Anti-NMDA Encephalitis

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OBJECTIVE: This review examines the use and outcomes of neuropsychological tests (NPT) in adults with autoimmune encephalitis (AE) with the objective of providing recommendations for NPT for clinical practice, observational research, and clinical trials. DATA SELECTION: PubMed search from inception to February 2023 yielded 8858 results which were screened for inclusion/exclusion criteria: ≥ one comprehensive NPT with comparison/normative reference value available of individual NPT scores, NPT reported within specific neuronal antibody group, English manuscript available, and sample size >5 (no case reports)...

BACKGROUND: Anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis rarely causes visible lesions in conventional MRI, yet advanced imaging detects extensive white matter damage. To improve prognostic capabilities, we evaluate the T1-weighted/T2-weighted (T1w/T2w) ratio, a measure of white matter integrity computable from clinical MRI sequences, in NMDAR encephalitis and examine its associations with cognitive impairment. METHODS: T1-weighted and T2-weighted MRI were acquired cross-sectionally at 3 Tesla in 53 patients with NMDAR encephalitis (81% women, mean age 29 years) and 53 matched healthy controls...

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BACKGROUND: There is mounting evidence suggesting that autoimmune encephalitis (AE) can be observed as a neurological complication in patients with COVID-19. This review aimed to summarize the clinical manifestations, types, and outcomes of COVID-19-associated AE. METHODS: A systematic search was conducted in the PubMed, Embase, and Web of Science databases to identify case reports and case series related to COVID-19-associated AE from 1 January 2020 to 31 March 2023...

Anti-NMDA receptor autoantibodies (NMDAR-Abs) in patients with NMDAR encephalitis cause severe disease symptoms resembling psychosis and cause cognitive dysfunction. After passive transfer of patients' cerebrospinal fluid or human monoclonal anti-GluN1-autoantibodies in mice, we find a disrupted excitatory-inhibitory balance resulting from CA1 neuronal hypoexcitability, reduced AMPA receptor (AMPAR) signaling, and faster synaptic inhibition in acute hippocampal slices. Functional alterations are also reflected in widespread remodeling of the hippocampal proteome, including changes in glutamatergic and GABAergic neurotransmission...

BACKGROUND/AIMS: Early diagnosis of Anti-N-methyl-D-aspartate receptor (anti-NMDAR) encephalitis with non-invasive imaging modalities benefiting is crucial to guarantee prompt treatments decision-making and good prognosis for patients. The present study aimed to explore the correlation of MRI features with brain metabolism characteristics of 18 F-fluorodeoxyglucose positron emission tomography (18 F-FDG PET) and to describe the metabolic patterns in Anti-N-methyl-D-aspartate receptor (anti-NMDAR) encephalitis at acute and subacute phases...

Autoimmune encephalitis with anti-NMDA (N-methyl-D-aspartate) receptor encephalitis is the most common type. This condition can be associated with underlying malignancy, making appropriate screening essential. Prompt identification and intervention of autoimmune encephalitis is essential for a successful outcome and full recovery from this serious and potentially fatal condition. Immunotherapy is typically used to suppress the immune response and reduce brain inflammation. The choice of therapy depends on the type and severity of autoimmune encephalitis, as well as the presence of an underlying tumour...

BACKGROUND: Previous studies suggest a relationship between central nervous system inflammatory demyelinating diseases and anti-N-methyl-d-aspartate receptor (NMDAR) encephalitis. Also, the overlap between anti-NMDAR encephalitis and multiple sclerosis (MS) has been reported. However, the pathogenesis and clinical characteristics are still obscure. CASE PRESENTATION: A 33-year-old woman presented with diplopia and sensory ataxia at the onset. The cerebrospinal fluid (CSF) anti-NMDAR antibodies were positive (1:3...

Pediatric and adult autoimmune encephalitis (AE) are often associated with Abs to the NR1 subunit of the N-methyl-d-aspartate (NMDA) receptor (NMDAR). Very little is known regarding the cerebrospinal fluid humoral immune profile and Ab genetics associated with pediatric anti-NMDAR-AE. Using a combination of cellular, molecular, and immunogenetics tools, we collected cerebrospinal fluid from pediatric subjects and generated 1) flow cytometry data to calculate the frequency of B cell subtypes in the cerebrospinal fluid of pediatric subjects with anti-NMDAR-AE and controls, 2) a panel of recombinant human Abs from a pediatric case of anti-NMDAR-AE that was refractory to treatment, and 3) a detailed analysis of the Ab genes that bound the NR1 subunit of the NMDAR...

Anti-N-Methyl-D-Aspartate Receptor (NMDAR) antibody encephalitis is a potentially lethal form of autoimmune encephalitis in which auto-antibody production occurs against the NMDAR, NR1 subunit, causing massive dysregulation of neuro-transmission. This syndrome is often paraneoplastic (ovarian teratomas) having a male-to-female ratio of 1:4 and most often manifests with neuropsychiatric signs and symptoms, including hallucinations, memory loss, and diminished conscious level leading to death. Immunotherapy and surgical removal of the predisposing malignancy often result in prompt relief of symptoms...

