anti-NMDAR Encephalitis Research Articles

Association of ovarian teratoma with anti-N-methyl-D-aspartate receptor encephalitis: a case report and narrative review.

Anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis is a potentially life-threatening autoimmune disorder which is strongly associated with ovarian teratomas in young female patients. The primary aim is to highlight the importance of considering NMDAR encephalitis in the differential diagnosis of young female patients presenting with acute or subacute neuropsychiatric symptoms, especially when accompanied by ovarian teratomas. This case report and literature review detail the presentation, diagnosis, and treatment of a 35-year-old G4P3 Indigenous woman who initially presented with neuropsychiatric symptoms and fever, having a history of extensive drug and alcohol use. Misdiagnosed initially, the patient's lack of response to standard treatments led to further investigations, revealing paraneoplastic anti-NMDAR encephalitis secondary to a left ovarian teratoma. The report examines the treatment regimen followed, including prednisolone, intravenous immunoglobulin, rituximab injections, and laparoscopic bilateral salpingo-oophorectomy. This case underscores the critical need for increased clinical vigilance for anti-NMDAR encephalitis in patients, particularly young females, presenting with neuropsychiatric symptoms and potential ovarian teratomas. The literature review accompanying the case report provides valuable insights into the presentation, diagnosis, and management of this complex condition. Lastly, this study emphasised the diagnostic challenges inherent in paraneoplastic neuropsychiatric syndromes, advocating for a multidisciplinary approach in similar clinical scenarios.

Catatonia in anti-NMDA receptor encephalitis: a case series and approach to improve outcomes with electroconvulsive therapy

BackgroundAnti-N-methyl-D-aspartate (NMDA) receptor encephalitis has been recognised to present with the syndrome of catatonia. In severe cases dysautonomia is representative of malignant catatonia. The treatment with benzodiazepines (BZDs) and electroconvulsive therapy (ECT) may decrease morbidity and mortality in patients presenting with anti-NMDA receptor encephalitis and catatonia.MethodsThis is a retrospective case series of eight patients with anti-NMDA receptor encephalitis treated with ECT. We use clinical prediction scores (Clinical Assessment Scale for Autoimmune Encephalitis [CASE] and anti-NMDAR Encephalitis One-Year Functional Status scores) to compare expected outcomes and observed outcomes.ResultsCASE scores in our group ranged between 5 and 19, with a mean score of 13.8 (median 15.5). NEOS scores ranged from 2 to 4, with a mean and median of 3. Of the eight patients, six had a favourable modified Rankin Score (0–2) at a follow-up of 8 to 12 months. Patients received an average of 29.9 ECT treatments in total.ConclusionsBased on clinical prediction scores, this cohort had better than expected functional outcomes. We discuss the use of BZDs and ECT in these cases and propose a treatment algorithm for patients who present with catatonic syndrome in anti-NMDA receptor encephalitis.

Clinical characteristics and prognosis analysis of acute symptomatic seizures secondary to autoimmune encephalitis

ObjectiveThis study aimed to analyze the clinical characteristics and prognosis of patients with autoimmune encephalitis (PWAE) who experienced seizures during the acute phase.MethodsClinical data were collected from 84 patients diagnosed with AE at the General Hospital of Ningxia Medical University between January 2015 and January 2023. Patients were divided into seizure and non-seizure groups. Clinical characteristics of both groups were compared, including differences between anti-NMDAR and anti-LGI1 encephalitis within the seizure group. Due to the limited sample size and to avoid overfitting, we focused on univariate logistic regression analysis to identify individual prognostic factors.ResultsA total of 84 patients were enrolled, with 76.19% (64/84) in the seizure group and 23.81% (20/84) in the non-seizure group. The seizure group had a longer hospital stay (p = 0.013), higher rates of impaired consciousness (p = 0.001), and more frequent intensive care unit (ICU) admission (p = 0.011). They also had higher peripheral blood neutrophil-to-lymphocyte ratio (NLR), leukocyte count, and uric acid levels (p = 0.038, p = 0.006, p = 0.020), and were more likely to show slow-wave rhythms on electroencephalography (EEG) (p = 0.031). At 2-year follow-up, there was no significant difference in prognosis between the seizure and non-seizure groups (p = 0.653), with 35.94% (23/64) of the seizure group having a poor prognosis. Status epilepticus (SE), complications, endotracheal intubation, mRS score at discharge, APE2, and RITE2 scores increased the risk of poor prognosis (OR > 1), while intensive care and albumin reduced the risk (OR < 1).ConclusionSeizures are common in the early stages of AE, with faciobrachial dystonic seizures (FBDS) characteristic of anti-LGI1 encephalitis and SE and super-refractory status epilepticus (Sup-RSE) frequently observed in anti-NMDAR encephalitis. Seizure semiology across AE subtypes lacks specificity, and no symptoms clearly distinguish immune-mediated from non-immune causes. While seizures are linked to AE severity, particularly in anti-NMDAR encephalitis, they do not appear to impact overall prognosis. SE, complications, endotracheal intubation, modified Rankin Scale (mRS) score at discharge, Antibody-Prevalence in Epilepsy and Encephalopathy (APE2) score, Response to Immunotherapy in Epilepsy and Encephalopathy (RITE2) score, intensive care, and albumin were identified as significant prognostic factors.

