioration. Further MRI was done which shows diffuse left cerebral atrophy.

The authors investigated immunomodulatory treatments in 15 patients with Rasmussen encephalitis (RE) (14 with childhood and one with adolescent onset RE). Positive time-limited …

… Rasmussen encephalitis (RE) is a chronic inflammatory disease leading … However long-term immunotherapy seems to prevent or slow … Rasmussen encephalitis is a progressive disease …

Rasmussen encephalitis (RE) is a severe immune-mediated neurologic disease characterized by unihemispheric inflammation, progressive neurologic deficits, and intractable focal epilepsy. Functional hemispherectomy is considered to be the only effective intervention for this devastating disease.1,2

… Rasmussen encephalitis (RE) is characterized by unihemispheric inflammation, progressive … Immunotherapies may slow down tissue and function loss, whereas convincing effects on …

Rasmussen encephalitis (RE) is a rare but devastating unihemispheric brain disorder that often affects children.1 The clinical picture is characterized by intractable focal epilepsy and progressive decline of functions associated with the affected hemisphere.2 Despite its known inflammatory background and T-cell involvement, immunotherapy appears to slow rather than halt disease progression, and hemispherotomy appears to be the only solution for intractable epilepsy.1–4 A potential early therapeutic window has been suggested, and new therapeutic agents have become available.1 A monoclonal antibody targeting CD52 that leads to long-term depletion of lymphocytes (alemtuzumab) has previously been considered as a possible treatment option for RE, but clinical data are limited.1,5

INTRODUCTION: Late-onset Rasmussen encephalitis (LORE) is a rare, unihemispheric, progressive, inflammatory disorder causing severe neurological dysfunction and drug-resistant epilepsy with onset during late adolescence or adulthood. Due to the scarcity of available evidence, this study aims to improve its clinical characterization and summarize the distinctive features. DEVELOPMENT: Three illustrative cases are presented, including the clinical, neurophysiological, and neuroimaging work-up. Our findings are discussed with reference to previous evidence gathered through a comprehensive search. The reported patients presented adult onset within a wide age range. The initial clinical manifestation was variable, including refractory focal epilepsy, progressive hemiparesis, and epilepsia partialis continua, in line with previous findings. Progressive hemiatrophy with frontal or posterior predominance in MRI and extensive hypometabolism in functional neuroimaging were documented. Unihemispheric slow background activity and epileptiform discharges progressively developed during the long-term follow-up, as described in the literature. According to the European consensus diagnostic criteria, 2 patients met the Part A and one the Part B criteria. As reported in previous publications, slower neurological decline was observed with immunotherapy. CONCLUSIONS: Despite the wide range of clinical manifestations at onset, overall, LORE presents milder neurological deterioration and responds favorably to immunotherapy, which implies a better prognosis. Further studies are needed to establish the best strategy.

Rasmussen's encephalitis, a syndrome characteristically present-ing in children with the onset of partial motor seizures followed by progressive hemiparesis and cognitive impairment, and accompanied by unilateral cerebral atrophy, was described nearly 50 years ago, yet the cause and optimum treatment remain unclear. Although it was originally presumed to have a viral aetiology, the possible roles of antibody-mediated mechanisms and more recently cell-mediated immunity in its pathogenesis have come under increasing scrutiny in the last ten years. These developments are discussed, together with a review of the clinical features. The advances in treatment which have accompanied these changes are also assessed.

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.

