Receptor Encephalitis Patients Research Articles

Risk Factors and Prognosis in Anti-NMDA Receptor Encephalitis Patients with Disturbance of Consciousness.

Disturbance of consciousness is common in patients with severe anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis. However, little is known about it. This study aimed to analyze the clinical manifestations and prognostic factors of anti-NMDAR encephalitis with disturbance of consciousness. In this retrospective study, the clinical features, treatment results, and long-term outcomes of anti-NMDAR encephalitis patients with disturbance of consciousness were analyzed, and multivariate logistic regression was used to analyze the factors affecting their prognosis. In the group with disturbance of consciousness, the incidences of seizures, involuntary movements, pulmonary infection, mechanical ventilation, intensive care unit (ICU) admission, neutrophil-lymphocyte ratio (NLR), abnormal cerebrospinal fluid index, plasma exchange, and immunosuppressive therapy were higher than those in the group without disturbance of consciousness (all P<0.05). During the follow-up period (median: 36 months, range: 12-78 months), the modified Rankin scale (mRS) score, the maximum mRS score during hospitalization, the mRS score at discharge, and the mRS score at 12 months after discharge were higher in the disturbance of consciousness group (all P < 0.001). However, there was no significant difference in long-term outcomes and recurrence between the two groups. Multivariate logistic regression analysis showed that mechanical ventilation, elevated IgG index, and delayed immunotherapy were independent risk factors for poor outcomes in patients with anti-NMDAR encephalitis with disturbance of consciousness at 12 months (odds ratio: 22.591, 39.868, 1.195). The receiver operating characteristics (ROC) curve analysis showed that the area under the curve (AUC) of mechanical ventilation, elevated IgG index, and delayed immunotherapy was 0.971 (95% CI=0.934-1.000, P<0.001). Mechanical ventilation, elevated IgG index, and delayed immunotherapy may be the influencing factors of poor prognosis of anti-NMDAR encephalitis patients with disturbance of consciousness. Although their condition is relatively serious, most patients with anti-NMDAR encephalitis with disturbance of consciousness will achieve favorable long-term outcomes after long-term treatment.

Plasma lipid profiles and homocysteine levels in anti-N-methyl-D-aspartate receptor encephalitis.

We aimed to investigate whether lipid profiles and homocysteine levels in patients with anti-N-methyl-D-aspartate receptor encephalitis are related to clinical presentation and prognosis, which may contribute to further research on the pathogenesis and treatment of this disease. This study included a total of 43 patients with anti-N-methyl-D-aspartate receptor encephalitis and 43 sex-age-matched healthy controls. Baseline demography, clinical data, patient outcomes, and ancillary examination results were recorded. Patients were followed up every 2-3 months during the first year. The modified Rankin Scale score was used to evaluate the therapeutic effect and clinical outcome. Among the 43 patients included in this study, 55.81% were male, the mean age of onset was 27 years old, and the median modified Rankin Scale score on admission was 3.0. Apolipoprotein A-1 was significantly lower in patients with anti-N-methyl-D-aspartate receptor encephalitis compared with healthy controls (p = 0.004). Compared with healthy controls, homocysteine (p = 0.002), apolipoprotein B (p = 0.004), Lpa (p = 0.045), and apolipoprotein B/apolipoprotein A-1 (p = 0.001) were significantly increased in patients with anti-N-methyl-D-aspartate receptor encephalitis. According to the modified Rankin Scale scores, 6 months after discharge, 72.09% of patients had a good prognosis and 27.91% had a poor prognosis. In the good prognosis group, age (p = 0.031), lipoprotein a (p = 0.023), apolipoprotein A-1 (p = 0.027) at baseline, and the modified Rankin Scale score on admission (p = 0.019) were significantly higher than those in the poor prognosis group. This study suggests the possibility that serum lipid profile and homocysteine play an important role in the pathogenesis of anti-N-methyl-D-aspartate receptor encephalitis, providing support for lipid-lowering treatment of anti-N-methyl-D-aspartate receptor encephalitis patients.

Clinical characteristics, treatments, outcome, and prognostic factors of severe autoimmune encephalitis in the intensive care unit: Standard treatment and the value of additional plasma cell-depleting escalation therapies for treatment-refractory patients.