Increased serum phenylalanine/tyrosine ratio associated with the psychiatric symptom of anti-NMDAR encephalitis.

Encephalitis associated with antibodies against the N-methyl-D-aspartate receptor (NMDAR) results in a distinctive neuro-psychiatric syndrome. It has been reported that the serum phenylalanine-tyrosine (Phe/Tyr) ratio increases during infection. However, the connection between phenylalanine-tyrosine metabolism and psychiatric symptoms remains unclear. We enrolled 24 individuals with anti-NMDAR encephalitis and 18 individuals with non-inflammatory neurological diseases (OND). Chromatography was used to measure serum levels of phenylalanine and tyrosine. Serum and cerebrospinal fluid (CSF) TNF-α levels were obtained from the clinical database. The modified Rankin Scale (mRS) score and Glasgow Coma Scale (GCS) score were recorded during the acute phase. The area under the curve (AUC) of the receiver operating characteristic curve was used to assess prediction efficacy. In NMDAR patients, levels of serum Phe and the ratio of serum Phe/Tyr were higher compared to OND patients. The serum Phe/Tyr ratio was also elevated in NMDAR patients with psychiatric syndrome. Furthermore, serum Phe and Tyr levels were correlated with inflammatory indexes. The serum Phe/Tyr ratio is elevated in NMDAR patients with psychiatric syndrome and is associated with severity. Therefore, the serum Phe/Tyr ratio may serve as a potential prognostic biomarker.

Impact of Clinical Progression on Brain Volume Changes in Patients with Anti-NMDAR Autoimmune Encephalitis

Impact of Clinical Progression on Brain Volume Changes in Patients with Anti-NMDAR Autoimmune Encephalitis

Serum Neurofilament Light Chain as a Biomarker in Pediatric Seronegative and Anti-NMDA Receptor Autoimmune Encephalitis Compared to First Episode of Psychosis Caused by Primary Psychiatric Disease

Serum Neurofilament Light Chain as a Biomarker in Pediatric Seronegative and Anti-NMDA Receptor Autoimmune Encephalitis Compared to First Episode of Psychosis Caused by Primary Psychiatric Disease

Multimodal Intervention in Pediatric Anti-NMDA Receptor Encephalitis

Multimodal Intervention in Pediatric Anti-NMDA Receptor Encephalitis

A Case Report of Anti-NMDA Receptor Encephalitis with Relapse and Recurrence of Teratoma

A Case Report of Anti-NMDA Receptor Encephalitis with Relapse and Recurrence of Teratoma

A Case of Anti-NMDAR Encephalitis Misinterpreted as Bacterial Meningitis

A Case of Anti-NMDAR Encephalitis Misinterpreted as Bacterial Meningitis

Peripherally Administered Therapeutic One-armed Antibody Reversed Behavioral Abnormalities in a Marmoset Model of Anti-NMDAR Encephalitis

Peripherally Administered Therapeutic One-armed Antibody Reversed Behavioral Abnormalities in a Marmoset Model of Anti-NMDAR Encephalitis

Proteome Remodeling in a Peptide Immunization-based Animal Model of Anti-NMDAR Encephalitis

