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

Conclusions: The 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.

Anti-N-Methyl-D-Aspartate Receptor Encephalitis: Neuropsychiatric and Multidisciplinary Approach to a Patient Not Responding to First-Line Treatment

This case report presents the clinical vignette of a 30-year-old male patient, who presented with altered sensorium, disorganized behavior, and perceptual disturbance. Initially, he was put on standard psychotropic medications that did not help. After a careful review, the patient was diagnosed with anti-NMDA receptor encephalitis endorsed by the identification of CSF antibodies. There are several points to be highlighted within this case study. Initially, clinical suspicion and keeping a broad differential, despite what may appear as exclusively psychiatric symptoms, are important to prevent any delay in the appropriate testing or procedures that can hone in on the diagnosis. Successful treatment hinges on prompt induction of first-line treatment with steroids, and potentially second-line therapy with adjunct IVIG or immunosuppressants. Communication within the multidisciplinary approach, often combining clinicians from a myriad of subspecialties, is paramount to positive patient outcomes. Clinicians should remain alert and suspicious regarding the possibility of anti-NMDA receptor encephalitis in a patient with predominantly disorganized or psychotic symptoms who may not be responding to conventional antipsychotic therapy.

Anesthesia for ovarian teratoma resection using remimazolam and remifentanil in a patient with anti-N-methyl-D-aspartate receptor encephalitis: two case reports

Conclusions: Remimazolam and remifentanil are useful general anesthetic agents for patients with anti-NMDA receptor encephalitis. Further studies are warranted.

Recurrent Optic Neuritis in Paediatric Anti-N-Methyl-D-Aspartate Receptor (Anti-NMDAR) Encephalitis: Case Report and Literature Review

A report a case of a 10-year-old girl who first presented with anti-NMDAR encephalitis and later developed four episodes of left optic neuritis. Blurring of vision, pain on eye movement, and headache were the striking features in all episodes of optic neuritis. Optic neuritis and anti-NMDAR encephalitis are reversible with intensive immunotherapy. Early and accurate diagnosis can lead to better management of this condition.

Clinical Characteristics of Anti-N-Methyl-D-Aspartate Receptor Encephalitis Overlapping with Demyelinating Diseases: A Review

NMDAR may occur simultaneously or sequentially with different demyelinating diseases. Compared with the age of patients with classical NMDARe, patients with overlapping MS or AQP4-positive NMOSD tended to be slightly older. They also had lower incidence of ovarian teratoma. Overlapping syndrome patients frequently presented atypical symptoms and/or MRI abnormalities when compared with classical NMDARe. MRI scans and detection of OB, AQP4, and MOG antibodies are helpful for identifying overlapping syndromes. Favorable outcome was observed in patients overlapping with MOGAD, but no robust comparison can be drawn with the patients overlapping with AQP4-Ab-positive NMOSD and MS regarding the small number of available data. Long-term follow-up investigation is needed because of the long interval between NMDARe and demyelinating diseases.

Evaluating the incidence and predictors of anti-NMDAR encephalitis in a contemporary cohort of patients diagnosed with dermoid tumors: A national inpatient sample analysis

CONCLUSION: Patient predictors of developing anti-NMDAR encephalitis include age, race, ethnicity and patients who go on to develop anti-NMDAR encephalitis have a significantly increased hospital LOS and cost compared to those who do not. Future research, including multi-center clinical trials and longitudinal data, is necessary to fully cement the findings of this manuscript.

Cervical lymph nodes and ovarian teratomas as germinal centres in NMDA receptor-antibody encephalitis

In conclusion, these data provide evidence for tissue-compartmentalized GC-driven production of NR1-IgGs in a prototypical autoantibody-mediated human CNS disease. Our findings offer insights that affect understanding pathogenesis and may influence clinical care and trial designs in patients with NMDAR-antibody encephalitis. More broadly, we provide a paradigm to study systemic-CNS immune interactions in humans, in both health and across varied CNS diseases.

State-dependent signatures of anti-N-methyl-D-aspartate receptor encephalitis

Our analyses identified distinct brain states with characteristic patterns of functional connectivity (FC) alterations and shifted temporal dynamics in patients with anti-NMDA receptor encephalitis that remained undetected in conventional static analyses. Critically, dynamic FC measures correlated with disease severity and psychiatric symptoms, suggesting that altered resting-state dynamics carry meaningful clinical information about anti-NMDA receptor encephalitis. Given converging findings in other neuropsychiatric diseases, time-resolved FC analysis holds promise for an improved characterization and understanding of brain functioning in these disorders.

Cerebral Metabolic Network in Patients With Anti-N-Methyl-D-Aspartate Receptor Encephalitis on 18F-FDG PET Imaging

This study conducted a multiparameter quantitative evaluation of network changes in the brain regions of patients with anti-NMDAR encephalitis using a graph theory analysis method. The detected metabolic brain network abnormalities help improve the understanding of the neuropathological and physiological mechanisms of patients with anti-NMDAR encephalitis.

Brain MRI features of anti-N-methyl-D-aspartate (anti-NMDA) receptor encephalitis secondary to central nervous system infection in adult patients

Conclusion: Anti-NMDAR encephalitis secondary to CNS infection has its imaging features-extensive lesion distribution, leptomeningeal enhancement, early atrophy, and necrosis-that could deepen the understanding of the pathophysiology and manifestation of the autoimmune encephalitis besides the classic type.

Anti-N-Methyl-D-Aspartate Receptor Encephalitis (ANMDARE) in a Patient With Hashimoto’s Thyroiditis

This is a very interesting case study of a man with Hashimotos’s Thyroiditis who was found to have anti-NMDAr encephalitis.
The details of the patient repeatedly reporting to emergency room will be familiar to those in the AE community.
“Here we present a unique case of a patient with Hashimoto thyroiditis diagnosed with ANMDARE who presented with rapid mental cognitive decline. His atypical presentation made the diagnosis challenging as various etiologies of encephalitis were considered. Early treatment allowed for the improvement of symptoms, although relapse could not be prevented.”
Interesting and enlightening read.

Neutrophil Percentage-to-Albumin Ratio: A Good Parameter for the Evaluation of the Severity of Anti-NMDAR Encephalitis at Admission and Prediction of Short-Term Prognosis

The purpose of this study was to investigate the association of neutrophil percentage-to-albumin ratio (NPAR) with the severity at admission and discharge (short-term prognosis) in patients with anti-N-methyl-D-aspartic acid receptor (NMDAR) encephalitis.

 A total of 181 patients with anti-NMDAR encephalitis diagnosed at the First Affiliated Hospital of Zhengzhou University were included. The results showed that NPAR had good sensitivity and specificity in assessing disease severity at admission and predicting short-term prognosis. The multivariable logistic regression models based on NPAR and other influencing factors had good discrimination, consistency, accuracy, calibration ability, applicability, and validity in assessing the severity at admission and predicting short-term prognosis.

Rituximab as Second-Line Treatment in Anti-NMDAR Encephalitis after Herpes Simplex Encephalitis in Children

The long-term response of two infants with anti-N-methyl-D-aspartate receptor (anti-NMDAR) post herpes simplex encephalitis treated with rituximab is reported here. Rituximab may improve the course of the disease and should be considered early as second-line treatment. Data on the long-term effect of rituximab in B cell depletion and immunoglobulins levels in infants are needed.

Abnormal Brain MRI Findings in Anti-N-Methyl-D-Aspartate Receptor Encephalitis and Correlation With Outcomes

Thirty-seven (71.15%) of the patients showed abnormalities on brain MRI; these patients were more likely to be men and showed abnormalities on electroencephalography. Patients who showed normal or abnormal MRI findings did not differ significantly in terms of clinical symptoms, rates of mortality or relapse, or mRS scores after 3 and 12 months. However, patients with abnormal MRI showed significantly lower MMSE scores than those with normal MRI after 3 and 12 months.

In all the 52 patients, 35 (67.31%) showed good outcomes after 3 months, and this rate increased to 76.92% after 12 months. In 3 months, five (9.62%) of the patients had died and six (11.54%) had experienced relapse (Table 2). In 12 months, the number of mortalities did not increase, but relapse rate increased to 15.38%

Contemporary advances in anti-NMDAR antibody (Ab)-mediated encephalitis

This easy to understand review delineates pathogenic and clinical advances to date in anti-NMDAR antibody-mediated encephalitis. Anti-NMDAR Ab-mediated encephalitis thus represents an increasingly well-characterized, proportionately common, treatable form of antibody-mediated encephalitis, often with a unique combination of clinical and paraclinical features, benefiting from rapid advances in clinical research over the past two decades. Timely diagnosis and treatment are associated with more favorable patient outcomes. As with other CNS inflammatory disorders however, avoidance of misdiagnosis is also of paramount.

The Spectrum of Movement Disorders in Anti-N-Methyl-D-Aspartate Receptor (NMDAR) Encephalitis Both in Children and Adults: An Experience From a Single Tertiary Care Center

Patients suffering from anti-NMDAR encephalitis may exhibit a wide range and combination of movement disorders. Orofacial dyskinesia, dystonia of face, trunk and limb, chorea, myoclonus, tremor, choreoathetosis, opsoclonus-myoclonus, and ataxia have been reported in approximately 86% of adults and 84% of children. Another case series in children and adolescents showed that stereotyped movements (85%) and orofacial dyskinesia (45%) were the most common hyperkinetic movement disorder associated with anti-NMDAR encephalitis. The other associated hyperkinetic movements in this series were dystonia, chorea, and myorhythmia.

Current Status of Biomarkers in Anti-N-Methyl-D-Aspartate Receptor Encephalitis

Contains an overview of anti-NMDAr enccephalitis, Clinical and Paraclinical Features as Markers of Anti-NMDAR Encephalitis, and Molecular Biomarkers in Anti-NMDAR Encephalitis.

The increasing knowledge about the pathophysiology of the immune system is providing new perspectives on the mechanism underlying autoimmune neurological disorders and offering novel candidates as biomarkers. However, the development of biomarkers in rare disorders, such as anti-NMDAR encephalitis, may be challenging due to the difficulties related to collecting large cohorts of patients to achieve solid conclusions. For these reasons, the majority of biomarkers validated in anti-NMDAR encephalitis are clinical or paraclinical, whereas most soluble biomarkers are still at the early phases of their development, with the notable exception of CSF IgG NMDAR antibodies that have been widely implemented in clinical practice.

Impairment of early neuronal maturation in anti-NMDA-receptor encephalitis

Results

Serum and cerebrospinal fluid of the index patient statistically significantly impaired dendritic outgrowth of cultured rat cortical primary neurons. Serum of the index patient also statistically significantly delayed centrosome elimination. Impaired dendritic outgrowth and delayed centrosome elimination were not perfectly rescued by changing to normal culture media. Serum of the index patient also statistically significantly reduced the branching of dendrites.

Conclusions

This is the first demonstration of the damage by anti-NMDAR antibodies on early dendritic development in vitro. As a strategy to protect embryonic neurons, our findings may support the efficacy of early immunotherapy for anti-NMDAR encephalitis in pregnancy.

A peculiar case of psychosis: anti-NMDAr encephalitis

Background Psychosis in pregnancy is rare and could be life-threatening. It requires prompt evaluation and proper management accordingly. Anti-N-methyl-d-aspartate receptor (anti-NMDAr) encephalitis following herpes simplex virus (HSV) infection is a rare cause of psychosis during pregnancy. Case presentation A 20-year-old woman at 18 weeks gestation presented with agitation and a 3-day history of hallucinations. She had a prior hospitalization for HSV encephalitis 6 weeks before. Her laboratory workup was unremarkable except for positive anti-NMDAr antibodies in the CSF. The patient was treated with high-dose corticosteroids and plasmapheresis, and she was discharged 2 weeks later fully recovered. Conclusions Anti-NMDAr encephalitis can be the culprit behind a new-onset of psychosis in pregnancy. Early diagnosis and treatment are crucial.

Electroencephalography characteristics to predict one-year outcomes in pediatric anti-NMDA receptor encephalitis

Results: Nine patients (6 females) (range 1.9-16.7 years) were included. Five of nine patients had loss of posterior dominant rhythm (PDR) and three of nine patients had absent sleep architecture. Loss of PDR correlated with a worse mRS score at one year (2.8 versus 0.5, p = 0.038). Loss of PDR and loss of sleep architecture was associated with increased inpatient rehabilitation stay and in higher number of immunotherapy treatments administered. In multivariate analysis, absence of sleep architecture (p = 0.028), absence of PDR (p = 0.041), and epileptiform discharges (p = 0.041) were predictors of mRS at one year.

Conclusions: Loss of normal PDR, absence of sleep architecture, and epileptiform discharges are associated with worse outcomes at one year which has not been reported before. EEG characteristics may help prognosticate in NMDARE. Larger studies are needed to confirm these findings.

Longitudinal analysis of regional brain changes in anti-NMDAR encephalitis: a case report

Results suggest that reversible cortical volume loss in anti-NMDA encephalitis has a regional specificity that mirrors many of the clinical symptoms associated with the disorder and tracks the dynamics of disease severity over time. This case illustrates how quantitative morphometric techniques can be applied to clinical imaging data to reveal patterns of brain change that may provide insight into disease pathophysiology. More widespread application of this approach might reveal regional and temporal patterns specific to different types of autoimmune encephalitis, providing a tool for diagnosis and a surrogate marker for monitoring treatment response.

