Anti-N-Methyl-D-Aspartate Receptor Encephalitis

Disorder characterized by symptoms of CATATONIA; HYPOVENTILATION; DYSKINESIAS; ENCEPHALITIS; and SEIZURES followed by a reduced CONSCIOUSNESS. It is often followed by a viral-like prodrome. Many cases are self-limiting and respond well to IMMUNOMODULATORY THERAPIES against the NMDA RECEPTORS antibodies.

Also Known As:

Anti-NMDA Receptor Encephalitis; Anti-NMDAR Encephalitis; Non-paraneoplastic Anti-N-Methyl-D-Aspartate Receptor Encephalitis; Non-paraneoplastic Anti-NMDA Receptor Encephalitis; Non-paraneoplastic Anti-NMDAR Encephalitis; Paraneoplastic Anti-N-Methyl-D-Aspartate Receptor Encephalitis; Paraneoplastic Anti-NMDA Receptor Encephalitis; Paraneoplastic Anti-NMDAR Encephalitis; Anti N Methyl D Aspartate Receptor Encephalitis; Anti NMDA Receptor Encephalitis; Anti NMDAR Encephalitis; Anti-N-Methyl-D-Aspartate Receptor Encephalitides; Anti-NMDA Receptor Encephalitides; Anti-NMDAR Encephalitides; Anti-NMDAR Encephalitides, Non-paraneoplastic; Anti-NMDAR Encephalitides, Paraneoplastic; Anti-NMDAR Encephalitis, Non-paraneoplastic; Anti-NMDAR Encephalitis, Paraneoplastic; Encephalitides, Anti-N-Methyl-D-Aspartate Receptor; Encephalitides, Anti-NMDA Receptor; Encephalitides, Anti-NMDAR; Encephalitides, Non-paraneoplastic Anti-NMDAR; Encephalitides, Paraneoplastic Anti-NMDAR; Encephalitis, Anti-N-Methyl-D-Aspartate Receptor; Encephalitis, Anti-NMDA Receptor; Encephalitis, Anti-NMDAR; Encephalitis, Non-paraneoplastic Anti-NMDAR; Encephalitis, Paraneoplastic Anti-NMDAR; Non paraneoplastic Anti N Methyl D Aspartate Receptor Encephalitis; Non paraneoplastic Anti NMDA Receptor Encephalitis; Non paraneoplastic Anti NMDAR Encephalitis; Non-paraneoplastic Anti-NMDAR Encephalitides; Paraneoplastic Anti N Methyl D Aspartate Receptor Encephalitis; Paraneoplastic Anti NMDA Receptor Encephalitis; Paraneoplastic Anti NMDAR Encephalitis; Paraneoplastic Anti-NMDAR Encephalitides; Receptor Encephalitides, Anti-NMDA; Receptor Encephalitis, Anti-NMDA

Networked: 804 relevant articles (47 outcomes, 69 trials/studies)

Relationship Network

Disease Context: Research Results

Related Diseases

1.	Neoplasms (Cancer)
2.	Status Epilepticus (Complex Partial Status Epilepticus)
3.	Encephalitis (Encephalitis, Rasmussen)
4.	Autoimmune Diseases of the Nervous System
5.	Seizures (Absence Seizure)

Experts

1.	Dalmau, Josep: 37 articles (01/2022 - 12/2008)
2.	Tanaka, Keiko: 17 articles (02/2022 - 01/2010)
3.	Dale, Russell C: 14 articles (11/2021 - 05/2014)
4.	Iizuka, Takahiro: 12 articles (05/2021 - 03/2009)
5.	Wang, Honghao: 12 articles (02/2021 - 01/2018)
6.	Qiu, Wei: 11 articles (01/2022 - 01/2017)
7.	Zhou, Dong: 11 articles (01/2022 - 12/2016)
8.	Shu, Yaqing: 10 articles (01/2022 - 01/2017)
9.	Graus, Francesc: 10 articles (01/2021 - 02/2013)
10.	Titulaer, Maarten J: 10 articles (12/2019 - 09/2012)