Proteome Remodeling in a Peptide Immunization-based Animal Model of Anti-NMDAR Encephalitis

Anti-NMDA receptor encephalitis fatal case was mistaken as a debut of schizophrenia: the devil is in the psychopathological details

The article presents the clinical case of the patient Victor, 42 years old, who was admitted to a psychiatric hospital in an acute psychotic state with schizophreniform symptoms. His illness progressed rapidly, and at different stages, epileptic paroxysms and disorders of consciousness joined the symptoms typical of schizophrenia. It was then that the question arose of the need to revise the diagnosis of schizophrenia, to carry out a targeted neurological examination and to correct of the treatment regimen. Victor’s case, which ended in death, raises questions for psychiatrists about finding significant differential diagnostic criteria for distinguishing schizophrenia from schizophrenia-like psychoses. To avoid mistakes, it is proposed to rely on the so-called. “diagnostic clues” and “red flags” in identifying the psychopathology of anti-NMDA receptor encephalitis.

Anti-NMDAR encephalitis in a paediatric patient: A case report

A rare autoimmune disease called anti-NMDAR encephalitis usually affects young people and frequently manifests as cognitive deterioration, seizures, and psychiatric symptoms. The diagnostic challenge and the importance in recognizing the clinical features and employing appropriate laboratory and imaging techniques for accurate and timely diagnosis will be discussed. We describe the example of an 8-year-old girl who suddenly began exhibiting strange behavioral and speech problems. Anti-NMDAR encephalitis was diagnosed after further investigation. This casee illustrates how different clinical presentations can lead to a delay in the disorder’s diagnosis. Healthcare practitioners continue to have difficulties in diagnosing heterogeneous clinical manifestations of anti- NMDAR encephalitis, particularly in the juvenile age range. Bangladesh Journal of Medical Science Vol. 23 No. 04 October’24 Page : 1233-1237

Specific clinical and radiological characteristics of anti-NMDA receptor autoimmune encephalitis following herpes encephalitis.

Herpes simplex virus encephalitis (HSE) frequently triggers secondary anti-N-methyl-D-aspartate receptor encephalitis (NMDARE), but markers predicting the occurrence of this entity (HSE-NMDARE) are lacking. We conducted a retrospective description of patients with HSE-NMDARE diagnosed between July 2014 and August 2022 and compared them to both patients with regular forms of HSE and NMDARE. Among the 375 patients with NMDARE, 13 HSE-NMDARE were included. The median age was 19years (0.5-73), 4/13 (31%) were children < 4years old, and 7/13 (54%) were male. The median time between HSE and NMDARE onset was 30days (21-46). During NMDARE, symptoms differed from HSE, including increased behavioral changes (92% vs 23%, p = 0.008), movements disorders (62% vs 0%, p = 0.013), and dysautonomia (54% vs 0%, p = 0.041). Compared to 21 patients with regular HSE, patients with HSE-NMDARE more often achieved severity-associated criteria on initial MRIs, with extensive lesions (11/11, 100% vs 10/21, 48%, p = 0.005) and bilateral diffusion-weighted imaging sequence abnormalities (9/10, 90% vs 6/21, 29%, p = 0.002). Compared to 198 patients with regular NMDARE, patients with HSE-NMDARE were more frequently males (7/13, 54% vs 43/198, 22%; p = 0.015) and children < 4 (4/13, 31% vs 14/198, 7%; p = 0.016), with a worse 12-month mRS (2[1-6] vs 1[0-6], p = 0.023). Herein, patients with HSE-NMDARE have a poorer long-term prognosis than patients with regular NMDARE. We report a greater rate of severity-associated criteria on initial MRIs for HSE-NMDARE compared to regular HSE, which may help identify patients with higher risk of HSE-NMDARE.