Lyme Disease and Associated NMDAR Encephalitis: A Case Report and Literature Review

Taking into account the marked clinical improvement after treatment with glucocorticosteroids, we suggest that NMDAR encephalitis is a possible autoimmune complication in neuroborreliosis patients requiring additional immunotherapy.” conclusion: “In our case, we believe that neuroborreliosis activated certain autoimmunity mechanisms, resulting in NMDAR antibodies and clinically manifested encephalitis. Additionally, we think that the NMDAR antibodies were not only an accidental finding, but they were pathogenic. We propose that immunotherapy for patients with positive NMDAR antibodies should be considered if there is even a slight possibility of improvement.

Extreme Delta Brush: The Key to Anti-NMDA Receptor Encephalitis

Conclusions: Anti-NMDA-receptor encephalitis is a life-threatening condition that is treatable when diagnosed timely and immunosuppressive treatment is established promptly. Presence of EDB, although only present in a minority of patients, can streamline investigation and allow for rapid initiation of therapy.

Long-term cognitive outcome in anti-NMDA receptor encephalitis

In conclusion, this study presents comprehensive longitudinal data for the cognitive outcome in
NMDAR encephalitis. All patients had cognitive deficits about 2 years after disease onset, mainly affecting memory and executive function. After 4 years, moderate or severe cognitive deficits persisted
in 2/3 of patients despite good functional neurological outcome, indicating that cognitive function is an important outcome measure in addition to the functional neurological scales. Impaired cognitive
outcome was predicted by delayed treatment and higher disease severity. However, continued improvement of cognitive function was observed for several years after disease onset in some patients.
Our results demonstrate that cognitive deficits are frequent and severe longterm sequelae following
NMDAR encephalitis. These deficits show a slow and incomplete recovery and persist beyond recovery of other neuropsychiatric symptoms of the disease. Consequently, our findings call for rapid diagnosis and treatment at disease onset as well as for continued and customized cognitive rehabilitation to improve the longterm outcome.

Successful treatment with immunoadsorption therapy in four patients with severe and refractory anti-N-methyl-D-aspartate receptor encephalitis

There is still no optimal treatment for patients with severe anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis refractory to first-line therapy (including intravenous methylprednisolone [IVMP] and intravenous immunoglobulin [IVIG]). A small study has shown that immunoadsorption (IA) is effective in treating anti-NMDAR encephalitis. However, the effectiveness and safety of IA in the treatment of patients with refractory and severe anti-NMDAR encephalitis is not fully known. Four patients with severe anti-NMDAR encephalitis are reported, which were refractory to the first-line immunotherapy including IVMP and IVIG. Immunoadsorption is performed during the fulminant stage of disease, and the effectiveness and safety of IA are assessed. The modified Rankin Scale (mRS) is used to assess neurological conditions before and after IA. Four patients with the most severe form of anti-NMDAR encephalitis (two with teratoma and two with unknown origin) did not respond to one or more rounds of IVMP plus IVIG. They all required intensive care unit (ICU) support including long-term mechanical ventilation, and thus developed ICU-related complications. Gradual and steady improvement was observed after IA treatment. Except for mild hypotension in patient 1, no other adverse events were observed during IA. Two patients had good early overall recovery on discharge. The other two patients had a good outcome with mRS of 2 at the 12-month follow-up. This small case series suggests that IA may be an effective treatment option to accelerate the recovery of patients with severe and refractory anti-NMDAR encephalitis.

Use and Safety of Immunotherapeutic Management of N-Methyl-d-Aspartate Receptor Antibody Encephalitis: A Meta-analysis

A meta-analysis of 1,550 patients with anti-NMDAR encephalitis. Key findings

– Factors associated with good functional outcomes were adolescent age and first-line treatment with either plasmapheresis alone, corticosteroids with IVIG, or corticosteroids with IVIG and plasmapheresis. 

– Factors associated with poor functional outcome were infant or older adult age, ICU admission, EEG extreme delta brush pattern, lack of immunotherapy ≤30 days of onset, and maintenance IVIG treatment for ≥six months. 

– Relapsing disease was associated with adolescent age, and non-relapsing disease was associated with rituximab use or maintenance IVIG for ≥six months. 

Four-year-old anti-N-methyl-D-aspartate receptor encephalitis patient with ovarian teratoma: A case report

CASE SUMMARY
This case report describes the successful treatment of anti-N-methyl-D-aspartatereceptor (NMDAR) encephalitis by early laparoscopic ovarian cystectomy and immunotherapy in a 4-year-old female child. And to the best of our knowledge,
this detailed case report describes the youngest patient to date with anti-NMDAR encephalitis who underwent laparoscopic ovarian cystectomy.
CONCLUSION
Although the younger the patient is, the less likely a tumor will be detected, we still emphasize that all patients with suspected or confirmed anti-NMDAR encephalitis should be screened for ovarian tumors if possible. Prompt initiation of immunotherapy and tumor removal are crucial for good outcomes.

The Effectiveness of Electroconvulsive Therapy on Catatonia in a Case of Anti-N-Methyl-D-Aspartate (Anti-NMDA) Receptor Encephalitis

The successful remission of catatonia associated with anti-NMDAR encephalitis using ECT treatment in our patient adds to the growing list of successful treatments reported in previous studies. ECT should be incorporated as standard protocol in the management of catatonia associated with anti-NMDAR encephalitis used in treating catatonia and associated psychiatric symptoms seen in these patients. Early treatment can be associated with better clinical outcomes.

High Level of Soluble CD146 In Cerebrospinal Fluid Might be a Biomarker of Severity of Anti-N-Methyl-D-Aspartate Receptor Encephalitis

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Disruption of the blood–brain barrier (BBB) is an important pathophysiological process of anti-N-methyl-D-aspartate receptor (anti-NMDAR) encephalitis. A recent multi-center study showed that soluble (s) CD146 is a potential biomarker for monitoring early BBB damage and central nervous system inflammation.

This data suggested that higher expression of CSF sCD146 correlated with more serious neurological damage. Therefore, levels of CSF sCD146 may represent a promising indicator for monitoring disease and optimizing clinical treatment decisions in the early stages of anti-NMDAR encephalitis.

Anti-N-Methyl-D-Aspartate Receptor Encephalitis: A Detailed Review of the Different Psychiatric Presentations and Red Flags to Look for in Suspected Cases

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This review aimed to discuss the different ways anti-NMDAR encephalitis could present and manifest on the psychological, mainly, and the neurological spectrum of signs and symptoms. The signs and symptoms expressed by anti-NMDAR encephalitis patients can be categorized into psychosis (represented by delusions and hallucinations), catatonia, seizures, speech and movement abnormalities, and autonomic instability, in addition to cognitive dysfunction.

The temporal association between those signs and symptoms varied among cases; first presentations tend to overlap with psychiatric diagnoses, especially on the schizophrenic spectrum. Catatonia is one of the disease hallmarks as well. Movement disorders are commonly seen among the anti-NMDAR encephalitis pediatric population. The autoimmune link between anti-NMDAR encephalitis and schizophrenia has been described before; antibody-positive patients go on to develop neurological manifestations later on. Anti-NMDAR encephalitis can also present with affective manifestations. Narcolepsy and hypersomnia as manifestations of anti-NMDAR encephalitis have been described, as well as the association between alcohol or drug abuse and NMDAR encephalitis. There are some clinical red flags that could be of use to make the diagnosis.

Physicians trying to familiarize themselves with this diagnosis, this is a good place to start. The review included a general scheme that covers almost all aspects of the psychiatric presentation, similarities, and differences in contrast to other differential diagnoses, in addition to clinical pearls that aid diagnosis. However, this paper did not discuss the different ways this disorder presents in pediatrics; hence, this remains an area future reviews might be more inclusive of.

Anti-NMDAR encephalitis for psychiatrists: the essentials

Highly Recommended

Learning objectives:

(a) What is autoimmune encephalitis?

(b) What is anti-NMDAR encephalitis?

(c) When should anti-NMDAR encephalitis be suspected?

(d) How should suspected anti-NMDAR encephalitis be investigated?

(e) How should the practical management and treatment of anti-NMDAR encephalitis be approached?

(f) What is the prognosis for anti-NMDAR encephalitis?

Anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis predominantly presents with psychiatric symptoms. Psychiatrists need to be alert to this diagnostic possibility, especially in female adolescents and young adults, as exemplified by the real (de-identified) case outlined below. Earlier diagnosis and immunotherapy improves long-term outcomes. Collaboration with neurology colleagues is essential for optimal care. ‘Red flags’ for autoimmune encephalitis and ‘diagnostic clues’ act as helpful aide memoires for this uncommon condition. The gold standard for testing is the detection of NMDAR antibodies in cerebrospinal fluid, but serum can be tested as a more accessible (but less reliable) preliminary step. The results of routine blood tests, magnetic resonance imaging of the head and electroencephalograms can be normal or show non-specific changes. Diagnostic criteria exist to define probable and definite cases. Immunotherapy for anti-NMDAR encephalitis is effective for many patients, but recovery is prolonged and relapses can occur.

Anti-N-Methyl-D-Aspartate Receptor Encephalitis with Serum Anti-Thyroid Antibodies: A Case Report and Literature Review

We report a rare case in which an autoimmune involvement of thyroid gland was concurrent with an anti-NMDArE. It would be useful for clinical practice to clarify whether the presence of anti-thyroid antibody an characterize the clinical course, prognosis, and response to treatment of the idiopathic type of anti-NMDArE.

Capgras syndrome and confabulation unfurling anti NMDAR encephalitis with classical papillary thyroid carcinoma: First reported case

A case of ANMDARE with intriguing presentation of Capgras syndrome and confabulations, who was found to have a malignant papillary thyroid carcinoma, which has been rarely reported.

Bortezomib in anti-N-Methyl-d-Aspartate-Receptor (NMDA-R) encephalitis: A systematic review

Highlights

• Proteasome inhibitor bortezomib could have a role in anti NMDA receptor encephalitis.

• Half of patients treated with bortezomib improved, but side effects were common.

• Larger and more homogeneous cohorts should be included in future trials.

Clinical Features and Outcomes of Anti-N-Methyl-d-Aspartate Receptor Encephalitis in Infants and Toddlers

Results: A total of 41 patients (age range: six to 34 months; median age: 23 months; female: 19) were enrolled in this study. Nineteen (46%) patients exhibited classical anti-NMDAR encephalitis, whereas 22 (54%) patients exhibited anti-NMDAR encephalitis after viral encephalitis. There was a high presentation of movement disorders (100%), developmental regression (90%), abnormal behaviors (90%). All patients were administered first-line therapy, with only 17% of them being administered second-line immunotherapy. Two patients succumbed to the disease, whereas none of them relapsed. At the long-term follow-up (more than one year), 20 of 35 (57%) exhibited satisfactory outcomes (modified Rankin Scale ≤2). Compared with patients with classical anti-NMDAR encephalitis (n = 18), patients after viral encephalitis (n = 17) were more likely to have worse clinical outcomes. They exhibited a higher modified Rankin Scale/Pediatric Cerebral Performance Category score and more frequent seizures. A predictor of poor outcome was presentation after viral encephalitis (odds ratio 35.7, 95% confidence interval 4.64 to 275.03, P = 0.001).

Conclusion: Anti-NMDAR encephalitis in infants and toddlers clinically presents with movement disorders, developmental regression, and abnormal behaviors. Interestingly, this group had a higher proportion of patients after viral encephalitis, which is regarded as the only risk factor for poor outcomes.

The Insula Is a Hub for Functional Brain Network in Patients With Anti-N-Methyl-D-Aspartate Receptor Encephalitis

This research aimed to assess changes in the functional brain network in patients with anti-NMDAR encephalitis and whether these changes lead to cognitive impairment.

Conclusion: This research describes topological abnormalities in the functional brain network in anti-NMDAR encephalitis. These results will be conducive to understand the structure and function of the brain network of patients with anti-NMDAR encephalitis and further explore the neuropathophysiological mechanisms.

Clinical characteristics, treatment and long-term prognosis in patients with anti-NMDAR encephalitis

Both paediatric and adult patients presented with similar clinical features but the paediatric population had female preponderance. The functional outcome and number of relapse were comparable in both the paediatric and adult groups. Patients with parenchymal changes on MRI and abnormal EEG showed poorer response compared to those with normal MRI and/or EEG at the onset. Patients have lesser severity of symptoms at relapse than in the first episode. An early diagnosis and treatment are essential for better long-term functional outcome.

Clinical Study of Autonomic Dysfunction in Patients With Anti-NMDA Receptor Encephalitis

Autonomic dysfunction is a common symptom of anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis; however, it has been poorly researched.

Conclusion: Sinus tachycardia is the most common type of autonomic dysfunction in anti-NMDAR encephalitis. Compared to patients without autonomic dysfunction, those with autonomic dysfunction had a higher incidence of epilepsy, involuntary movements, decreased consciousness, pulmonary infections, abnormal liver function, ICU admissions, and mechanical ventilation; moreover, the severity of the disease was greater, and their prognosis worse. Therefore, such patients require intensive immunotherapy.