To investigate severe autoimmune encephalitis (AE) in the intensive care unit (ICU) with regard to standard treatment in responsive patients and additional escalation therapies for treatment-refractory cases. This retrospective, single-center study analyzed medical records of ICU-dependent AE patients for clinical characteristics, treatments, prognostic factors, and neurological outcome as quantified by modified Rankin Scale (mRS) and Clinical Assessment Scale for Autoimmune Encephalitis (CASE). From 40 enrolled patients (median age=52 years; range=16-89 years) with AE mediated by neuronal surface antibodies (nsAb; 90%) and AE with onconeuronal antibodies (10%), 98% received first-line therapy. Of those, 62% obtained additional second-line therapy, and 33% received escalation therapy with bortezomib and/or daratumumab. Good neurological outcome, defined as mRS=0-2, was observed in 47% of AE with nsAb (CASE=5), 77% of anti-N-methyl D-aspartate receptor encephalitis patients (CASE=1), whereas AE patients with onconeuronal antibodies had the poorest outcome (mRS=6, 100%). Treatment-refractory AE patients with nsAb requiring escalation therapy achieved similarly good recovery (mRS=0-2, 39%, CASE=3) as patients improving without (mRS=0-2, 54%, CASE=4), although they presented a higher disease severity at disease maximum (mRS=5 100% versus 68%, CASE=24 versus 17; p= 0.0036), had longer ICU stays (97 versus 23 days; p= 0.0002), and a higher survival propability during follow-up (p= 0.0203). Prognostic factors for good recovery were younger age (p= 0.025) and lack of preexisting comorbidities (p= 0.011). Our findings suggest that treatment-refractory AE patients with nsAb in the ICU can reach similarly good outcomes after plasma cell-depleting escalation therapy as patients already responding to standard first- and/or second-line therapies.

Gut microbiome changes in anti-N-methyl-D-aspartate receptor encephalitis patients

BackgroundAnti-N-methyl-D-aspartate receptor (NMDAR) encephalitis is a type of autoimmune encephalitis. The underlying mechanism(s) remain largely unknown. Recent evidence has indicated that the gut microbiome may be involved in neurological immune diseases via the "gut-brain axis". This study aimed to explore the possible relationship between anti-NMDAR encephalitis and the gut microbiome.MethodsFecal specimens were collected from 10 patients with anti-NMDAR encephalitis and 10 healthy volunteers. The microbiome analysis was based on Illumina sequencing of the V3-V4 hypervariable region of the 16S rRNA gene. The alpha, beta, and taxonomic diversity analyses were mainly based on the QIIME2 pipeline.ResultsThere were no statistical differences in epidemiology, medication, and clinical characteristics (except for those related to anti-NMDAR encephalitis) between the two groups. ASV analysis showed that Prevotella was significantly increased, while Bacteroides was reduced in the gut microbiota of the patients, compared with the controls. Alpha diversity results showed a decrease in diversity in the patients compared with the healthy controls, analyzed by the Shannon diversity, Simpson diversity, and Pielou_E uniformity based on the Kruskal–Wallis test (P = 0.0342, 0.0040, and 0.0002, respectively). Beta diversity analysis showed that the abundance and composition of the gut microbiota was significantly different between the two groups, analyzed by weighted and unweighted UniFrac distance (P = 0.005 and 0.001, respectively).ConclusionsThe abundance and evenness of bacterial distribution were significantly lower and jeopardized in patients with anti-NMDAR encephalitis than in healthy controls. Thus, our findings suggest that gut microbiome composition changes might be associated with the anti-NMDAR encephalitis. It could be a causal agent, or a consequence.

Clinical characteristics of anti-N-methyl-d-aspartate receptor encephalitis in patients with a long-term history of mental disorders