Rasmussen's encephalitis (RE) is a rare disease of the central nervous system characterized by severe epileptic seizures, progressive degeneration of a single cerebral hemisphere, …

Abstract Objects To investigate the expression of human papillomavirus (HPV)-specific antigen in the brain tissue of patients with Rasmussen’s Encephalitis (RE) and its possible link to …

… Summary: Purpose: A limited number of cases of adult-onset Rasmussen’s encephalitis (A-RE) have been reported, but the features of the syndrome are still unclear. The aim of this …

… Rasmussen's encephalitis (RE) is a devastating disease characterized by progressive … The European consensus statement on the pathogenesis, diagnosis and treatment has recently …

… Rasmussen's encephalitis (RE) is a rare unilateral inflammatory brain disorder that causes … Unlike RE [3], T lymphocytes are not thought to play a significant pathogenic role in acute …

… , such as Rasmussen encephalitis. A definite involvement of … been demonstrated in Rasmussen encephalitis, although the … role of neuroinflammation as pathogenic factor, no definite …

… Rasmussen's encephalitis (RE) is a rare immunomediated disorder characterized by unilateral hemispheric atrophy, drug-resistant focal epilepsy, and progressive neurological deficits. …

Abstract Rasmussen’s encephalitis is an immune-mediated brain disorder characterised by progressive unilateral cerebral atrophy, neuroinflammation, drug-resistant seizures and cognitive decline. However, volumetric changes and epileptiform EEG activity were also observed in the contralateral hemisphere, raising questions about the aetiology of contralateral involvement. In this study, we aim to investigate alterations of white matter integrity, structural network topology and network efficiency in Rasmussen’s encephalitis using diffusion-tensor imaging. Fourteen individuals with Rasmussen’s encephalitis (11 female, median onset 6 years, range 4–22, median disease duration at MRI 5 years, range 0–42) and 20 healthy control subjects were included. All subjects underwent T1-weighted structural and diffusion-tensor imaging. Diffusion-tensor images were analysed using the fixel-based analysis framework included in the MRtrix3 toolbox. Fibre density and cross-section served as a quantitative measure for microstructural white matter integrity. T1-weighted structural images were processed using FreeSurfer, subcortical segmentations and cortical parcellations using the Desikan-Killiany atlas served as nodes in a structural network model, edge weights were determined based on streamline count between pairs of nodes and compared using network-based statistics. Global efficiency was used to quantify network integration on an intrahemispheric level. All metrics were compared cross-sectionally between individuals with Rasmussen’s encephalitis and healthy control subjects using sex and age as regressors and within the Rasmussen’s encephalitis group using linear regression including age at onset and disease duration as independent variables. Relative to healthy control subjects, individuals with Rasmussen’s encephalitis showed significantly (family-wise-error-corrected P < 0.05) lower fibre density and cross-section as well as edge weights in intrahemispheric connections within the ipsilesional hemisphere and in interhemispheric connections. Lower edge weights were noted in the contralesional hemisphere and in interhemispheric connections, with the latter being mainly affected within the first 2 years after disease onset. With longer disease duration, fibre density and cross-section significantly (uncorrected P < 0.01) decreased in both hemispheres. In the contralesional corticospinal tract, fibre density and cross-section significantly (uncorrected P < 0.01) increased with disease duration. Intrahemispheric edge weights (uncorrected P < 0.01) and global efficiency significantly increased with disease duration in both hemispheres (ipsilesional r = 0.74, P = 0.001; contralesional r = 0.67, P = 0.012). Early disease onset was significantly (uncorrected P < 0.01) negatively correlated with lower fibre density and cross-section bilaterally. Our results show that the disease process of Rasmussen’s encephalitis is not limited to the cortex of the lesioned hemisphere but should be regarded as a network disease affecting white matter across the entire brain and causing degenerative as well as compensatory changes on a network level.

Dual pathology has previously been reported in less than 10% of cases of Rasmussen's encephalitis (RE). Given the rarity of RE, it appears unlikely that dual pathology in RE is merely …

Rasmussen encephalitis (RE) is a rare form of severe unihemispheric epilepsy established to be an autoimmune disease. Here we demonstrate the presence of autoantibodies against …

… Background: Despite advances in our understanding of the autoimmune pathogenesis of RE… showed further changes typical of Rasmussen’s encephalitis, with inflammatory infiltrates …