Mature ovarian teratoma associated with anti-N-Methyl-Daspartate receptor encephalitis: A case report

Up to 77% of NMDAR encephalitis patients are initially seen by psychiatrists and misdiagnosed with new-onset schizophrenia or bipolar disorder.3 Given approximately 60% of cases in women are caused by an ovarian teratoma, it is essential to rule out an ovarian mass when anti-NMDA encephalitis is the differential diagnoses. If untreated, the disease can lead to lethal hypoventilation, autonomic instability, and catatonia.

Decreased inflammatory cytokine production of antigen-specific CD4+ T cells in NMDA receptor encephalitis

The present study is to our knowledge the first direct ex vivo quantitative and qualitative analysis of circulating autoreactive T cells involved in NMDA receptor encephalitis. Our data reveal the unexpected finding that NR1-reactive CD4+ TH cells are not elevated but rather reduced in patients with NMDAR-E and that these cells produce less inflammatory cytokines.

Based on our findings, reduced NR1-specific TH cell numbers are not related to therapeutic immunosuppression.

Our study further suggests that a similar reduction of TH cell reactivity toward the disease-defining antigen is not a universal finding in the still expanding types of autoimmune encephalitis. We unexpectedly observed a reduced number and profound disease-specific functional alterations of NR1-reactive TH cells in patients with NMDAR-E that were not related to immunotherapies, challenging the idea that increased pro-inflammatory NMDAR-reactive TH cells contribute to disease pathogenesis.

Association of Polymorphisms in Inflammatory Cytokines Encoding Genes With Anti-N-methyl-D-Aspartate Receptor Encephalitis in the Southern Han Chinese

Conclusions: We found that patients with anti-NMDAR encephalitis exhibit a distinct immunological profile, and we found that the decreased frequency of G allele in IL-1β rs16944 showed a protective role for anti-NMDAR encephalitis in the Southern Han Chinese.

IRAK4 Deficiency Presenting with Anti-NMDAR Encephalitis and HHV6 Reactivation

Only a few infantile cases with anti-NMDAR encephalitis have been so far reported. We identified a 10-month-old boy with IRAK4 deficiency presenting with anti-NMDAR encephalitis and human herpes virus 6 (HHV6) reactivation. The diagnosis of IRAK4 deficiency was confirmed by the identification of compound heterozygous mutations c.29_30delAT (p.Y10Cfs*9) and c.35G>C (p.R12P) in the IRAK4 gene, low levels of IRAK4 protein expression in peripheral blood, and defective fibroblastic cell responses to TLR and IL-1 (TIR) agonist. We established a novel NF-κB reporter assay using IRAK4-null HEK293T, which enabled the precise evaluation of IRAK4 mutations. Using this system, we confirmed that both novel mutations identified in the patient are deleterious. Our study provides a new simple and reliable method to analyze IRAK4 mutant alleles. It also suggests the possible link between inborn errors of immunity and early onset anti-NMDAR encephalitis.

New Onset Focal Seizure Clusters in Children: Expanding the Spectrum of Anti NMDAR Encephalitis

This is the first series of Anti NMDAR encephalitis presenting as new onset seizure clusters in children. Unlike the existing literature, these children did not develop any other symptoms. We propose that focal encephalitis could be the reason for this monosymptomatic presentation.

Cycloid psychosis as a psychiatric expression of anti-NMDAR encephalitis. A systematic review of case reports accomplished with the authors' cooperation

Objective: We reviewed the psychotic symptoms of anti-NMDA receptor encephalitis (NMDARE) to differentiate its presentation from those found in a primary psychiatric disorder. We hypothesized that the cycloid psychosis (CP) phenotype would be a frequent clinical presentation in the psychiatric phase of NMDARE. The complete CP phenotype was frequently the expression of psychotic symptoms in NMDARE.

Conclusion: We suggest that patients with a first psychotic episode who initially exhibit the CP phenotype should undergo CSF analysis to determine whether antibodies against neuronal cell surface or synaptic receptors are present to rule out a possible diagnosis of autoimmune encephalitis.

Clinical and electrographic features of persistent seizures and status epilepticus associated with anti-NMDA receptor encephalitis (anti-NMDARE)

Measured electrographic features were not associated with persistent seizures. Seizures associated with anti-NMDARE are primarily focal seizures originating in the frontal lobes. Younger patients may be at increased risk of epileptogenesis and status epilepticus. Continuous EEG monitoring helps identify subclinical seizures, but specific EEG findings may not predict the severity or persistence of seizures during hospitalization.

Neurocritical care for Anti-NMDA receptor encephalitis

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In this review, we summarize the clinical presentations of the acute stage of anti-N-methyl-d-aspartate (NMDA) receptor encephalitis and the neurocritical care strategy in intensive care units. Anti-NMDA receptor encephalitis has characteristic clinical features and is predominantly seen in young adults and children. Most patients have five stages of clinical presentation, including a prodromal phase, psychotic and/or seizure phase, unresponsive and/or catatonic phase, hyperkinetic phase, and gradual recovery phase.

This article summarizes the presentation of anti-NMDAr autoimmune encephalitis in young adults and children. It discusses the stages and course that the disease often follows in the intensive care unit

Finding the Cause of Psychosis: A Challenging Case of Anti-NMDAR Encephalitis

anti-NMDAR encephalitis is a very rare entity in male patients, especially in those without any underlying malignancies. However, physicians should consider this diagnosis as a differential in any young patient presenting with unexplained neuropsychiatric symptoms. Younger patients who present with an initial episode of bizarre behavior should be assessed to rule out major organic causes, as delay in diagnosis leads to poor patient outcomes. Although the literature suggests a predominance of anti-NMDAR encephalitis occurs in women with ovarian teratomas, this case demonstrates that the diagnosis should also be considered in men without any signs and symptoms of a neoplastic process, and a thorough workup for the infectious source should be conducted

Anti-NMDAR encephalitis is associated with IRF7, BANK1 and TBX21 polymorphisms in two populations

Highly Recommended In the past there was one identified portion of a gene HLA which was susceptible to allowing autoantibodies targeting a subunit of the NMDAr receptor, this study looked at different groups of patients with AE along with other people and was able to identify 3 other unrelated causes related to NMDAr, it showed that B cell activation, TH1 response, virus infection and type 1 IFN signaling pathway were also involved in the pathogenesis or the abnormalities that start the disease process, so thus far 4 subunit or smaller portions of the process of the disease beginning are identified

Clinical outcomes of pediatric Anti-NMDA receptor encephalitis

Highlights Despite the favorable outcome, mild cognitive and language deficits can persist in pediatric anti-NMDAR encephalitis patients.

Younger onset patients (<12 years) tended to recover more slowly than older onset patients (12–18 years).
Clinical outcome measure should be specific to delineate the differential effect on outcome according to age of onset.

Long-term persistence of NMDAR antibodies after encephalitis with de novo occurrence of demyelinating disorder

The issue of persistence of NMDAR antibodies after encephalitis is not fully elucidated and their relationship with demyelinating disorders has been suggested. This case report provides evidence of long-term persistence of NMDAR antibodies even 15 years after the encephalitis and raises the suspicion of a possible causal relationship between NMDAR antibodies and demyelinating disorders in the form of multiple sclerosis.

Neuropsychiatric symptoms and caregivers' distress in anti-N-methyl-D-aspartate receptor encephalitis

Objective: To explore the characteristics of psychiatric symptoms and caregivers’ distress in anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis. 72 patients with anti-NMDAR encephalitis were investigated in Peking from 2011 to 2018.

Results: A highly positive correlation existed between severity scores of neuropsychiatric symptoms and caregiver distress.

Conclusion: Patients with anti NMDAR encephalitis often present severe neuropsychiatric symptoms, which aggravate the burden on caregivers. Immunotherapy significantly improves neuropsychiatric symptoms, and thus reduces the distress of caregivers.

A Pounding Problem: A Case of Recurrent Headache Caused by Anti-NMDA Receptor Encephalitis

Case report: A previously healthy 25-year-old woman presented on several occasions to the Emergency Department with a severe pounding headache that initially responded well to treatment. She later developed signs consistent with meningoencephalitis along with altered mental status and neuropsychiatric changes. She was diagnosed with anti-NMDAR encephalitis after hospitalization. WHY SHOULD AN EMERGENCY PHYSICIAN BE AWARE OF THIS?: Anti-NMDAR encephalitis is an under-recognized condition with diverse presentations. Recurrent headaches that improve with treatment may be an early sign of this disorder. Anti-NMDAR encephalitis should be considered in patients with recurrent undifferentiated headaches, and an appropriate work-up should be performed. Early recognition and diagnosis of this condition is critical to optimize favorable patient outcomes, as delays to diagnosis may lead to fatalities and long-term neurologic sequelae.

Delta brush variant: A novel ictal EEG pattern in anti‐NMDAR encephalitis

Key Points

  • Delta brush variance is a novel ictal electroencephalogram pattern in anti‐NMDAR encephalitis.

  • Delta brush variance is characterized as generalized delta rhythm with fast spike activity riding on it.

  • Delta brush variance is derived from extreme delta brush in the florid phase of the disease.

  • Cortex‐subcortical area interaction may contribute to the evolution between delta brush variance and extreme delta brush.

Autoimmune encephalitis mediated by B-cell response against N-methyl-d-aspartate receptor

This study demonstrates that the B-cell response can lead to an autoimmune reaction against NMDAR that drives encephalitis-like behavioural impairments. It also provides a relevant platform for dissecting encephalitogenic mechanisms in an animal model, and enables the testing of therapeutic strategies targeting the immune system in anti-NMDAR encephalitis.

Study of B Cell Repertoire in Patients With Anti-N-Methyl-D-Aspartate Receptor Encephalitis

To our knowledge, this is the first study that revealed a heavy chain common clone and the shared clonotypes appeared in most enrolled patients with anti-NMDAR encephalitis. The major findings of our research include: first, a heavy chain common clone of B cell receptor appeared in 11 of 12 patients, but not in healthy people or patients with anti-LGI1 encephalitis, MS, or NMOSD; second, four shared clonotypes presented among different patients with anti-NMDAR encephalitis; third, the V-D-J gene family usage preference of patients with anti-NMDAR encephalitis were different from healthy people, although they have similar antibody mutation rate.

 Meanwhile, our method had been proved by this study to be effective for studying low-throughput single-cell immune repertoire, which could be applied in diseases like the anti-NMDAR encephalitis.

The most common heavy chain clone IGHV1-18*04, IGHD1-26*01/IGHD2-2*03/ IGHD2-8*01,IGHJ3*02_(CDR3)ARVGSKYGFETFDI appeared in 11 of 12 patients including PA24 (with ovarian teratoma).

Chorea in children: etiology, diagnostic approach and management

Sydenham chorea is considered the most common cause of acute childhood-onset chorea, but its prevalence has decreased in Western countries. However, in younger children other etiologies such as dyskinetic cerebral palsy, anti-NMDAR receptor encephalitis, other autoimmune conditions, or mutations in NKX2-1, ADCY-5, FOXG1, GNAO1, GPR88, SLC2A1, SQSTM1, ATP8A2, or SYT-1 should be considered. In this manuscript, we review the main causes, diagnosis and management of chorea in children.

Validation of the NEOS score in Chinese patients with anti-NMDAR encephalitis

Results: Among the 111 patients with anti-NMDAR encephalitis recruited from 364 potentially eligible participants, 87 (78.4%) had good functional status at 1 year, whereas the remaining 24 (21.6%) had poor functional status. The AUC of the NEOS score for 1-year poor functional status was 0.86 (95% CI 0.78-0.93, p < 0.001). The increased NEOS was associated with higher risk of 1-year poor functional status in patients with anti-NMDAR encephalitis.

Conclusions: The NEOS score is considered a reliable predictor of the risk of 1-year poor functional status in Chinese patients with anti-NMDAR encephalitis. This score could help to estimate the velocity of clinical improvement in advance.

Not Your Average Seizure: A Case of N-Methyl-D-Aspartate Receptor Encephalitis and Review of Literature

Highly Recommend

A case report we recommend for the discussion section. A complete and easy to understand review of anti-NMDAr, diagnosis, treatment, and onset presentation.

Influencing electroclinical features and prognostic factors in patients with anti-NMDAR encephalitis

Anti-N-methyl-d-aspartate receptor (anti-NMDAR) encephalitis is an autoimmune disease associated with IgG antibodies against the NR1 subunit of the NMDA receptor1,2. Patients develop subacute psychiatric symptoms, memory loss, movement disorders and seizures. Seizures can occur at any stage but most commonly manifest early

Anti-NMDA Receptor Encephalitis

Nice introduction to anti-NMDAr AE. We recommend this paper for patients and clinicians who are just being introduced to anti-NMDAr

Olanzapine and Lorazepam Used in the Symptomatic Management of Excited Catatonia Secondary to Anti-N-Methyl-D-Aspartate Receptor Encephalitis

This case illustrates the need to consider autoimmune encephalitis in cases of catatonia. It also presents a case in which symptoms of anti-NMDA receptor encephalitis potentially remitted without immunotherapy or mass resection.

Anti-NMDA receptor encephalitis associated with ovarian tumor: the gynecologist point of view

Objectives: Primary objective of our work was to present a review in a very schematic and practical way for gynecologists, about the data on anti-NMDAr encephalitis in terms of epidemiology, clinical symptomatology, treatment and prognosis. The second objective was to propose a decision tree for gynecologists to guide them, in collaboration with neurologists and anesthesiologists, after the diagnosis of NMDAr encephalitis associated with an ovarian mass.