Drugs and Biologics

Drugs and Important Biological Agents (IBA) related to Anti-N-Methyl-D-Aspartate Receptor Encephalitis:

1.	Rituximab (Mabthera)FDA Link 01/01/2019 - "Our results supported that reduced dosage rituximab was effective and safe in treating anti-NMDAR encephalitis. " 12/15/2013 - "Rituximab ameliorates anti-N-methyl-D-aspartate receptor encephalitis by removal of short-lived plasmablasts." 01/01/2020 - "Conclusion: Our study provides evidence that rituximab can efficiently improve the clinical symptoms of anti-NMDAR encephalitis in children. " 01/01/2019 - "Reduced dosage rituximab in the treatment of anti-N-methyl-d-aspartate receptor encephalitis: An observation study in Chinese patients." 02/03/2023 - "Pharmaceutical care of rituximab in the treatment of children with refractory anti-NMDAR encephalitis: A case report."
2.	Intravenous Immunoglobulins (IVIG)FDA Link 09/01/2014 - "Emergency physicians should discuss the possibility of empirical intravenous immunoglobulin administration with neurology consultants if anti-NMDAR encephalitis is suspected. " 12/01/2021 - "Four patients with severe anti-NMDAR encephalitis are reported, which were refractory to the first-line immunotherapy including IVMP and IVIG. " 08/01/2012 - "A 24-year-old woman with a known diagnosis of anti-NMDA receptor encephalitis using intravenous immunoglobulin therapy became pregnant. " 12/01/2021 - "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. " 11/01/2016 - "Some entities commonly treated with IVIG in current practice, such as anti-NMDAR encephalitis and transverse myelitis, are not listed in most guidelines. "
3.	SteroidsIBA 01/01/2021 - "High dose steroids therapy was significantly associated with higher in-hospital infectious complication rates and a poor short-term prognosis in relatively severe anti-NMDAR encephalitis patients. " 04/29/2015 - "Steroid unresponsive anti-NMDA receptor encephalitis during pregnancy successfully treated with plasmapheresis." 01/01/2021 - "High Dose Steroids as First-Line Treatment Increased the Risk of In-Hospital Infections in Patients With Anti-NMDAR Encephalitis." 01/01/2021 - "Early recognition of anti-NMDAR encephalitis and initiation of treatment with steroids and immunosuppressant gained a favorable outcome. " 01/01/2019 - "[18F]fluorodeoxyglucose positron emission tomography (FDG-PET) showed pronounced relative hypermetabolism of her association cortices and a relative hypometabolism of the primary cortices, on the basis of which an anti-NMDA receptor encephalitis diagnosis was made, and treatment with a steroid pulse was initiated. "
4.	Methylprednisolone FDA LinkGeneric 10/01/2014 - "Marked improvement of anti-N-methyl-D-aspartate receptor encephalitis by large-dose methylprednisolone and plasmapheresis therapy combined with 18F-fluorodeoxyglucose positron emission tomography imaging: A case report." 01/01/2022 - "Efficacy and tolerability of intravenous immunoglobulin versus intravenous methylprednisolone treatment in anti-N-methyl-d-aspartate receptor encephalitis." 01/01/2015 - "Intrathecal treatment with methotrexate and methylprednisolone seems to be a promising alternative treatment for some paediatric cases of resistant anti-NMDAR encephalitis. " 04/05/2016 - "Intravenous immunoglobulin combined with high-dose methylprednisolone therapy is effective for severe anti-NMDAR encephalitis. " 01/01/2021 - "A 16-year-old male patient, with acute psychosis, epileptic seizure, and altered consciousness, was diagnosed as having anti-NMDAR encephalitis and treated by intravenous immunoglobulin and high-dose pulse intravenous methylprednisolone. "
