Anti-NMDA

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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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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: First episode of psychosis (FEP) is a clinical condition that usually occurs during adolescence or early adulthood and is often a sign of a future psychiatric disease. However, these symptoms are not specific, and psychosis can be caused by a physical disease in at least 5% of cases. Timely detection of these diseases, the first signs of which may appear in childhood, is of particular importance, as a curable treatment exists in most cases. However, there is no consensus in academic societies to offer recommendations for a comprehensive medical assessment to eliminate somatic causes...

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...

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)...

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...

BACKGROUND: Autoimmune encephalitis (AE) is an increasingly recognized neuroinflammatory disease entity in which early detection and treatment leads to the best clinical outcomes. Movement disorders occur in AE but their characteristics are not well defined. OBJECTIVES: To identify the frequency, classification, and prognostic significance of movement disorders in AE. METHODS: We conducted a systematic review and random-effects meta-analysis of movement disorders in cell surface antibody mediated AE...

The clinical implications of the presence of anti-N-methyl-D-aspartate receptor (NMDAR)-specific intrathecal immunoglobulin G synthesis and whether it determines the diagnosis of anti-NMDAR encephalitis have not been thoroughly investigated yet. Thus, the aim of this study was to investigate whether the detection of intrathecal anti-NMDAR-specific IgG synthesis contributes to the diagnostic confirmation of anti-NMDAR encephalitis, to disease severity, and to prognosis in patients with positive serum anti-NMDAR-IgG...

BACKGROUND: Status dystonicus (SD) is a life-threatening movement disorder emergency characterized by increasingly frequent and severe episodes of generalized dystonia, requiring urgent hospital admission. The diverse clinico-etiological spectrum, high risk of recurrence, and residual disabilities complicate functional outcomes. AIM: We aim to describe the clinico-etiological spectrum, radiology, therapeutic options, and follow-up of patients with pre-status dystonicus (pre-SD) and SD...