Conclusion: Anti-NMDA antibody encephalitis should not be ignored by gynecologists whose role in management is central. Ovarian teratoma is common, especially in women of reproductive age. Complementary examinations in search of an ovarian teratoma must, therefore, be systematic to envisage a possible surgical excision that may improve patient prognosis.

Refractory anti-NMDAR encephalitis successfully treated with bortezomib and associated movements disorders controlled with tramadol: a case report with literature review

We report a case of severe anti-NMDAR encephalitis in a 29-year-old woman, not responsive to first- and second-line treatments, with persistent involuntary motor manifestations. Starting three months after symptom onset, four cycles of bortezomib have been administered; subsequently we observed a progressive improvement of neurological status. Meanwhile, motor manifestations were controlled after the administration of tramadol, a non-competitive NMDA receptor antagonist.

Evaluation of the proposed anti‐N‐Methyl‐D‐Aspartate receptor encephalitis clinical diagnostic criteria in psychiatric patients

This study retrospectively assessed the proposed criteria in patients presenting to psychiatric services for the first time with known anti‐NMDAR antibody status.

Results: The proposed criteria were applied to 641 cases (500 antibody positive, 141 antibody negative), demonstrating a sensitivity which increased from around 19% after 1 week to 49% by the point of discharge. Specificity was 100% at both time points. The mean average time to become positive using the proposed criteria was 19.5 days compared to 34.9 days for return of antibody testing.

Conclusions: High specificity of the proposed criteria, seen in this study, suggests that cases which are positive can be considered for expedited commencement of treatment. However, if clinical suspicion is high despite criteria being negative, it is essential to test CSF for anti‐NMDA antibody.

Anti-N-Methyl-D-Aspartate-Receptor Encephalitis: A 10-Year Follow-Up

Anti-N-methyl-D-aspartate-receptor (NMDA-R) encephalitis is an autoimmune disease of the brain first described in 2007. The aim of this paper is to present a 10-year follow-up case history.
Conclusion: Anti-inflammatory treatment led to almost full recovery with persistent disappearance of catatonic symptoms; however, a dysexecutive syndrome led to ongoing relevant problems with good response to low-dose atypical neuroleptics and cognitive behavioral therapy (CBT). The patient had persistent EEG alterations that indicated continuing neuronal network instability. Therefore, the case demonstrates the importance of multidisciplinary outpatient treatment following acute therapy for anti-NMDA-R encephalitis in patients with ongoing psychiatric deficits. For the symptomatic treatment of executive dysfunctions, “classical” psychiatric treatment may be helpful in the course of the disease.
 

Anti-NMDA receptor encephalitis in a psychiatric Covid-19 patient: A case report

First report of a patient with anti-NMDAR encephalitis 3 weeks after hospital admission for #COVIDー19

 

Current Progress on Assessing the Prognosis for Anti-N-Methyl-D-Aspartate Receptor (NMDAR) Encephalitis

At present, research on the prognosis of anti-NMDAR encephalitis remains limited. We summarized the relevant research on the anti-NMDAR encephalitis, with a focus on factors affecting prognosis, such as disease pathogenesis, clinical manifestations, auxiliary examination, and treatments. Autonomic dysfunction and related complications may affect long-term prognosis, with cognitive impairment being the main dysfunction encountered during the long-term recovery of patients with anti-NMDAR encephalitis. When the abovementioned clinical manifestations appear, they should be actively treated as soon as possible in order to improve the chances for a positive outcome.

Anesthetic care for patients with anti-NMDA receptor encephalitis

None of the patients exhibited adverse events, including hemodynamic instability, thermoregulatory problems, or respiratory events perioperatively. Postoperatively, there was no observed deterioration in clinical status attributed to anesthetic care.

 

Sleep Disorders In Anti-NMDAR Encephalitis

Objective: To describe the sleep disorders in anti-NMDA receptor encephalitis (anti-NMDARe)

Background: Patients with anti-NMDARe often have sleep problems. The severity and complexity of the accompanying neuropsychiatric symptoms, mask these sleep disorders, complicate their assessment, and explain the lack of prior studies

Conclusions: Sleep disturbances are frequent in anti-NMDARe. They show a temporal pattern (predominantly insomnia at onset; hypersomnia during recovery), associate with behavioral and cognitive changes, and can occur with confusional arousals during NREM sleep.

 

Clinical characteristics of anti - N - methyl - D - aspartate receptor encephalitis in children

The clinical data of 42 cases of anti-NMDAR encephalitis at Department of Pediatrics, Second Xiangya Hospital, Central South University from January 2015 to March 2018 were collected. The clinical features and followed-up outcomes were analyzed retrospectively. CONCLUSIONS: Seizures, mental and behavior disorder, dyskinesias, speech disorder and autonomic instability are common clinical manifestations of anti-NMDAR encephalitis in children. The effect of immunotherapy is significant, and the time to start immunotherapy and the severity of the disease

Catatonia in Anti-N-Methyl-D-Aspartate (NMDA) Receptor Encephalitis Misdiagnosed as Schizophrenia

The aim of this case report is to highlight the importance of close surveillance for neuropsychiatric symptoms, especially catatonia, and to recognize autoimmune encephalitis in the differential diagnosis of psychotic disorders with neurological symptoms and resistance or intolerance to antipsychotics. A prompt diagnosis will contribute to a faster onset of therapy and an overall improvement in prognosis.

Cerebral functional activity and connectivity changes in anti-N-methyl-D-aspartate receptor encephalitis: A resting-state fMRI study

 Study highlight that decreased spontaneous neural activities and abnormal FC exhibited in the patients with anti-NMDAR encephalitis, which may participate in the process of cognition and emotion deficits. These results may help to elucidate the clinical radiological contradictions in anti-NMDAR encephalitis and contribute to deeper understanding of the pathophysiological mechanism of the disease.

Pregnancy outcomes in anti-NMDA receptor encephalitis

  Overall, the current findings and previously reported cases suggest that in pregnant patients with anti-NMDAR encephalitis, fetal exposure to maternal NMDAR antibodies infrequently associates with overt neurologic deficits.

A Metabolic Brain Pattern Associated With Anti-N-Methyl-D-Aspartate Receptor Encephalitis

 
In this study, we aim to provide supporting evidence that brain 18F-FDG-PET may be helpful in identifying likely patterns of regional brain glucose metabolism.

Co-occurence of Anti-N-Methyl-D-Aspartate Receptor Encephalitis and Anti-myelin Oligodendrocyte Glycoprotein Inflammatory Demyelinating Diseases: A Clinical Phenomenon to Be Taken Seriously

Anti-N-methyl-D-aspartate receptor (NMDAR) immunoglobulin G antibodies which exist on myelin sheaths, composed of oligodendrocytes, especially target GluN1 subunits and are highly characteristic of anti-NMDAR encephalitis which is a newly recognized autoimmune encephalitis (AE) characterized by psychiatric symptoms, behavioral abnormalities, seizures, cognitive impairment and other clinical symptoms.

Human gestational NMDAR autoantibodies impair neonatal murine brain function

The data collectively support a model in which asymptomatic mothers can harbor low-level pathogenic human NR1 AB that are diaplacentally transferred causing neurotoxic effects on neonatal development.

Neuronal NMDAR Currents of the Hippocampus and Learning Performance in Autoimmune Anti-NMDAR Encephalitis and Involvement of TNF-α and IL-6

The psychopathology of NMDAR-antibody encephalitis in adults

Mouse model of anti-NMDA receptor post–herpes simplex encephalitis

Anti-NMDAR encephalitis with concomitant varicella zoster virus detection and nonteratomatous malignancy

EEG findings in NMDA encephalitis – A systematic review

Predictive value of electroencephalography in anti-NMDA receptor encephalitis

The Movement disorder associated with NMDAR antibody-encephalitis is complex and characteristic: an expert video-rating study

Anti–NMDAR encephalitis in a patient with Crohn disease receiving adalimumab

Membrane-bound and soluble forms of an NMDA receptor extracellular domain retain epitopes targeted in auto-immune encephalitis

Anti-N-methyl-d-aspartate receptor encephalitis in children: Incidence and experience in Hong Kong

Ion channels in EEG: isolating channel dysfunction in NMDA receptor antibody encephalitis

Early identification of anti-NMDA receptor encephalitis presenting cerebral lesions in unconventional locations on magnetic resonance imaging

B cell-based therapies in CNS autoimmunity: differentiating CD19 and CD20 as therapeutic targets

Anti-N-methyl-D-aspartate receptor encephalitis associated with acute Toxoplasma gondii infection

Anti-N-methyl-d-aspartate receptor encephalitis in children: Incidence and experience in Hong Kong

Treatable Immune System Disorder Could Be Mistaken For Schizophrenia or Bipolar Disorder

Serum cystatin C and anti-N-methyl-D-aspartate receptor encephalitis

Suicidality is a common and serious feature of anti-N-methyl-D-aspartate receptor encephalitis

Arterial spin labeling perfusion imaging demonstrates cerebral hyperperfusion in anti-NMDAR

Dynamic disorganization of synaptic NMDA receptors triggered by Abs from psychotic Pts

Serum complement levels in anti-N-methyl-D-aspartate receptor encephalitis

Successful combined targeting of B- and plasma cells in treatment refractory anti-NMDAR

Superficial white matter damage in anti-NMDA receptor encephalitis

NMDA- indications for testing and how it is diagnosed

Persistence of parenchymal and perivascular T-cells in treatment-refractory NMDAr

UPENN~ Anti-NMDAR Encephalitis What is anti-NMDA Receptor Encephalitis?

Comparative Outcomes in Children and Adults with anti-NMDA Receptor Encephalitis

NMDA-receptor antibodies alter cortical microcircuit dynamics

Anti-N-Methyl-D-aspartate Receptor Encephalitis: A Severe, Potentially Reversible Autoimmune Encephalitis

Cognitive outcomes following anti-NMDAr encephalitis: A systematic review

Ovarian teratoma development after anti-NMDA receptor encephalitis treatment

Herpes simplex virus-induced anti-NMDA receptor encephalitis: a systematic literature review

First reported cases of anti-NMDAr encephalitis in Vietnamese adolescents and adults

Bortezomib for Treatment of Anti-NMDA Receptor Encephalitis

Anti-NMDA Receptor Antibody positivity A Systematic Review of the Literature

NMDAr presents in atypical types

Bortezomib for treatment of therapy-refractory anti-NMDA receptor encephalitis

Anti-NMDA receptor encephalitis: An emerging differential diagnosis in the psychiatric community

Potentially effective off-label: Bortezomib Treatment for Patients With NMDAr

Not So Rare But rarely Dx: From Demonic Possession to Anti-NMDr

Promising results from animal model research in NMDAr w/ many potential applications

New onset Headache in Patients W/ AE associated w/ NMDAr antibodies

Structural Hippocampal Damage Following Anti-N-Methyl-D-Aspartate Receptor Encephalitis

Anti-NMDA Receptor Encephalitis: A Need for Increased Awareness Among Psychiatrists

Intravenous methylprednisolone versus therapeutic plasma exchange for treatment of NMDA

Anti-NMDA-receptor encephalitis: A neuropsychiatric syndrome associated with ovarian teratoma

Molecular Pathogenesis of Anti-NMDAR Encephalitis

anti-NMDAr concomitant with multifocal subcortical white matter lesions on MRI

Blood and CSF test for NMDA: Test results for CSF is 100% but Blood 87.5-94.7%.

Overlapping demyelinating syndromes and anti-NMDA receptor encephalitis

Increased prevalence of diverse NMDAr antibodies in patients with an initial dx of schizophrenia

Treatment and prognostic factors for long-term outcome in patients with anti-N-Methyl-D-Aspartate (NMDA) receptor encephalitis: a cohort study

Association between the NMDA glutamate receptor GRIN2B gene and obsessive–compulsive disorder

N-methyl D-aspartate receptor encephalitis: A new addition to the spectrum of autoimmune encephalitis

An overview of NMDA receptor physiology and clinical significance

Severity of Hospitalized Children with Anti-NMDAR Autoimmune Encephalitis

Random forest regression model presented that the overall prediction power of severity reached 0.806, among which the number of polynucleated cells in cerebrospinal fluid contributed the most. Potential pathogenic causes exhibited that the proportion of mycoplasma was the highest, followed by Epstein-Barr virus. Conclusion: Our findings provided evidence for early identification of autoimmune encephalitis in children, especially in severe cases.

Human Umbilical Cord Mesenchymal Stem Cells for Severe Neurological Sequelae due to Anti-N-Methyl-d-Aspartate Receptor Encephalitis: First Case Report

The first case of a study describing a child who recovered from severe neurological sequelae due to anti-NMDA receptors after three infusions of allogeneic.

This case report suggests thatumbilical cord–derived mesenchymal stem/stromal cells (UC-MSCs) therapy may ameliorate severe neurological sequelae due to anti-NMDA receptor encephalitis. A study with a larger sample size should be performed to evaluate the efficacy of UC-MSCs for AE as well as severe neurological sequelae due to AE.