BackgroundAnti-N-methyl-d-aspartate (NMDA) receptor encephalitis is an autoimmune disorder characterized by complex neuropsychiatric syndromes during disease onset. Although this disease has been well documented in the last decade, clinical characteristics of anti-NMDA receptor encephalitis in patients with long-term diagnostic history of mental disorders remain unclear.MethodsHere, we reviewed and analyzed series of anti-NMDA receptor encephalitis patients with a long-term medical history of psychiatric disorders through a review of literature using PubMed, web of science and Embase database. In addition, we described a patient of anti-NMDA receptor encephalitis with a long-term history of major depressive disorder.ResultsA total of 14 patients with anti-NMDA receptor encephalitis and a long-term history of mental disorders were included in our study. We found that most patients were adult (92.9%) and female (78.6%). These patients often first visited a psychiatric department (71.43%). The mean disease course of psychiatric disorders was more than 9 years. Speech impairment (71.4%), abnormal behaviors (64.3%), and catatonia (64.3%) were the most common clinical symptoms. Most patients (85.7%) had a satisfactory prognosis after immunotherapy.ConclusionAnti-NMDA receptor encephalitis in individuals with mental disorders is an underestimated condition, yet it presents complex clinical symptoms. Mental and behavioral impairments are more frequently observed in newly diagnosed anti-NMDA receptor encephalitis patients with a long-term history of mental disorders than those without mental illness. A diagnosis of anti-NMDA receptor encephalitis should be considered when patients with mental illness show sudden fluctuations in psychiatric symptoms.

Correlation analysis between regional homogeneity and executive dysfunction in anti-N-methyl-D-aspartate receptor encephalitis patients.

Anti-N-methyl-d-aspartate receptor (NMDAR) encephalitis is characterized by a range of cognitive impairments, especially in executive function. Our study aims to identify the abnormal regional homogeneity (ReHo) in anti-NMDAR encephalitis patients and its relationship with the executive function. Forty patients and 42 healthy volunteers undertook an Attention Network Test and a resting-state functional magnetic resonance imaging scan. ReHo analysis was performed to investigate the neuronal activity synchronization in all subjects. Based on ReHo analysis, a multivariate pattern analysis (MVPA) was carried out to identify the brain regions that differed the most between the two groups. Compared to controls, the patients had higher executive control scores (p<0.05). The patients presented reduced ReHo values in the bilateral posterior cerebellar lobe, anterior cerebellar lobe, midbrain, bilateral caudate nucleus, right superior frontal gyrus, right middle temporal gyrus, bilateral inferior parietal lobule and the left middle frontal gyrus. The ReHo values of the bilateral inferior parietal lobule in patients were found to be negatively associated with executive control scores. The classification of patients and controls using MVPA had an accuracy of 76.83%, a sensitivity of 82.50%, a specificity of 71.43% and the area under the curve was 0.83. Our study provides evidence of abnormal cerebral function in anti-NMDAR encephalitis patients, which may contribute to unveiling the neuropathological mechanisms of anti-NMDAR encephalitis and their influences on executive dysfunction. The MVPA classifier, based on ReHo, is helpful in identifying anti-NMDAR encephalitis patients from healthy controls.

Anti-GABA-B receptor encephalitis with small cell lung cancer presenting with septic shock during immunotherapy

Abstract Introduction: Most patients with anti-γ-aminobutyric acid-B (anti-GABA-B) receptor encephalitis present with cancer and have a poor response to immunotherapy. Moreover, septic shock, a subset of sepsis, is rarely reported in these patients during immunotherapy. Patient concerns: A 55-year-old man presented with epileptic seizure and cognitive impairment. He was previously healthy and had no family history of psychiatric disorders but had a history of smoking and emphysema. Diagnoses: Combined with the symptoms and antibody testing, anti-GABA-B encephalitis with anti-Hu antibody was diagnosed. During immunotherapy, he presented with septic shock. Further investigations and pathological examination confirmed the diagnosis of small cell lung cancer. Interventions: The patient was treated with five days of intravenous immunoglobulin (0.4/kg/d), ten days of methylprednisolone (1.0 g once a day and half the dose every three days), plus ten days of oxycarbazepine (0.3 g b.i.d.) and magnesium valproate (0.25 g b.i.d.). He received nine days of conventional treatment for septic shock in addition to meropenem (1 g intravenously every 8 hours) and etimicin (0.3 g intravenously per day). After that, he received one month of prednisone 30 mg with dose reduction 5 mg every two weeks, oxcarbazepine 0.3 g b.i.d. and valproic acid 0.25 g b.i.d. for antiepileptic treatment, olanzapine 5 mg qd + qn for antipsychotic symptoms. Outcomes: He was able to communicate with others normally and also had improved memory and clear thinking. Mini-Mental State Examination and Modified Rankin Scale were 23/30 and 1. At discharge, the titer of anti-GABA-B receptor antibody in serum (1:10) was significantly lower than the titer in serum on the day of admission (1: 100). Conclusion: Anti-GABA-B receptor encephalitis patients with cancer can respond well to immunotherapy. Personalized treatment should be considered to avoid complications like septic shock in anti-GABA-B receptor encephalitis with cancer during immunotherapy.