A Unique Presentation of Excitatory Catatonia Alongside AntiNmda Receptor Encephalitis: A Case Report

Introduction: Anti-NMDA receptor encephalitis, first described in 2007, is a prominent cause of autoimmune encephalitis, predominantly affecting females. Its clinical presentation includes a prodromal phase with flu-like symptoms, followed by psychosis, unresponsiveness, hyperkinesis, and recovery [1]. Catatonia can coexist sounds better than cooccur in patients with anti-NMDA receptor encephalitis, complicating diagnosis and effective treatment. Case Summary: This case report discusses a 41-year-old African American female with a history of seizure disorder, schizoaffective disorder with catatonia, developmental delay, Catatonia in itself is not the diagnosis as per dsm 5. We could use it as a specifier with other diagnosis, be it schizoaffective disorder or seizure disorder here. So we could mention as schizoaffective disorder with catatonia. and recent bereavement. Her symptoms included variable motor hyperactivity, alternating between restlessness and sedation, with an initial Busch Francis Rating Scale score of 18. Initially diagnosed with excitatory catatonia secondary to schizoaffective disorder and treated with benzodiazepines, her condition failed to improve, prompting further investigation. Despite additional treatment for a urinary tract infection and medication adjustment, which included adding valproic acid and amantadine, her condition remained uncontrolled. Subsequent lumbar puncture revealed inflammatory markers, and her anti-NMDA receptor IgG antibody assay was positive at 1:1280, confirming the diagnosis of anti-NMDA receptor encephalitis. Treatment was escalated to include methylprednisolone and intravenous immunoglobulin, alongside six sessions of ECT for management of her catatonia. The patient showed significant improvement, achieving functional recovery and independence in daily activities before discharge. Conclusion: This case emphasizes the importance of considering anti-NMDA receptor encephalitis in patients with unexplained, refractory neuropsychiatric symptoms, such as catatonia, and highlights the value of a multidisciplinary approach in managing and rehabilitating affected individuals. Prompt diagnosis and treatment are crucial to improving outcomes and reducing the risk of persistent cognitive deficits.

Management of neuropsychiatric symptoms in anti NMDAR encephalitis: a case report

Anti-N-Methyl-D-Aspartate receptor (NMDAR) encephalitis is recognized as the most commonly diagnosed autoimmune encephalitis and is one of the most common causes of encephalitis. A wide spectrum of symptoms such as psychosis, catatonia, behavioral and memory changes, seizures, abnormal movements and autonomic dysregulation usually requires a multidisciplinary treatment approach.Early recognition and prompt initiation of immunotherapy improves patient outcomes.We present a case of a 16-year-old female who had seizures and later had catatonia and psychotic symptoms.Our patient was treated with Rituxumab when he did not get enough benefit from the first-line treatments. Significant improvement in both neurological and psychiatric symptoms occurred after rituxumab treatment.This case highlights the importance of multidisciplinary approach in patients with NMDAR encephalitis and the importance of early transition to second-line treatments in patients who do not respond adequately to first-line treatments. It is aimed to draw attention to the fact that early diagnosis and treatment can dramatically improve neuropsychiatric symptoms, and therefore clinicians should definitely remember NMDAR encephalitis in the differential diagnosis.

Challenges in the diagnosis of anti-NMDAR disease in a young male patient: a case report

BackgroundAnti-N-methyl-D-aspartate receptor (anti-NMDAR) encephalitis is an autoimmune disorder that is increasingly recognized as an important cause of autoimmune encephalitis. It is especially important to consider, because its symptoms can be severe, yet potentially treatable. The best outcome depends on prompt immunotherapy and complete tumor removal if present. Its diverse presentations often cause delay in its diagnosis and treatment.Case presentationWe describe here a 15-year-old male who developed anti-NMDA encephalitis that was a particular challenge to diagnose. The course of his disease was also complicated with sinus thrombosis. He received immunotherapy in the form of IV steroids, plasma exchange, IVIG and finally rituximab together with anticoagulation resulting in complete improvement in his condition.ConclusionsAnti-NMDAR disease should be suspected in any young individual who develops encephalopathy associated with seizures, psychiatric symptoms and/or movement disorders. Identification of NMDAR antibodies confirms the diagnosis and should prompt early intervention with immunotherapy and neoplastic workup.