Encephalitis is a disease typically caused by viral infections or autoimmunity. The most common type of autoimmune encephalitis is anti-N-methyl-D-aspartate receptor (NMDAR) antibody-mediated, known as anti-NMDA receptor encephalitis, which is a rare disease. Specific EEG patterns, including "extreme delta brush" (EDB), have been reported in patients with anti-NMDA receptor encephalitis. The aim of this study was to develop an intelligent diagnostic model for encephalitis based on EEG signals. A total of 131 Participants were selected based on reasonable inclusion criteria and divided into three groups: health control (35 participants), viral encephalitis (58 participants), and anti NMDAR receptor encephalitis (55 participants)...

OBJECTIVE: To report a rare case of anti-N-Methyl-d-aspartate receptor encephalitis (anti-NMDARE) presented by mental and behavioral changes and seizures accompanied with respiratory failure. CASE REPORT: A 37-year-old multiparous woman was initially presented with abnormal mental behavior and the diagnosis of schizophrenia was made, but the disease progressed rapidly to general convulsion and acute respiratory failure. Although active treatment, including steroids, intravenous immunoglobulins (IVIGs) and plasma exchange was applied, no significant improvement was obtained...

New onset refractory status epilepticus (NORSE) is a rare but critical condition characterized by refractory status epilepticus (RSE) in an individual without prior history of epilepsy or known structural, toxic, or metabolic cause. Postinfectious immune activation is an important cause of NORSE. Early testing for autoimmune antibodies is strongly recommended (Wickstrom et al., 2022). We report a case of NORSE triggered by Japanese encephalitis (JE) in an unvaccinated US adult traveler. Her CSF later revealed positive anti-N-methyl-d-aspartate (NMDA)-receptor antibody...

Anti-NMDA receptor (Anti-NMDAR) encephalitis is an autoimmune disease that presents with diverse symptoms. Since literature is scarce on the overlap with multiple sclerosis (MS), this report aims to elucidate the distinctive clinical presentation and diagnostic challenges of anti-NMDAR encephalitis in MS patients. A 73-year-old woman with secondary progressive multiple sclerosis, after experiencing status epilepticus and subsequent non-convulsive status epilepticus, presented with neuropsychiatric symptoms and autonomic nervous dysfunction...

Anti-NMDA receptor encephalitis (NMDARE), an autoimmune encephalitis associated with autoantibodies against the N-methyl-D-aspartate (NMDA) receptor, affects predominantly young women and is associated with psychiatric symptoms, seizures, movement disorders, and autonomic instability. Traditional treatments of anti-NMDA receptor encephalitis involve corticosteroids, intravenous immunoglobulin, plasmapheresis, cyclophosphamide, and rituximab. However, many controversies remain in the treatment for NMDA receptor encephalitis, such as optimal timing and combination of different immunotherapies, the role of newer strategies (e...

Anti-N-methyl-D-aspartate (anti-NMDA) receptor encephalitis is an autoimmune disease triggered by antibodies against the NR1 subunit of this receptor. It has a wide variety of presentations, including abnormal behavior, psychosis, seizures, abnormal movement, insomnia, and irritability. The diagnosis is confirmed by the presence of one of the six main symptoms and anti-NR1 immunoglobulin G (IgG)-positive antibodies in the cerebrospinal fluid (CSF) after the exclusion of other disorders. We present a case of an 18-year-old female with progressive paresthesia and muscle weakness that compromised walking and psychiatric symptoms...

UNLABELLED: Anti-N-methyl-D-aspartate receptor (Anti-NMDAR) encephalitis is a rare autoimmune disease, characterized by the presence of neuropsychiatric symptoms. It is sometimes mistaken for a psychiatric disorder and other times not considered in the differential diagnosis of an encephalitic process. Correct identification of this disease and prompt treatment are key for optimal recovery, which might take weeks to months. Many patients manifest severe symptoms, with depressed level of consciousness, breathing dysfunction and dysautonomia requiring admission to the Intensive Care Unit (ICU)...

BACKGROUND AND OBJECTIVES: Neural antibodies are detected by tissue-based indirect immunofluorescence assay (IFA) in Mayo Clinic's Neuroimmunology Laboratory practice, but the process of characterizing and validating novel antibodies is lengthy. We report our assessment of human protein arrays. METHODS: Assessment of arrays (81% human proteome coverage) was undertaken using diverse known positive samples (17 serum and 14 CSF). Samples from patients with novel neural antibodies were reflexed from IFA to arrays...

OBJECTIVE: Anti-N-methyl-D-aspartate receptor encephalitis (anti-NMDARE) is autoimmune encephalitis with a characteristic neuropsychiatric syndrome and persistent cognition deficits even after clinical remission. The objective of this study was to uncover the potential noninvasive and quantified biomarkers related to residual brain distortions in convalescent anti-NMDARE patients. METHODS: Based on resting-state electroencephalograms (EEG), both power spectral density (PSD) and brain network analysis were performed to disclose the persistent distortions of brain rhythms in these patients...