Does the NEOS Score Predict Outcomes in Pediatric Anti-NMDA Receptor Encephalitis Patients? (1860)

Does the NEOS Score Predict Outcomes in Pediatric Anti-NMDA Receptor Encephalitis Patients? (1860)

Distinct cerebral 18F-FDG PET metabolic patterns in anti-N-methyl-D-aspartate receptor encephalitis patients with different trigger factors.

Aim:Anti-N-methyl-D-aspartate receptor (anti-NMDAR) encephalitis is a subgroup of treatable autoimmune encephalitis, characterized by rapid development of psychosis, cognitive impairments and seizures. Etiologically, anti-NMDAR encephalitis could be divided into three subgroups, which are paraneoplastic (especially associated with ovarian teratoma), viral encephalitis-related and cryptogenic. Each type is different in clinical course, treatment strategies and prognosis. In this study, we aim to investigate whether anti-NMDAR encephalitis patients with different trigger factors exhibit distinct cerebral metabolic patterns detected by 18F-fluorodeoxyglucose positron emission tomography imaging.Methods:24 patients with anti-NMDAR encephalitis in acute phase from Huashan Hospital, Fudan University (Shanghai, China) were recruited in this study. Each patient was classified into one of etiological subgroups. Positron emission tomography images of individual patients were analyzed with both routine visual reading and computer-supported reading by comparison with those of the same 10 healthy controls using a voxel-wise statistical parametric mapping analysis.Results:Patients in both the cryptogenic (13 patients) and paraneoplastic (five patients) subgroups showed hypermetabolism in the frontal-temporal lobes and basal ganglia, covarying with hypometabolism in the occipital regions. Notably, the abnormal metabolism was usually asymmetric in the cryptogenic subgroup, but relatively symmetric in the paraneoplastic subgroup. Moreover, the other six patients secondary to viral encephalitis presented with significant hypometabolism in the bilateral occipital regions, as well as in the unilateral temporal lobes and part of basal ganglia (also is virus infection side), but hypermetabolism in the contralateral temporal areas.Conclusion:This study revealed that patients with anti-NMDAR encephalitis triggered by different factors presented distinct cerebral metabolic patterns. Awareness of these patterns may help to better understand the varying occurrence and development of anti-NMDAR encephalitis in each subgroup, and could offer valuable information to the early diagnosis, treatment and prognosis of this disorder.Trial registration numberChiCTR2000029115 (Chinese clinical trial registry site, http://www.chictr.org)

The differential expression and potential roles of circular RNAs in children with anti-NMDA receptor encephalitis

To explore the role of circular RNAs (circRNAs) in pediatric anti-N-methyl-d-aspartate (NMDA) receptor encephalitis, we assessed the profiles of circRNAs and mRNAs in blood leukocytes from anti-NMDA receptor encephalitis patients and healthy controls using microarray analysis.1196 circRNAs and 719 mRNAs (change ≥2, p < .05) were dysregulated in anti-NMDA receptor encephalitis patients, relative to healthy controls, based on microarray data. Further bioinformatic analysis demonstrated that the host genes of dysregulated circRNAs are primarily associated with receptor internalization.In conclusion, circRNAs may be implicated in the pathogenesis of pediatric anti-NMDA receptor encephalitis.

Anti-NMDA Receptor Encephalitis: Efficacy of Treatment for Male Patients and miRNA Biomarker.

Anti-N-methyl-D-aspartate (NMDA) receptor encephalitis is an acute form of encephalitis. Treatments for the anti-NMDA receptor encephalitis usually include steroids, intravenous immunoglobulin, plasma exchange, plasmapheresis, rituximab, cyclophosphamide and tumor resection. We aimed to compare the efficacy of the treatments including intravenous immunoglobulin, plasma exchange, plasmapheresis, rituximab or cyclophosphamide for male anti- NMDA receptor encephalitis patients without tumor and to discuss potential biomarkers for this disease. The Fisher exact test and the contingency table analysis were used to analyze the treatment efficacy for 43 male and 76 female patients. In addition, a hierarchical tree method was adopted to analyze the difference in the treatment efficacy between male and female patients. The p-values of testing whether the efficacy rate of plasmapheresis (or plasma exchange) for the male patient is greater than a threshold are significantly different from the pvalues for the other two treatments. In addition, the hierarchical tree method shows that the treatment strategy associating with early recovery is different for male and female patients. The results revealed that the efficacy rate of plasmapheresis (or plasma exchange) is not inferior to that of intravenous immunoglobulin and rituximab (or cyclophosphamide) for male patients without tumor. In addition, B-cell attracting C-X-C motif chemokine 13 (CXCL13) and microRNA let-7b have the potential to be the treatment response biomarkers for anti-NMDA receptor encephalitis. They may not be useful prognostic biomarkers for this encephalitis unless they are not biomarkers for other autoimmune encephalitides.