Clinical Characteristics of Anti-N-Methyl-d-Aspartate Receptor Encephalitis Overlapping with Demyelinating Diseases: A Review

NMDARe may occur simultaneously or sequentially with different demyelinating diseases. Compared with the age of patients with classical NMDARe, patients with overlapping MS or AQP4-positive NMOSD tended to be slightly older. They also had lower incidence of ovarian teratoma. Overlapping syndrome patients frequently presented atypical symptoms and/or MRI abnormalities when compared with classical NMDARe. MRI scans and detection of OB, AQP4, and MOG antibodies are helpful for identifying overlapping syndromes. Favorable outcome was observed in patients overlapping with MOGAD, but no robust comparison can be drawn with the patients overlapping with AQP4-Ab-positive NMOSD and MS regarding the small number of available data. Long-term follow-up investigation is needed because of the long interval between NMDARe and demyelinating diseases.

Ovarian teratoma related anti-N-methyl-D-aspartate receptor encephalitis: A case series and review of the literature

The six patients enrolled for this study presented with typical symptoms resulting in a diagnosis of ovarian teratoma induced anti-NMDAR encephalitis. Appropriate interventions led to a positive outcome in all the patients, with five of six patients reporting full recovery and the sixth patient recovering with a few deficits. No death was recorded. The literature survey comprising of 155 patients cases across 130 case reports of anti-NMDAR encephalitis clearly indicated an upward trend in the reports/diagnosis in China, particularly in the surveyed time from 2014 through 2019. The majority of patients (150/155) underwent surgical intervention resulting in positive outcome. No treatment intervention was mentioned for one case while the four patients who were not surgically operated succumbed to the disease.

Anti-N-Methyl-D-Aspartate Receptor Encephalitis: Neuropsychiatric and Multidisciplinary Approach to a Patient Not Responding to First-Line Treatment

This case report presents the clinical vignette of a 30-year-old male patient, who presented with altered sensorium, disorganized behavior, and perceptual disturbance. Initially, he was put on standard psychotropic medications that did not help. After a careful review, the patient was diagnosed with anti-NMDA receptor encephalitis endorsed by the identification of CSF antibodies. There are several points to be highlighted within this case study. Initially, clinical suspicion and keeping a broad differential, despite what may appear as exclusively psychiatric symptoms, are important to prevent any delay in the appropriate testing or procedures that can hone in on the diagnosis. Successful treatment hinges on prompt induction of first-line treatment with steroids, and potentially second-line therapy with adjunct IVIG or immunosuppressants. Communication within the multidisciplinary approach, often combining clinicians from a myriad of subspecialties, is paramount to positive patient outcomes. Clinicians should remain alert and suspicious regarding the possibility of anti-NMDA receptor encephalitis in a patient with predominantly disorganized or psychotic symptoms who may not be responding to conventional antipsychotic therapy.

The clinical relevance of serum versus CSF NMDAR autoantibodies associated exclusively with psychiatric features: a systematic review and meta-analysis of individual patient data

Background

A variety of psychiatric syndromes are associated with NMDAR autoantibodies; however, their clinical relevance when only present in the serum is unclear. We explored whether patients with CSF NMDAR autoantibodies could be distinguished from patients with serum-only NMDAR autoantibodies.

Conclusion

Patients with an isolated psychiatric syndrome with CSF NMDAR autoantibodies can potentially be distinguished from those with serum-only NMDAR autoantibodies based on clinicodemographic and investigation findings.

Refractory Anti-NMDA Receptor Encephalitis in Early Pregnancy

The patient was treated with an escalating immunotherapy regimen from 11 to 15 weeks of gestation, including steroids, plasma exchange, IV immunoglobulins, and rituximab, with no clinical response. At 16 weeks of gestation, she received cyclophosphamide with clinical improvement after 4 weeks. She subsequently gave birth to a healthy, term baby boy, who continued to do well at the follow-up.

Case report of anti-NMDA receptor encephalitis in a 24-year-old female: an uncommon presentation

In conclusion, anti-NMDA receptor encephalitis is a rare disease underdiagnosed due to the variability of the initial symptoms. In general, emergency physicians are not familiar with this disease. In case of patient with suggestive clinical picture of anti NMDAR encephalitis presenting to the emergency room, lumbar puncture should be performed as soon as possible to look for CSF pleocytosis and oligoclonal bands. Abnormal EEG findings and brain MRI can help to not delay the initiation of immunosuppressive treatment. The diagnosis is confirmed by the detection of antibodies anti-NMDAR in the CSF. The possibility of this pathology should be taken into account as a differential diagnosis for patients presenting with acute psychosis or encephalitis before diagnosing a psychiatric illness.

Disease progression and brain atrophy in NMDAR encephalitis: Associated factor & clinical implication

INTERPRETATION: In NMDAR encephalitis, cerebellar volume reduction was progressive once developed. Cerebellar volume reduction might reflect disease burden and extent of progression and be associated with poor outcomes in multiple functional domains.

Case Report: Cotard's Syndrome in Anti-N-methyl D-aspartate (NMDA) Receptor (Anti-NMDAR) Encephalitis

Cotard’s syndrome is uncommon psychopathology among patients with psychotic illnesses. Limited cases had been reported regarding the occurrence of this syndrome in anti-NMDAR encephalitis which itself is a relatively new disease that often presents with florid psychotic symptoms. This poses difficulties in differentiating it from a primary psychiatric illness. Late recognition of anti-NMDAR encephalitis can lead to death as it can progress to autonomic instability in its natural course of illness

This case report sheds some useful insights on the relatively rare co-occurrence of Cotard’s syndrome and anti-NMDAR encephalitis. Even though experiencing treatment-related complications and receiving only a week of antipsychotic therapy, the patient managed to make a full recovery. She did not require long-term antipsychotic medication to remain well. The main good prognostic factor in this patient is the early suspicion of this condition. 

Atypical anti-NMDA receptor encephalitis associated with varicella zoster virus infection

there are very few reports that has linked a varicella zoster virus (VZV) reactivation with anti-NMDAR encephalitis. In this report, we describe a case of a 57-year-old man presented with atypical clinical presentation of anti-NMDAR encephalitis with gait ataxia, complete ophtalmoplegia, and abolished reflexes followed by drowsiness and confusion. Favorable outcome with combined antiviral treatment and immunomodulatory therapy was observed. Concomitant VZV reactivation with autoimmune encephalitis is possible. Prognosis and therapeutic options in this rare condition remain to be clarified.

Brain MRI features of anti-N-methyl-D-aspartate (anti-NMDA) receptor encephalitis secondary to central nervous system infection in adult patients

Conclusion: Anti-NMDAR encephalitis secondary to CNS infection has its imaging features-extensive lesion distribution, leptomeningeal enhancement, early atrophy, and necrosis-that could deepen the understanding of the pathophysiology and manifestation of the autoimmune encephalitis besides the classic type.

Neuropsychiatric phenotypes of anti-NMDAR encephalitis: a prospective study

Study supports the notion of a neurobehavioral phenotype of ANMDARE characterized by a fluctuating course with psychotic and affective symptoms, catatonic signs, and global cognitive dysfunction, often accompanied by seizures and dyskinesia. The catatonia-delirium comorbidity could be a distinctive neurobehavioral phenotype of ANMDARE.

Clinical outcome in an infant with anti-NMDA receptor encephalitis: Case report and literature review

We update the infants with anti-NMDAR encephalitis in the literature. Clinical outcomes suggest that patients with anti-NMDAR encephalitis are mostly poor in the infants, excluding our case. We propose that early and appropriate treatments are critical for timely diagnosis and rapid improvement.

The Phenomenology of Anti-NMDA Receptor Encephalitis: A Comparison with "Primary Mental Confusion" in Late 19th Century French Psychiatry

Conclusions: From the results, it is suggested that the phenomenological features understood as indicative of anti-NMDA receptor encephalitis include: (1) antecedent common cold-like symptoms (31.9%) in the prodromal phase, (2) delirium or acute confusional state (65.9%), (3) symptoms considered to be sudden personality changes (40.4%) in the psychotic phase, (4) central hypoventilation (14.9%) in the unresponsive phase, (5) motor disturbances (57.4%), and (6) autonomic symptoms, mainly without fluctuations (48.9%), in the hyperkinetic phase. These features were found to be similar to “primary mental confusion” (confusion mentale primitive) in French psychiatry in the late 19th century. We believe that classical psychiatry can contribute considerably to the interpretation of biological research results.

Characteristics and Outcomes of Paroxysmal Sympathetic Hyperactivity in Anti-NMDAR Encephalitis

Cardiac autonomic dysfunction was the most common subtype (77.8%). Patients with a higher incidence of ovarian teratoma, mechanical ventilation, neurological intensive care unit admission, and elevated glucose and NMDAR antibody titer in the CSF were more likely to exhibit autonomic dysfunction or PSH. Episodes of PSH can be suppressed by monotherapy in patients without prior sedative drug use with an efficacy of 90%.

Paroxysmal sympathetic hyperactivity (PSH) is a common clinical condition in patients with anti-NMDAR encephalitis, especially in severe cases, and can be effectively managed by several drug monotherapies. Despite necessitating longer hospital stay, autonomic dysfunction or PSH do not seem to compromise the neurological recovery of patients.

Clinical Relevance of Cerebrospinal Fluid Antibody Titers in Anti-N-Methyl-d-Aspartate Receptor Encephalitis

Our study made several noteworthy contributions to the CSF Ab titers of anti-NMDAR encephalitis. Patients starting with psychiatric symptoms showed a higher titer than those with seizures and memory deficits, especially among youths in the subacute phase. Yet, the level of the titers was unaffected by age, duration, gender or other complications within 15–30 days of onset. Regarding the clinical severity, the titers showed a weak correlation with the mRS, but no correlation with the CASE. Further larger prospective studies are needed to better validate our findings, and related experiments are urgently required to explore their potential mechanisms.

A video-based discussion of movement disorders in paediatric anti NMDAR encephalitis: A case series from Eastern India

Movement disorders are a distinct feature of anti NMDAR encephalitis, particularly in paediatric age group.

There is a wide spectrum of movement disorders often in combination, which necessitates proper delineation of the individual movements as elaborated in the illustrative videos.
Early and prompt recognition of movement semiology is essential to initiate therapy at earliest.
Anti NMDAR antibody mediated movement disorders may respond favourably to immunomodulation.
Understanding and recognizing the spectrum of movement disorders in anti NMDAR encephalitis is particularly important in resource limited series in order to curtail cost of unnecessary investigations.

Clinical Relevance of Cerebrospinal Fluid Antibody Titers in Anti-N-Methyl-d-Aspartate Receptor Encephalitis

This study of 76 patients made several noteworthy contributions to the CSF Ab titers of anti-NMDAR encephalitis. Patients starting with psychiatric symptoms showed a higher titer than those with seizures and memory deficits, especially among youths in the subacute phase. Yet, the level of the titers was unaffected by age, duration, gender or other complications within 15–30 days of onset. Regarding the clinical severity, the titers showed a weak correlation with the mRS, but no correlation with the CASE.

Isolated Memory Loss in Anti-NMDAR Encephalitis

This case shows that anti-NMDAR encephalitis can present with isolated memory loss. Neural antibody testing in these patients could play a pivotal role in early diagnosis and prompt treatment.

Impairment of early neuronal maturation in anti-NMDA-receptor encephalitis

Conclusions: This is the first demonstration of the damage by anti-NMDAR antibodies on early dendritic development in vitro. As a strategy to protect embryonic neurons, our findings may support the efficacy of early immunotherapy for anti-NMDAR encephalitis in pregnancy.

Electroencephalography characteristics to predict one-year outcomes in pediatric anti-NMDA receptor encephalitis

Conclusions: Loss of normal posterior dominant rhythm (PDR), absence of sleep architecture, and epileptiform discharges are associated with worse outcomes at one year which has not been reported before. EEG characteristics may help prognosticate in NMDARE. Larger studies are needed to confirm these findings.

Electroencephalography characteristics to predict one-year outcomes in pediatric anti-NMDA receptor encephalitis

Conclusions: Loss of normal posterior dominant rhythm (PDR), absence of sleep architecture, and epileptiform discharges are associated with worse outcomes at one year which has not been reported before. EEG characteristics may help prognosticate in NMDARE. Larger studies are needed to confirm these findings.

Anti-N-Methyl-D-Aspartate Receptor (NMDAR) Encephalitis in Children and Adolescents: A Systematic Review and Quantitative Analysis of Reported Cases

Results

The sample (n=283) had a mean age of 10.8 years with 75.3% females. The most common prodromal and presenting symptom was seizure (29.7% and 63.3%, respectively). Partial and full recovery did not differ for prodromal and presenting symptoms. Multivariate logistic regression analysis found that (1) delusion were significantly associated with higher odds and aggressive behavior was associated with lower odds for abnormal findings on magnetic resonance imaging (MRI); (2) waxing and waning of symptoms were significantly associated with higher odds for abnormal electroencephalograms (EEG), and (3) increased age and psychosis were each significantly associated with increased odds, and sleep disturbance and orofacial dyskinesia with lower odds for tumor presence.

Conclusion

Given the pattern of findings, routinely obtaining MRI and EEG should be considered for anti-NMDAR encephalitis in children and adolescents presenting with delusion and waxing and waning of symptoms, respectively. Investigation of tumors should be considered in patients with anti-NMDAR encephalitis especially when psychosis is present.

Comprehensive Meta-Analysis Supports Early Treatment for Best Outcome in NMDA Receptor Encephalitis

Researchers have brought together all the reported cases in the literature—1550 patients from 652 reports—since the 2007 discovery of NMDARE, analyzing the range of treatments and the factors associated with outcomes and relapse rates.