The association between neurological symptoms and the outcomes of N-methyl-D-Aspartate receptor encephalitis treatment

Objective: To analyze the relationship between neurological symptoms and treatment outcomes of anti-NMDA receptor encephalitis at the Neurology Center of Bach Mai Hospital. Subjects: We selected 50 who were diagnosed with encephalitis at Bach Mai Hospital from January 1st, 2020, to October 31st, 2023. Methods: Cross-sectional descriptive study. Results: Among the 50 patients studied, 48% achieved good treatment outcomes, while 52% had poor outcomes. There was evidence of a significant difference in treatment outcomes between the group with symptoms such as consciousness disorders, dyskinesia and movement disorders, speech disorders, and dysautonomia compared to the group without these symptoms (p&lt;0.05). There was no significant difference in treatment outcomes between the group with symptoms such as seizures, memory impairment, and sleep disorders compared to the group without these symptoms (p&gt;0.05). Conclusion: In our study, 48% of patients achieved good treatment outcomes at discharge. Symptoms of consciousness disorders, dyskinesia and movement disorders, speech disorders, and dysautonomia were associated with treatment outcomes.

Chaihu Guizhi Decoction prevents cognitive, memory impairments and sensorimotor gating deficit induced by N-methyl-d-aspartate receptor antibody in mice

Ethnopharmacological relevanceAnti-NMDAR encephalitis is one of the most common types of autoimmune encephalitis, primarily presenting with prodromal symptoms, such as fever and headache, followed by a range of neurological and psychiatric symptoms. Chaihu Guizhi Decoction (CGD), a traditional Chinese medicine formulated by Zhang Zhongjing in the Eastern Han Dynasty, has been effectively used in clinical practice to treat the symptoms of Taiyang and Shaoyang disorders, including fever, headache, and psychiatric disorders. Aim of the studyTo demonstrate the protective effects of CGD in an animal model of anti-NMDAR encephalitis and explore the potential mechanisms involved. Materials and methodsUHPLC-HRMS was used to identify CGD's chemical components and serum metabolomic profiles. Network pharmacology and molecular docking were performed to predict potential targets of CGD for the treatment of anti-NMDAR encephalitis. The effect of CGD on anti-NMDAR encephalitis was evaluated using a mouse model induced by patients' antibodies. Behavioral tests were performed to assess cognitive impairment and schizophrenia-like behaviors. The effect of CGD on the cell-surface NMDAR GluN1 subunit in cultured neurons treated with patient antibodies was detected by immunofluorescence. Golgi staining was used to observe morphological changes in hippocampal dendrites. The expression of NMDAR-interacting proteins and various neuroreceptors in the hippocampus were examined to validate the targets predicted using network pharmacology and molecular docking. ResultsCGD alleviated cognitive, memory, and sensorimotor gating deficits in mice treated with anti-NMDAR encephalitis patients’ antibodies. Further experiments demonstrated the effect of CGD in preventing NMDAR reduction both in vitro and in vivo. Meanwhile, CGD regulated NMDAR-interacting proteins and dopamine receptors but did not affect hippocampal dendritic morphology and synaptic density. Additionally, CGD modifies metabolic pathways associated with anti-NMDAR encephalitis and other neurological and psychiatric disorders. ConclusionsCGD exhibited protective effects against anti-NMDAR encephalitis by mitigating the antibody-induced reduction in NMDAR and NMDAR-interacting proteins.

Ofatumumab treatment for severe refractory anti-NMDAR encephalitis: A case series

Rituximab is recommended as the preferred second-line immunotherapy for autoimmune encephalitis (AE). However, Ofatumumab (OFA), a novel fully human anti-CD20 antibody, has been reported infrequently in patients with AE. Among the various forms of AE, anti-N-methyl-d-aspartate receptor (anti-NMDAR) encephalitis is the most common and severe. This study presents three cases of severe anti-NMDAR encephalitis treated with OFA following the failure of first-line immunotherapy. The results indicated that the patients experienced no significant adverse reactions after receiving OFA, and their clinical symptoms improved markedly within one week of treatment. One month post-treatment with OFA, scores on the Glasgow Coma Scale (GCS) and the Barthel Index of Activities of Daily Living (Barthel-ADL) increased, while scores on the modified Rankin Scale (mRS), Clinical Assessment Scale in Autoimmune Encephalitis (CASE), and Paroxysmal Sympathetic Hyperactivity Assessment Measure (PSH-AM) decreased. During the three-month and six-month follow-up periods, patients exhibited further symptomatic improvement, suggesting that OFA is a safe and effective treatment option for anti-NMDAR encephalitis. These findings propose a novel therapeutic strategy for severe refractory anti-NMDAR encephalitis.