Immunotherapy in Probable autoimmune encephalitis without detected autoantibody

Abstract Along with the rapidly expanding spectrum of autoimmune encephalitis (AE), probable AE with no detected autoantibody (AE-NoDab) has become a major category of AE. However, its clinical features, treatment, and prognosis were not demonstrated. In our institutional cohort of all consecutive patients with possible AE in 2012 – 2018, patients diagnosed with AE-NoDab were included in this study. AE-NoDab was subcategorized into limbic encephalitis (LE), acute disseminated encephalomyelitis (ADEM), and antibody negative probable AE (pAE). Patients’ clinical severity was assessed at every week for the first 12 weeks, at every month for the next 9 months, and then at every three months, using the modified Rankin scale (mRS) and the Clinical Assessment Scales in Autoimmune Encephalitis (CASE, score range 0–27) scores. Combination immunotherapy regimens used at each time point were categorized as steroid+immunoglobulin (SI), SI+Rituximab (anti CD-20 mAb, SIR), and SIR+ Tocilizumab (anti IL-6 mAb, SIRT). 136 patients (LE 52[38.2%], ADEM 14[10.3%], and pAE 70 [51.5%]) were followed-up for 39.0±19.6 (range 10–80) months. At last follow-up, 76 (55.9%) patients had favorable mRS outcomes (scores 0–2). Compared to 55 anti-NMDA receptor encephalitis patients, baseline severity was higher, use of immunotherapy was lower, and outcome was poorer in AE-NoDab. There was no difference in clinical characteristics, treatment, and outcomes among subcategories of AE-NoDab. In a linear mixed model analysis, using the SIRT regimen was more effective than conventional regimens such as SIR or SI in lowering CASE scores (both, P=0.001). Early diagnosis and use of combined immunotherapy consisting of SIRT is warranted to improve the poor outcomes of AE-NoDab.

Clinical Characteristics and Prognosis of Severe Anti-N-methyl-D-aspartate Receptor Encephalitis Patients.

Data concerning the characteristics and duration of the critical manifestations, treatment response, and long-term outcomes of severe anti-N-methyl-D-aspartate receptor (anti-NMDAR) encephalitis patients compared to those of non-severe patients are limited. This observational study was performed to explore the clinical characteristics and long-term outcomes of severe anti-NMDAR encephalitis patients. According to their characteristics on admission to the neurology intensive care unit, patients with anti-NMDAR encephalitis were divided into a severe group and a non-severe group. The demographics, clinical manifestations, main accessory examinations, immunotherapy, and outcomes of patients were recorded. Statistical analyses were employed to examine the differences in each observed indicator between the severe and non-severe groups. This study enrolled 111 patients with anti-NMDAR encephalitis, including 59 males and 52 females with a mean age of 27.7 ± 13.7years; 39 (35.1%) patients were in the severe group, and 72 (64.9%) patients were in the non-severe group. Compared to the non-severe group, the severe group exhibited a higher proportion of epilepsy, involuntary movement, disturbance of consciousness, autonomic dysfunction, and central hypoventilation. The cerebrospinal fluid (CSF) of all patients was positive for the NMDAR antibody, but only 57 patients (51.4%) tested positive for the NMDAR antibody in the blood. The proportion of patients with a strong positive NMDAR antibody titer in the severe group (48.7%) was higher than that in the non-severe group (29.2%). The proportion of patients receiving intravenous gamma immunoglobulin in the severe group was higher than that in the non-severe group (P = 0.003), and only patients in the severe group received plasma exchange, intravenous rituximab, and cyclophosphamide treatment. No significant difference was observed in the prognosis between the severe group and the non-severe group after 6months and during long-term follow-up. Most severe anti-NMDAR encephalitis patients will eventually achieve good long-term prognoses after receiving early, positive and unremitting combined immunotherapy and life support.