“The overriding message from this herculean undertaking,” said study leader Ming Lim, MD, PhD, “is that we need to recognize this condition early, and begin treatment right away. Treating it early and escalating as quickly as possible gives you a better outcome.” 

Autoimmune epilepsy due to N-methyl-d-aspartate receptor antibodies in a child: a case report

Our case report highlights the importance of early diagnosis of AEp, early treatment with immunotherapy, long-term clinical follow-up, and timely escalation and continuation of immunotherapy in achieving a good patient outcome, retaining intellectual development, and quality of life.

CSF Findings in Acute NMDAR and LGI1 Antibody–Associated Autoimmune Encephalitis

NMDAR- and LGI1-E are the 2 most common AE subtypes. Each is representative of 1 of 2 clusters of AE subtypes: NMDAR-E for those with prominent CSF inflammation and LGI1-E for those with little or no CSF inflammation. Beyond the expected quantitative differences, our detailed analysis of CSF findings in therapy-naive acute NMDAR- and LGI1-E shows that each of the 2 AE subtype exhibits a distinct pattern of CSF changes. These are characterized by differences in the interdependencies of CSF parameters and their association with age, disease duration, and disease severity.

CSF Findings in Acute NMDAR and LGI1 Antibody–Associated Autoimmune Encephalitis

NMDAR- and LGI1-E are the 2 most common AE subtypes. Each is representative of 1 of 2 clusters of AE subtypes: NMDAR-E for those with prominent CSF inflammation and LGI1-E for those with little or no CSF inflammation. Beyond the expected quantitative differences, our detailed analysis of CSF findings in therapy-naive acute NMDAR- and LGI1-E shows that each of the 2 AE subtype exhibits a distinct pattern of CSF changes. These are characterized by differences in the interdependencies of CSF parameters and their association with age, disease duration, and disease severity.

The Association of Ovarian Teratoma and Anti-N-Methyl-D-Aspartate Receptor Encephalitis: An Updated Integrative Review

This article reviewed studies from 2007 to 2020 and concluded some information regarding clinicopathological characteristics, such as age distribution, histological property, symptoms at presentation, and relapse rate. Though this entity’s incidence varies in different studies, we have concluded that the overall incidence of ovarian teratoma among anti-NMDAR encephalitis patients is 37.4%. However, little data shows the incidence rate of anti-NMDAR encephalitis in ovarian teratoma patients and, therefore, needs more future study.

We have emphasized the cellular and molecular pathways of the disease foundation and thus proposed a hypothetical mechanism of the pathogenesis in ovarian teratoma-related anti-NMDAR encephalitis. The pathological process involves an eccentric cellular composition of the teratoma, patients’ usual immune response, and the interfered interaction of receptors and proteins, which lead to autoantibody-induced encephalitis.

Understanding Seizures and Prognosis of the Extreme Delta Brush Pattern in Anti-N-Methyl-D-Aspartate (NMDA) Receptor Encephalitis: A Systematic Review

The etiology of the EDB remains essentially unknown. However, it has been postulated that in ANMDAR encephalitis, there is a disruption of the rhythmic neuronal activity. When antibodies block/target NMDAR, the rhythmic neuronal activity is disrupted, leading to the unique EDB pattern. Another theory suggests that delta activity is caused because of focal abnormalities in the brain, and the superimposition of the beta waves is related to the alterations of the NMDA receptors.

There was wide variability in the prevalence of EDB (6%-58%) in our sample which could be related to the timing of the EEG recording. The pooled prevalence was 30.89% (55/178) in patients with seizures. Overall, two studies concluded worse short-term outcomes in patients with EDB but the other two studies displayed a lack of such evidence. Some patients with EDB had prolonged hospital stays, increased ICU admission, and a higher frequency of status epilepticus. These findings suggest that the EDB pattern may be a marker of more severe disease and worse short-term outcomes, while long-term outcomes are not affected. Since treatment with Therapeutic Plasma Exchange (TPE)  in severe anti-NMDAR encephalitis can improve short-term clinical outcomes including epilepsy, we suggest that every patient with EDB pattern may benefit from this treatment.

Limbic encephalitis in a child with ovarian teratoma and influenza B. Case report and critical review of the history of autoimmune anti-N-methyl-d-aspartate receptor encephalitis

 Case report the appearance of clinical symptoms and signs of N-methyl-d-Aspartate (NMDA) receptor encephalitis in a patient presenting just days after contraction of influenza B. The offending mature ovarian teratoma was identified and removed on the 10th day after the appearance of symptoms, with subsequent nearly complete resolution of symptoms over the subsequent 6 months. We provide a focused literature review of the clinical and pathophysiologic literature of anti-NMDA receptor encephalitis pertaining to influenza B virus and the pediatric population. Taken together, this study contributes to the pathophysiological understanding of anti-NMDA receptor encephalitis and aids clinicians in its early recognition and management.

Thyroid Function and Anti-thyroid Antibodies in Pediatric Anti-NMDAR Encephalitis

 Anti-thyroid antibody positivity, abnormality of FT3, FT4, or TSH levels and NTIS are frequent in pediatric anti-NMDAR encephalitis. Thyroid antibody and thyroid hormone abnormalities could be improved through the course of treatment of anti-NMDAR encephalitis. Cases with ATAbs (+) are at older onset ages and more likely to be treated by intravenous immunoglobulin therapy more than once. Unlike adult anti-NMDAR encephalitis, NTIS might not be associated with the clinical characteristics of anti-NMDAR encephalitis in pediatric patients.

Presence of Anti-Thyroid Antibodies Correlate to Worse Outcome of Anti-NMDAR Encephalitis

120 patients were enrolled in the study. anti-thyroid antibodies (ATAbs) are prevalent in patients with anti-NMDAR encephalitis, especially in severe cases, and correlate with poor prognosis and impaired short-term neurological recovery.

Clinical Features of Coexisting Anti-NMDAR and MOG Antibody-Associated Encephalitis: A Systematic Review and Meta-Analysis

Dual-positivity for anti-NMDAR and anti-MOG antibodies is not commonly encountered in clinical settings. In patients with this disease phenotype, the incidence rate of relapse was very high, but the functional outcome might not be poor. Although overlapping symptoms and imaging changes of NMDARE and MOG-AD were observed in these patients, the overall symptoms seemed to be more similar to NMDARE, rather than MOG-AD.

Real-world experience of assessing antibodies against the N-methyl-D-aspartate receptor (NMDAR-IgG) in psychiatric patients. A retrospective single-centre study

To evaluate the frequency of anti-NMDAR encephalitis in a secondary mental health service and investigate the challenges of its diagnosis in routine clinical practice.

In 1661 patients assessed for NMDAR-IgG over 12 years, the positivity rate was 3.79%.

In a psychiatric setting, NMDAR-IgG seropositivity rates were low with a positive predictive value for encephalitis around 50% when screened patients had chronic presentations and absence of other diagnostic criteria for encephalitis or psychosis of autoimmune origin. Chronic neuropsychiatric problems in anti-NMDAR encephalitis are not uncommon, so better diagnostic and treatment strategies are still needed.

NMDA‑receptor encephalitis in Denmark from 2009 to 2019: a national cohort study

The incidence of NMDARE in Denmark is currently 0.17/100,000 persons per year, and has increased since 2009. NMDARE patients in Denmark display a higher median age, lower female:male ratio, a less frequent tumor associa-tion and need for ICU admission. Maintenance therapy did not reduce relapse rate. Poor outcome was seen with higher age, likely related to underlying etiology.

Status Epilepticus in Patients with Anti-NMDAR Encephalitis Requiring Intensive Care: A Follow-Up Study

Patients with critical anti-NMDAR encephalitis who present with status epilepticus (SE) have a high rate of refractory SE (RSE)/super RSE and recover more slowly than patients without SE, but most of them will eventually achieve good long-term neurological outcomes and live seizure free after the acute phase.

Extreme Delta Brush in Anti-NMDAR Encephalitis Correlates With Poor Functional Outcome and Death

Conclusions: The presence of EDB on EEG in anti-NMDAR encephalitis patients is associated with increased need for ICU admission, worse functional outcomes at 6 months, and risk of death.

Immunotherapies for Anti-N-M-methyl-D-aspartate Receptor Encephalitis: Multicenter Retrospective Pediatric Cohort Study in China

In conclusion, for patients with mRS ≥ 4 after first-line immunotherapy and patients with concomitant positive MOG-Ab, we recommend more aggressive treatments like second-line immunotherapy. When second-line immunotherapy is not applicable due to severe adverse effects or other reasons, repetitive first-line immunotherapy can be considered as an option. Both second-line and repetitive first-line immunotherapy are beneficial to reduce relapse rate. The treatment course of sequential oral prednisolone, as a routine maintenance treatment, can be shortened after fully evaluating patients’ conditions.

Anti-N-methyl-D-aspartate receptor encephalitis presenting as atypical psychosis in multiple sclerosis: a case report

There is literature to demonstrate a possible connection between anti-NMDAR encephalitis and demyelinating syndromes. As such, autoimmune encephalitis should be considered in patients with multiple sclerosis presenting with atypical symptoms. Determining the correct diagnosis is crucial to inform the appropriate treatment protocol, and to improve prognosis.

Anti N‐Methyl‐D‐Aspartate (NMDA) receptor encephalitis: from psychosis to cognitive impairment

 1.  N‐Methyl‐D‐Aspartate Receptor play an important role in cognition and behavior. NMDAR blocking agents may cause manifestations such as psychosis. 2 In recent years, the role of anti‐NMDAR antibodies in the development of psychiatric symptoms has been emphasized. 3 Anti‐NMDAR autoimmune encephalitis is a relatively new neuropsychiatric disease that has been described in the last two decades. 4 Characteristics such as psychosis, seizures, abnormal movements, coma, and dysautonomia are manifestations of this disorder. 5 So that up to two thirds of these patients are first referred to psychiatric centers. 6 Cognitive deficits such as working memory deficits have also been suggested as long‐lasting consequences of the disease. 7 In this study, while presenting a clinical case with early psychiatric manifestations, we point out the need to pay attention to rare neurological diagnoses in the presence of patients with common psychiatric manifestations.

The challenge of diagnosing and successfully treating anti-NMDA receptor encephalitis in a toddler

Nice case report on a 26-month-old Sudanese girl. This case study provides a nice overview of anti-NMDAr as well as a Discussion section parents will benefit from as well as doctors.

Full or substantial recovery occurs in nearly 80% of children with anti-NMDAR encephalitis, and this may be hastened by the use of immunotherapy; one or more relapses can occur in +25%-33% of children. A prompt diagnosis and early treatment should be the aim of therapy if full recovery is to be achieved

Anti-NMDA receptor encephalitis: a review of mechanistic studies

In a little more than a decade since the discovery of anti-NMDAR encephalitis, the research towards understanding its pathogenic mechanisms has advanced from the in vitro approach to the in vivo passive transfer model, and to in vivo active immunization models. There is general agreement that the NMDAR autoimmune antibodies are the culprit behind the encephalitis, as the NMDAR antibodies cross-link the NMDARs, disrupt NMDAR-EphB2 interactions, and cause NMDAR internalization. The internalization results in hypofunction of NMDARs, which is believed to be responsible for the impaired long-term potentiation and observed memory and behavioral deficits, and increased susceptibility to seizures. As we strive to better understand the disease, there are some critical unanswered questions.

CT and MRI characteristics of ovarian mature teratoma in patients with anti-N-methyl-D-aspartate receptor encephalitis

Purpose: The purpose of this study was to determine the computed tomography (CT) and magnetic resonance imaging (MRI) characteristics of ovarian mature teratoma in patients with anti-N-methyl-d-aspartate receptor encephalitis (NMDAR-E).

Conclusion: By comparison with teratomas in patients without NMDAR-E, teratomas in patients with NMDAR-E are smaller, have few teeth/calcification, and the amount of space occupied by fat components is smaller.

Critical Analysis of a Challenging Case of Post-Infectious N-Methyl-D-Aspartate Receptor Encephalitis

Infections are increasingly recognized as a common trigger of autoimmune disease, including autoimmune encephalitis. A significant association is particularly shown between HSV-1 encephalitis (HSVE) and a post-infectious autoimmune encephalitis mediated by neuronal autoantibodies, most notably anti-N-methyl-D-aspartate receptor (NMDAR) antibodies.

Here we present a case of a 19-year-old female with premorbid psychiatric disease and neuropsychiatric sequelae from HSVE who presented over a year after her initial HSVE with behavioral changes and positive anti-NMDAR antibodies.

Anti-N-Methyl-D-Aspartate Receptor Encephalitis with Decrease in Blood Flow in Cerebellum

Case study. In conclusion, our patient showed a decrease in blood flow in the cerebellum during long-term observation. We consider SPECT and neuropsychological tests to be important to detect mild cognitive sequelae in patients recovering from anti-NMDAR encephalitis. Her symptoms improved after oral administration of MTX. We suggest that oral MTX may be a promising alternative treatment for cases with anti-NMDAR encephalitis that do not respond to first-line and second-line treatments. Further data should be gathered regarding the effectiveness of oral administration of MTX for anti-NMDAR encephalitis.

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

This study gave detailed descriptions of distinct brain metabolic patterns related to patients with anti-NMDAR encephalitis triggered by different causes. These metabolic patterns with 18F-FDG PET scans, rather than MRI imaging, demonstrated high sensitivity associated with the disease, particularly in the cryptogenic and paraneoplastic subgroups. The awareness of such distinct patterns provided valuable information to
better understand the various occurrences and development of anti-NMDAR encephalitis in each subgroup, and may help to facilitate early diagnosis, treatment strategy and prognosis prediction of this elusive, but treatable, disorder.

Efficacy and safety of bortezomib in rituximab-resistant anti-N-methyl-d-aspartate receptor (anti-NMDAR) encephalitis as well as the clinical characteristics: An observational study

Highlights:

Treatment-resistant anti-NMDAR encephalitis patients improve significantly after Bortezomib.

Bortezomib depletes serum antibody-secreting cells in treatment-resistant anti-NMDAR encephalitis patients.
Bortezomib results in pathogenic antibody decrease by depleting antibody-secreting cells.

Anti-N-Methyl-D-Aspartate Receptor Encephalitis with Decrease in Blood Flow in Cerebellum

Anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis is the most common antineuronal antibody encephalitis among the autoimmune types of encephalitis found at present.
Progressive cerebellar atrophy potentially develops in patients with severe disabilities due to
anti-NMDAR encephalitis. Here, we report a patient with anti-NMDAR antibody encephalitis who showed a persistent decrease of blood flow in the cerebellum with slight cerebellar atrophy.

High serum creatinine is associated with reduction of psychiatric symptoms in female patients with anti-NMDAR encephalitis

Objective: Serum creatinine (SCR) has been shown to be associated with many neurodegenerative diseases. In this study, we investigated the relationship between SCR levels and the incidence of psychiatric symptoms in patients with anti-N-methyl-d-aspartate receptor (anti-NMDAR) encephalitis.

Conclusion: Our study indicated that the SCR level was negatively correlated with incidence of psychiatric symptoms in female patients, and a higher SCR level could be a protective factor for psychiatric symptoms in female patients with anti-NMDAR encephalitis.

Clinical features and management of coexisting anti-N-methyl-D-aspartate receptor encephalitis and myelin oligodendrocyte glycoprotein antibody-associated encephalomyelitis: a case report and review of the literature

Conclusions: The rates of coexisting anti-NMDA receptor encephalitis and MOG antibody-associated encephalomyelitis may be underestimated. Clinical symptoms such as seizures and cognitive decline accompanied by atypical central nervous system demyelination serve as warning signs of possible coexisting anti-NMDA receptor encephalitis and MOG antibody-associated encephalomyelitis. These patients could achieve good outcomes under proper immunotherapies.

Anesthesia for Pediatric Patients with Anti‐NMDA‐Receptor Encephalitis: a retrospective case series.

Conclusion: Although pediatric patients with anti-N-methyl-D-aspartate-receptor encephalitis experienced vital sign changes with anesthesia, they were not clinically significant and they behaved similarly to controls. Disease severity may be a risk factor for perioperative complications.

Case Report: Overlapping Multiple Sclerosis With Anti-N-Methyl-D-Aspartate Receptor Encephalitis: A Case Report and Review of Literature

NMDAR encephalitis. Anti-NMDAR encephalitis should be considered when MS patients present atypical symptoms (e.g., psychiatric symptoms, epilepsy, and movement disorder), and testing for anti-NMDAR antibodies is needed in such situations. Our observations suggest that there might be a possible link between anti-NMDAR encephalitis and MS. Recognition of these associations is important to avoid misdiagnoses and refine the treatment.

Lipid profiles and their potential inflammatory effects in anti-N-methyl-D-aspartate receptor encephalitis

Conclusions: Lipid profiles are associated with the pathogenesis and progression of anti-NMDAR encephalitis, and they are significantly correlated with different inflammatory markers, suggesting that the association of lipids with the disease might be influenced by the inflammatory response.

Autism Associated With Anti-NMDAR Encephalitis: Glutamate-Related Therapy

Earlier diagnosis of autoimmune encephalitis increases the potential of curative treatments by enabling the provision of timely immune therapy or immune-modulatory therapy, which can prevent long-term consequences, such as being misdiagnosed with autism. An immunophenotyped patient with symptoms of autism may need to obtain a diagnosis of autoimmune encephalitis to avoid being misdiagnosed with ASD. Additionally, in children with the underlying syndrome presentation of childhood disintegrative disorder, early-onset schizophrenia, or late-onset autism; in children with first episode of psychosis; and especially when autistic symptoms follow a febrile illness, autoimmune autism including diagnosis of anti-NMDAR encephalitis or other autoimmune dysfunctional encephalitis should be considered as a possible organic cause.

The Clinical Features and Prognosis of Anti-NMDAR Encephalitis Depends on Blood Brain Barrier Integrity

Highlights

Decreased consciousness, ICU admission, a higher Neutrophil-to-lymphocyte ratio (NLR), and CSF protein are related to the integrity of the BBB in anti-NMDAR encephalitis patients.

In anti-NMDAR encephalitis patients, albumin/serum albumin (QAlb) increased was positively correlated with the quantitative intrathecal IgG synthesis.
The dysfunction of BBB can be helpful in evaluating anti-NMDAR encephalitis patients’ prognosis.

Case report: anti-N-Methyl-D-Aspartate receptor encephalitis and bilateral temporal calcifications

Our findings confirm the rarity co-existing anti-NMDAR encephalitis and cerebral calcifications. In future clinical work, we need to elucidate the relationship between anti-NMDAR encephalitis and cerebral
calcifications, and the association between anti-NMDAR encephalitis and other co-existing autoimmune disorders.

Regionally Metastatic Merkel Cell Carcinoma Associated with Paraneoplastic Anti-N-methyl-D-aspartate Receptor Encephalitis

We present the case of a 59-year-old male with regionally metastatic Merkel cell carcinoma complicated by the paraneoplastic manifestation of anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis.

Clinical, cognitive and neuroanatomical associations of serum NMDAR autoantibodies in people at clinical high risk for psychosis

Serum neuronal autoantibodies, such as those to the NMDA receptor (NMDAR), are detectable in a subgroup of patients with psychotic disorders. It is not known if they are present before the onset of psychosis or whether they are associated with particular clinical features or outcomes. In a case-control study, sera from 254 subjects at clinical high risk (CHR) for psychosis and 116 healthy volunteers were tested for antibodies against multiple neuronal antigens.  Preliminary phenotypic analyses revealed that within the CHR sample, the NMDAR antibody seropositive subjects had higher levels of current depression, performed worse on the Rey Auditory Verbal Learning Task (p < 0.05), and had a markedly lower IQ (p < 0.01). NMDAR IgGs were not more frequent in subjects who later became psychotic than those who did not.

This study, NMDAR antibodies did not predict transition to psychosis, as defined by operationalized criteria, or nonremission from the CHR state. NMDAR antibody seropositivity was however associated with a deterioration in disability-associated functioning, short of transition, and indeed greater antibody titre was associated with greater deterioration in function, overall suggesting that NMDAR antibody serostatus should be further evaluated as a predictive marker of functional outcome.

Cerebrospinal fluid biomarkers of brain injury, inflammation and synaptic autoimmunity predict long-term neurocognitive outcome in herpes simplex encephalitis

Conclusions

Our findings show that neurofilament protein (NFL) levels are predictive of long-term neurocognitive outcome in HSE, and suggest a causative chain of events where brain tissue damage increases the risk of NMDAR autoimmunisation and subsequent prolongation of CSF inflammation. The data provides guidance for a future intervention study of immunosuppressive therapy administered in the recovery phase of HSE.

Cerebrospinal fluid CD20 positive B-cell expansion in a case of anti-NMDAR encephalitis

Highlights

CSF CD20+ cells increased in an anti-NMDAR encephalitis.
Low dose of rituximab was used in this patient.
These findings support the use of rituximab.

Keeping pace with the world: improving the clinical practice of anti-NMDAR encephalitis in China

Antibodies panel related to AE is among the first-line tests in many neurological centers in China which contributes to a more prompt and efficient immunotherapy. Increasing cases have enriched neurologists’ experience in treating AE. Encephalitis has been considered as a novel subspecialty and encephalitis clinics or encephalitis centers have been established by the neurologists across China.

Clinical and EEG characteristics analysis of autoimmune encephalitis in children with positive and negative anti-N-methyl- D-aspartate receptor antibodies

co-hort of 28 child patients.  Conclusions: The clinical manifestation of autoimmune encephalitis is complex and varied, but it is a curable disease. Immunotherapy should be considered as soon as possible, with or without autoantibodies. Most of the child patients had a good prognosis, while some of them had the sequelae of epilepsy, mild mental symptoms, and dyskinesia. It is necessary to improve the understanding of autoimmune encephalitis with/without positive antibodies, and make diagnosis and treatment as soon as possible, in order to improve the prognosis.

Teratoma Removal, Steroid, IVIG, Rituximab and Tocilizumab (T-SIRT) in Anti-NMDAR Encephalitis

In anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis, we analysed the efficacy of a combined immunotherapy protocol consisting of teratoma removal, steroid, intravenous immunoglobulin (IVIG), rituximab and tocilizumab (T-SIRT). This cohort study included seventy-eight consecutive patients treated for anti-NMDAR encephalitis between Jan 2014 and Oct 2019 in a national referral hospital.

We concluded that the early application of combined immunotherapy consisting of tocilizumab (T-SIRT) had better efficacy than was found for delayed or partial application of this combination in anti-NMDAR encephalitis.

Allosteric modulation of NMDA receptors prevents the antibody effects of patients with anti-NMDAR encephalitis

About 85% of patients respond to immunotherapy (and removal of an associated tumor if it applies), but it often takes several months or more than 1 year for patients to recover.

There are no complementary treatments, beyond immunotherapy, to accelerate this recovery. Previous studies showed that SGE-301, a synthetic analogue of 24(S)-hydroxycholesterol, which is a potent and selective positive allosteric modulator of NMDAR, reverted the memory deficit caused by phencyclidine (a non-competitive antagonist of NMDAR), and prevented the NMDAR dysfunction caused by patients’ NMDAR antibodies in cultured neurons. An advantage of SGE-301 is that it is optimized for systemic delivery such that plasma and brain exposures are sufficient to modulate NMDAR activity.

All antibody-mediated pathogenic effects (memory, synaptic NMDAR, long-term potentiation) were prevented in the animals treated with SGE-301. Additional investigations showed  SGE-301 prolonged the decay time of NMDAR-dependent spontaneous excitatory postsynaptic currents suggesting a prolonged open time of the channel; and (ii) it significantly decreased, without fully preventing, the internalization of antibody-bound receptors suggesting that additional, yet unclear mechanisms, contribute in keeping unchanged the surface NMDAR density. Overall, these findings suggest that SGE-301, or similar NMDAR modulators, could potentially serve as complementary treatment for anti-NMDAR encephalitis and deserve future investigations.

Co-expression of NMDA-receptor subunits NR1, NR2A, and NR2B in dysplastic neurons of teratomas in patients with paraneoplastic NMDA-receptor-encephalitis: a retrospective clinico-pathology study of 159 patients

Conclusions: Patients with teratomas and paraneoplastic anti-NMDARE revealed a cellular population of dysplastic neurons co-expressing NMDAR subunits, which were the potential source of autoantigens triggering anti-NMDARE. Some inflammatory cytokines may be involved in pathogenesis of paraneoplastic anti-NMDARE.

Detailed morphological analysis of rat hippocampi treated with CSF autoantibodies from patients with anti-NMDAR encephalitis discloses two distinct types of immunostaining patterns

In the highly heterogenous disease anti-NMDA-receptor 1 encephalitis we found evidence for at least two different subtypes. It will be very interesting to determine whether there also are two distinct clinical phenotypes.

Cognitive function and cerebral blood perfusion changes in patients with anti-N-methyl-D-aspartate receptor encephalitis

Conclusions: Patients with anti-NMDAR encephalitis have prevalent cognitive deficits such as memory, language, attention and executive dysfunction as well as changes in blood perfusion, which may be related to cognitive deficits. Changes in cerebral blood perfusion can be used as indicators for early diagnosis, disease monitoring and prognosis evaluation of anti-NMDAR encephalitis. Improving the cerebral blood flow of patients may promote the recovery of cognition.

Assessment of long-term psychosocial outcomes in anti-NMDA receptor encephalitis

Highlights

anti-NMDARE may have long-term negative psychosocial impact on patients.

Over 30% of participants did not resume work/school after illness.

Misdiagnosis was associated with decreased odds of a return to work/school

Psychiatry follow-up was associated with increased odds of return to work/school

Ongoing seizures and neuropsychiatric issues were associated with worse outcomes.

A Case of Autoimmune Polyendocrine Syndrome Type 3 (APS-3) Complicated With anti-N-methyl-D-aspartic Acid-Receptor (NMDA-R) Encephalitis

We report a rare case of autoimmune polyendocrine syndrome type 3 (APS-3) complicated by anti-NMDA-R encephalitis. After hospitalization for type 1 diabetes the 39-year-old patient developed various schizophreniform symptoms and seizures following cold-like symptoms. These findings are consistent with the diagnosis of anti-NMDA-R encephalitis. Early detection and treatment of anti-NMDA-R encephalitis should enhance positive outcome, considering that besides thyroid diseases and type 1 diabetes, various autoimmune diseases are associated with APS-3.