Analysis of electroencephalogram characteristics of anti-NMDA receptor encephalitis patients in China.

To explore the characteristics of electroencephalogram (EEG) in patients with anti-N-methyl-d-aspartate receptor (anti-NMDAR) encephalitis. Anti-NMDAR encephalitis patients admitted to the Department of Neurology between January 2012 and June 2016 were enrolled. All patients underwent electroencephalogram (EEG) at least once in the disease peak stage, and received tumor screening, symptomatic therapy, and immunotherapy. Patients received outcome evaluation every 6months after the immunotherapy, and modified Rankin scale (mRS) 0-2 was defined as favorable outcome. This study enrolled 62 cases of anti-NMDAR encephalitis patients, including 29 males (46.8%) and 33 females (53.2%). The patient ages were between 10 and 59 (mean 26.3±11.3) years. A total of 93 instances of EEG monitoring were performed on 62 patients. At the peak stage, EEG presentations showed 61 cases (98.4%) were abnormal, cranial MRI showed 29 cases (46.8%) were abnormal among all 62 patients. The main presentations of abnormal EEG were diffuse slowing (25 cases, 40.3%), epileptiform discharges (11 cases, 17.7%), extreme delta brush (EDB) (10 cases, 16.1%), polymorphic delta rhythm (6 cases, 9.7%), focal slowing (5 cases, 8.1%), and diffuse beta activities (4 cases, 6.5%). Patients with normal background, epileptiform discharges, polymorphic delta rhythm, and diffuse beta activity in EEG all had favorable long-term outcome. The majority of anti-NMDAR encephalitis patients had abnormal EEG. EEG could sensitively reflect the abnormal brain functions of patients and could assist with early clinical diagnosis and prognosis prediction. Diffuse slowing was the most common presentation on the EEG in patients with anti-NMDAR encephalitis. The EEG pattern of normal background, epileptiform discharges, polymorphic delta rhythm, and diffuse beta activities at the peak stage might suggest favorable long-term prognosis.

Analysis of EEG features of neuronal surface antibody associated encephalitis

Objective To summarize the clinical manifestations, EEG and head MRI features of neuronal surface antibody associated encephalitis, and to investigate the role of EEG in determining the relapse or fluctuation of this disease, characteristics of EEG corresponding to head MRI, and EEG features in different clinical stages. Methods A total of 23 patients with neuronal surface antibody associated encephalitis were divided into ascent, climax, descent and recovery stage according to their clinical course. The relation between EEG background activity, distribution of slow wave, epileptiform discharge, extreme delta brush (EDB) and relapse or fluctuation of the disease was analyzed. The relation between EEG features and head MRI abnormalities, and also EEG features in different stages were analyzed. Results There were 19 anti-N-methyl-D-aspartate (NMDA) receptor encephalitis patients, 3 anti-leucine-rich glioma-inactivated 1 (LGI1) antibody associated encephalitis and one anti-γ-aminobutyric acid B receptor (GABABR) antibody associated encephalitis. The frequencies of clinical presentations were psychological or cognitive dysfunction, epileptic seizure, conscious disturbance, speech dysfunction and movement disorder in descending order. Within 30.50 d from onset, 6 patients demonstrated slow wave background, of whom 2 relapsed or fluctuated; 5 patients had α rhythm background and none of them relapsed or fluctuated. In patients with anti-NMDA receptor encephalitis, the difference in first hospital stay ( Z = -0.785, P = 0.433) and relapse or fluctuation (Fisher's exact probability: P = 0.155) between EDB group and non-EDB group was not significant. There was no apparent correlation between EEG background activities and head MRI abnormalities in different stages. In ascent and climax stage, EEG background activities were predominantly slow wave, and the distribution of slow wave was relatively broader. EEG background changed to α rhythm from descent stage and slow wave distribution decreased in recovery stage. Conclusions Some presentations of EEG, such as early background activities may be correlated with disease relapse or fluctuation. The incompatibility of EEG and head MRI suggests the importance of functional examinations in patients with neuronal surface antibody associated encephalitis. EEG features vary in different stages. DOI: 10.3969/j.issn.1672-6731.2016.09.010

Anti-LGI1 and anti-NMDA receptor encephalitis show distinct patterns of brain glucose metabolism in FDG-PET

Pathogenic autoantibodies targeting the recently identified leucin rich glioma inactivated 1 protein (LGI1) and the NR1 subunit of the N-methyl-D-aspartat (NMDA) receptor induce clinically distinct subtypes of limbic encephalitis. A comparison of brain metabolic patterns in FDG-PETs of anti-LGI1 and anti-NMDA receptor encephalitis patients has not been performed yet.