Cerebellar ataxia as the initial symptom with lesions involving the cerebellum in patient with anti-NMDAR encephalitis: A rare case report and literature review

Highlights
. In this report, patient with anti-NMDAR IgG manifested gait ataxia as the initial symptoms, simultaneously with lesions involving the cerebellum. (Brain magnetic resonance imagery involving cerebellum has not been reported)
We exclude co-existence of multiple auto-antibodies include ataxia (ACA)-related antibodies, classic onconeural auto-antibodies, aquaporin-4 (AQP4) IgG, myelin ligodendrocyte glycoprotein (MOG) IgG,
We used cell based assay and tissue based assay to confirm the the diagnosis of definite anti-NMDAR IgG.

Sleep disorders in anti-NMDAR encephalitis

Objective: To describe the sleep disorders in anti-NMDA receptor encephalitis (anti-NMDARe).

Methods: Patients recovering from anti-NMDARe were invited to participate in a prospective observational single center study including comprehensive clinical, video-polysomnography (V-PSG) sleep assessment and neuropsychological evaluation. Age and sex-matched healthy participants served as controls.

Conclusions: Sleep disturbances are frequent in anti-NMDARe. They show a temporal pattern (predominantly insomnia at onset; hypersomnia during recovery), associate with behavioral and cognitive changes, and can occur with confusional arousals during NREM sleep.

A case of anti-N-methyl-d-aspartate receptor encephalitis

A 22-year-old female student, with no known previous illness or hospitalization.

Anti-N-methyl-d-aspartate receptor (anti-NMDAR) encephalitis was first described by Dalmau et al in 2007, describing an illness with predominantly neuropsychiatric symptoms in young women.  An association with paraneoplastic syndromes is recognized, the most common neoplasm being ovarian in origin – usually a teratoma.  However, neoplasms of the mediastinum (extragonadal teratoma), lymphoma, testicular cancer, and pontine glioma have also been described. Recognized early, it is highly responsive to treatment with a favourable outcome; 75% of patients achieving full recovery or having minor neurological deficits at 17 months. However, in most instances, many patients retain cognitive deficits for a long time.

Associations between seizures and MRI in patients with anti‐NMDAR encephalitis

Conclusions- Focal seizures were most common in patients with abnormal MRI lesions. In the acute stage of the disease, the abnormal MRI group was more likely than the normal MRI group to achieve seizure control. Abnormal MRI findings did not affect the overall good prognosis of patients with anti‐NMDAR encephalitis with seizures.

An Elderly Case of Paraneoplastic anti-NMDA Receptor Encephalitis Associated with Small-cell Lung Cancer Expressing NR1 Subunits

A 61-year-old Japanese man presented with anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis.
NR1 antibodies were detected in his cerebrospinal fluid. Chest computed tomography revealed lung tumor.
The patient was diagnosed with paraneoplastic anti-NMDAR encephalitis associated with lung cancer and
treated with two cycles of intravenous high-dose methylprednisolone and one cycle of intravenous immunoglobulin. However, he died one year later without improvement. An autopsy confirmed small-cell lung cancer
(SCLC). Immunohistochemistry revealed the expression of NR1 subunits in the tumor cells, suggesting that
SCLC may trigger NR1 autoimmunity though the expression of NR1 subunits as onconeural antigens, expanding the phenotypic spectrum of paraneoplastic neurological syndrome associated with SCLC

Evaluation of the proposed anti-N-Methyl-D-Aspartate receptor encephalitis clinical diagnostic criteria in psychiatric patients

This study retrospectively assessed the proposed criteria in patients presenting to psychiatric services for the first time with known anti-NMDAR antibody status. Antibody positive cases were derived from the literature (conception to December 2019) and a state wide (Queensland, Australia) cohort. Antibody negative cases were derived from a service wide (Metro South, Queensland, Australia) cohort of psychiatric cases which underwent antibody testing for routine organic screening. Sensitivity and specificity were calculated at 1 week following admission and the point of discharge.

Clinical and Magnetic Resonance Imaging Outcome Predictors in Pediatric Anti–N‐Methyl‐D‐Aspartate Receptor Encephalitis

To evaluate disease symptoms, and clinical and magnetic resonance imaging (MRI) findings and to perform longitudinal volumetric MRI analyses in a European multicenter cohort of pediatric anti–N‐methyl‐D‐aspartate receptor encephalitis (NMDARE) patients.

NMDAR Encephalitis Associated With Acute Chikungunya Virus Infection: A New Trigger?

Anti-NMDAR encephalitis is the most frequent cause of autoimmune encephalitis. Chikungunya (CHIK) is an arbovirus responsible for outbreaks of fever, cutaneous rash and arthritis in underdeveloped countries, and a trigger for autoimmunity.

Clinical and MRI Outcome Predictors in Pediatric Anti-NMDA Receptor Encephalitis

INTERPRETATION: Children with NMDARE exhibit significant brain volume loss and failure of age-expected brain growth. Abnormal MRI findings, a clinical presentation with sensorimotor deficits, and a treatment delay >4 weeks are associated with worse clinical outcome. These characteristics represent promising prognostic biomarkers in pediatric NMDARE.

A case of NMDAR encephalitis treated in the third trimester - novel arterial spin labeling findings and a review of literature

Highlights: Anti-NMDAR encephalitis during pregnancy could show a good prognosis. Arterial spin labeling can help to diagnose anti-NMDAR encephalitis during pregnancy. Early suspicion and proper immunotherapies is crucial for successful treatment.

Specific Psychiatric Features Identify Anti-NMDAR Encephalitis Before Neurological Deterioration

Objective: Evaluate the psychiatric presentation of anti-NMDAR encephalitis to identify features that help differentiate from a primary psychiatric disorder.

 

Conclusions: Several features including prodromal cognitive deficits, speech disturbance, antipsychotic sensitivity and catatonia are seen early in the course of anti-NMDAR encephalitis. These key features would not be typical of primary psychiatric disease and clinicians should have a low index of suspicion to proceed to lumbar puncture.

Frequency and clinical significance of seronegative anti-NMDAR encephalitis

Objective: To determine the frequency of anti-NMDAR encephalitis without detectable serum NMDAR antibodies (NMDAR-abs) and the potential changes in the syndrome presentation.

Conclusions: 13% of patients with anti-NMDAR encephalitis are seronegative. These patients are more likely to be male, with milder forms of the disease, and without tumors.

Different FDG-PET metabolic patterns of anti-AMPAR and anti-NMDAR encephalitis: Case report and literature review

The distinct cerebral hypometabolic patterns of autoimmune encephalitis were representative for receptor hypofunction and topographical distribution of antigenic receptors. The reversibility of hypometabolism marked the clinical recovery of autoimmune encephalitis and made FDG-PET of brain a valuable diagnostic tool.

NMDA receptor encephalitis and other antibody-mediated disorders of thesynapse

Investigations during the last 10 years have revealed a group of disorders mediated by antibodiesagainst ion channels and synaptic receptors, which cause both neurologic and psychiatric symp-toms. In this review, I discuss the process of discovery and immunologic triggers of these disor-ders, and use anti-NMDA receptor encephalitis to emphasize the importance of understandingthe underlying physiopathologic mechanisms in those diseases

Mycophenolate mofetil, azathioprine and methotrexate usage in paediatric anti-NMDAR encephalitis: A systematic literature review

NMDAR-Antibody Encephalitis: A new clinical way to identify patients

A transdiagnostic pattern of psychiatric symptoms in autoimmune encephalitis

RECOGNIZING PSYCHIATRIC PRESENTATIONS OF ANTI-NMDA RECEPTOR ENCEPHALITIS

Decreased occipital lobe metabolism by FDG-PET/CT

A score that predicts 1-year functional status in patients with anti-NMDA receptor encephalitis

NMDAR Encephalitis: A Possible Pathophysiological Model For Pediatric Neuropsychiatric Disorders

Frequency, symptoms, risk factors, and outcomes of Autoimmune Encephalitis

Monoclonal antibodies from a patient with anti‐NMDA receptor encephalitis

Anti-NMDA receptor encephalitis and nonencephalitic HSV-1 infection

NMDA Receptor Autoantibodies in Autoimmune Encephalitis Cause a Subunit-Specific Nanoscale Redistribution of NMDA Receptors

Initial clinical presentation of young children with N-methyl-d-aspartate receptor encephalitis

Ovarian teratoma associated Anti-N-methyl-D-aspartate receptor encephalitis

Magnetic resonance imaging and positron emission tomography in anti-NMDA receptor encephalitis: A systematic review

Cerebrospinal fluid pentraxin 3 and CD40 ligand in anti-N-menthyl-d-aspartate receptor encephalitis

Anti-NMDA receptor encephalitis presenting as postpartum psychosis—a clinical description and review

N-methyl-D-aspartate receptor antibody production from germinal center reactions: therapeutic implications

N‐methyl‐D‐aspartate receptor antibody production from germinal center reactions: Therapeutic implications

Elevated neuron-specific enolase and S100 calcium-binding protein B concentrations in cerebrospinal fluid of patients with anti-N-methyl-d-aspartate receptor encephalitis

High sensitivity and specificity in proposed clinical diagnostic criteria for anti-NMDAr

Second Opinion: Mystery Diagnosis IV

Decreased occipital lobe metabolism by FDG-PET/CT anti–NMDAr encephalitis biomarker

Cell and Single Molecule-Based Methods to Detect Anti-NMDAr

What is the promise of Bortezomib in refractory anti-NMDA receptor encephalitis?

Functional connectivity of large-scale brain networks in patients with anti-NMDAr encephalitis

NMDAR encephalitis: passive transfer from man to mouse by a recombinant antibody

Persistence of parenchymal and perivascular T-cells in treatment-refractory anti-N-methyl-D-aspartate receptor encephalitis

What is the evidence to support the use of plasma exchange as first‐line therapy for NMDAr antibody encephalitis?

Progressive hippocampal sclerosis after viral encephalitis: Potential role of NMDA receptor

Pathogen-mediated NMDAr autoimmunity and cellular barrier dysfunction in schizophrenia

anti-NMDAr encephaltis coexisting with NMO disorder or neurosyphilis

CSF cyto-/chemokine profile during acute HSV induced anti-NMDAr and in chronic sequelae

NMDAr: Untangling Apparant Inconsistencies for Clinical Practice

First reported cases of anti-NMDAr encephalitis in Vietnamese adolescents and adults

http://www.practiceupdate.com/C/48844/56?elsca1=emc_enews_topic-alert

Differences in treatment of anti-NMDA receptor encephalitis: results of a worldwide survey

What is Anti-NMDA Receptor Encephalitis?

NMDAr autoimmunity affects cognitive performance in herpes simplex encephalitis

Recognizing anti-NMDA receptor encephalitis psychosis on the psych ward

Variations of movement disorders in anti-NMDAr encephalitis: A nationwide study in Taiwan

NMDAr subunit NR1 abs have pathogenic potential irrespective of epitope and immunoglobulin class

All abs against the NMDAr subunit NR1 have pathogenic potential irrespective of epitope & Ig class

The genetic sibling of NMDA receptor encephalitis

First-episode psychosis in a 15 year-old female with clinical presentation of anti-NMDAr

NMDAr encephalitis following Herpes Simplex Encephalitis Appear Different in Adults than in Children

Anti-NMDA Receptor Encephalitis in the Polar Bear (Ursus maritimus)

CSF findings in patients with anti-N-methyl-d-aspartate receptor-encephalitis

Mayo Unravels a Mystery Disease For Minnisota Attorney

anti-NMDAr encephalitis: review of clinical presentation, diagnosis and treatment

Herpes simplex virus encephalitis is a trigger of brain autoimmunity

Diagnostic value of CSF findings in antibody-associated limbic and anti-NMDAR-encephalitis

Newly diagnosed brain disease may be misdiagnosed as psychological disorder

Parents warn of mystery illness misdiagnosed in kids (NMDA)

Clinical experience and laboratory investigations in patients with anti-NMDAR encephalitis

All naturally occurring autoantibodies against the NMDA receptor (discusses BBB permeability)

Anti-N-methyl-D-aspartate receptor encephalitis mimics neuroleptic malignant syndrome

Clinical Commissioning Policy: Rituximab for second line treatment for anti-NMDAR autoimmune encephalitis (all ages)

2017 NHS England will commission rituximab for second line treatment for anti-NMDAR autoimmune encephalitis in accordance with the criteria outlined in this document.

Our website is not a substitute for independent professional medical advice. Nothing contained on our website is intended to be used as medical advice. No content is intended to be used to diagnose, treat, cure or prevent any disease, nor should it be used for therapeutic purposes or as a substitute for your own health professional's advice. Although THE INTERNATIONAL AUTOIMMUNE ENCEPHALITIS SOCIETY  provides a great deal of information about AUTOIMMUNE ENCEPHALITIS, all content is provided for informational purposes only. The International Autoimmune Encephalitis Society  cannot provide medical advice.


International Autoimmune Encephalitis Society is a charitable non-profit 501(c)(3) organization founded in 2016 by Tabitha Andrews Orth, Gene Desotell and Anji Hogan-Fesler. Tax ID# 81-3752344. Donations raised directly supports research, patients, families and caregivers impacted by autoimmune encephalitis and to educating healthcare communities around the world. Financial statement will be made available upon request.

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Autoimmune Encephalitis Trivia Playing Cards

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