Immunological profiles of the CSF from anti-NMDA receptor encephalitis patients

Background: Anti-N-methyl-D-aspartate receptor autoantibodies (NMDAR-Abs) are thought to relate closely to the pathogenesis of NMDAR encephalitis. The antibody-titre /INS;parallels with the disease activities and antibody-deprivation treatments are effective. This disease is thought to be immune-mediated;/INS; however, studies have not been done extensively from the immunological aspects.

Anti-NMDA Receptor Encephalitis in Children Is Associated with Variable Outcome (P07.140)

Objective: To describe the presentation, clinical course and outcome of a series of pediatric anti-N-methyl-D-aspartate (NMDA) receptor encephalitis patients. Background Anti-NMDA receptor encephalitis is a recently described form of autoimmune encephalitis that typically presents with psychiatric disturbance, seizures, dyskinesias and autonomic instability. The condition is frequently associated with ovarian teratoma and is more common in adults. Prognosis is usually good with most patients experiencing either complete recovery or the persistence of only mild deficits. The clinical course and outcome is less well known in children. Design/Methods: Case series. We report on three pediatric patients who had similar clinical presentations and were ultimately found to have anti-NMDA receptor encephalitis. Results: Three previously healthy female children ages 4, 6 and 10 years presented with new onset complex partial seizures that proved intractable. Repeated electroencephalograms showed focal abnormalities. Cerebrospinal fluid studies showed mild to moderate pleocytosis with negative culture. All patients subsequently developed psychiatric disturbances varying from delirium to visual hallucinations. Oral motor dyskinesias were seen in all patients and labile blood pressure was present in two patients. CSF was found to be positive for anti-NMDA receptor antibodies in all three patients. Repeated pelvic ultrasounds have been normal. All patients received high dose steroids and IVIG and two patients received cyclophosphamide. One patient has recovered with only minimal deficits and only occasional seizures, one patient is in a vegetative state with daily seizures and one patient has severe cognitive and motor deficits with frequent seizures. Conclusions: Anti-NMDA receptor encephalitis is associated with variable outcome in children with poor outcome possibly being more prevalent than in adults. Disclosure: Dr. Aaen has nothing to disclose. Dr. Michelson has nothing to disclose. Dr. De La Pena has nothing to disclose. Dr. Przekop has nothing to disclose. Dr. Roddy has nothing to disclose. Dr. Shu has nothing to disclose. Dr. Ashwal has nothing to disclose.

Suppression of synaptic plasticity by cerebrospinal fluid from anti-NMDA receptor encephalitis patients

The functional effects of cerebrospinal fluid (CSF) from patients with anti-NMDA receptor (NMDAR) encephalitis on the NMDAR-mediated synaptic plasticity were evaluated by using mouse hippocampus slices. Anti-NMDAR antibody detection system was established by immunostaining recombinant NMDAR heteromers expressed in HEK cell culture as well as native NMDARs in cultured hippocampal neurons. Under a complete blind manner for the clinical information, CSF and sera collected from 36 pre-diagnosed patients were tested for anti-NMDAR antibodies. With this test, thirteen patients were diagnosed as anti-NMDAR encephalitis. CSF positive for anti-NMDAR antibodies suppressed induction of long-term potentiation (LTP) at Schaffer collateral-CA1 synapses in mouse hippocampal slices. LTP induction was not suppressed by CSF collected from herpes simplex virus (HSV) encephalitis or non-encephalitis control patients. Antibody absorption with NMDAR-expressing HEK cell culture reversed the suppression of LTP by anti-NMDAR encephalitis patients' CSF, confirming that anti-NMDAR antibodies suppressed LTP. The present experiments firmly support the proposal that the anti-NMDAR encephalitis autoantibody is responsible for cognitive disorders like amnesia accompanying this disease.