5.	Immunoglobulins (Immunoglobulin)IBA 06/01/2022 - "Her serum and cerebrospinal fluid were positive for anti-NMDARs, and a diagnosis of anti-NMDAR encephalitis was made, so she received a course of intravenous methyl prednisolone and immunoglobulin and was discharged after her neurological status improved. " 01/01/2019 - "We performed continuous intracerebroventricular infusion of cerebrospinal fluid (CSF) or purified immunoglobulin (IgG) from the CSF of patients with anti-NMDAR encephalitis or polyclonal rabbit anti-NMDAR IgG, in male C57BL/6 mice. " 02/02/2019 - "The first-line immunotherapy treatment for anti-NMDAR encephalitis was short course corticosteroid (high-dose impulse therapy and oral maintenance therapy for 1 month in acute period) and (or) immunoglobulin. " 08/16/2017 - "The second patient was a 14-year-old girl with classic anti-N-methyl-D-aspartate receptor encephalitis, treated successfully with intravenously administered immunoglobulins and methylprednisolone, followed by orally administered prednisone, who developed chronic arthritis of the hip. " 01/01/2014 - "We describe a case of an 18-year-old male diagnosed with anti-NMDAR encephalitis not associated with tumor, which did not respond to initial treatment with immunoglobulin, followed by corticosteroids, cyclophosphamide and evolved with significant clinical improvement after a prolonged course of plasmapheresis. "
6.	Cyclophosphamide (Cytoxan)FDA LinkGeneric 08/25/2022 - "Case of anti-NMDA receptor encephalitis in a female adolescent with favourable response to second-line treatment with cyclophosphamide." 01/01/2022 - "This case illustrates the effective use of cyclophosphamide in the second trimester of pregnancy for anti-NMDAR encephalitis. " 01/01/2013 - "[An 8-year-old boy with anti-NMDA receptor encephalitis, successfully treated with cyclophosphamide]." 09/01/2019 - "Aggressive immunotherapy including cyclophosphamide must be necessary even for recurrent cases of anti-NMDAR encephalitis." 06/01/2017 - "[Efficacy and safety of cyclophosphamide as a sequential immunotherapy drug for anti-N-methyl-D-aspartate receptor encephalitis in children]."
7.	N-Methyl-D-Aspartate Receptors (NMDA Receptors)IBA 01/01/2023 - "The present study aimed to explore the correlation of MRI features with brain metabolism characteristics of 18F-fluorodeoxyglucose positron emission tomography (18F-FDG PET) and to describe the metabolic patterns in Anti-N-methyl-D-aspartate receptor (anti-NMDAR) encephalitis at acute and subacute phases. " 01/01/2021 - "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. " 06/01/2020 - "This study aimed to identify to resting-state cerebral blood flow (CBF) connectivity alterations in patients with anti-N-methyl-d-aspartate receptor (anti-NMDAR) encephalitis. " 01/01/2019 - "This study aimed to determine the relation between electroclinical features and cerebrospinal fluid (CSF) antibody titers in patients with anti- N-methyl-d-aspartate receptor (anti-NMDAR) encephalitis. " 09/01/2018 - "Previous studies have shown that extreme delta brush and high beta/delta power ratio on electroencephalogram are suggestive of anti- N-methyl-d-aspartate receptor (anti-NMDAR) encephalitis. "
8.	AntibodiesIBA 04/15/2023 - "When positive results were shown in IHC but negative in CBA, other kinds of antibodies associated AE instead of anti-NMDAR encephalitis should be taken into account. " 10/10/2023 - "Although these studies frequently use anti-N-methyl-D-aspartate receptor encephalitis to rationalize the findings, they rarely adopt the same rigorous investigations or address the clinical and pathogenic significance of the antibodies or discuss the limitations related to the biological sample or antibody-testing techniques. " 12/01/2018 - "In the present study, we reported 1 patient with anti-NMDAR encephalitis diagnosed by the detection of anti-NMDAR antibodies in serum and cerebrospinal fluid (CSF). " 04/28/2010 - "These studies establish the cellular mechanisms through which antibodies of patients with anti-NMDAR encephalitis cause a specific, titer-dependent, and reversible loss of NMDARs. " 12/01/2023 - "Clinical signs, neuroimaging findings, and serum analysis of anti-NMDAR antibodies confirmed the diagnosis of anti-NMDAR encephalitis. "
9.	Biomarkers (Surrogate Marker)IBA 01/01/2018 - "Marked medial occipital lobe hypometabolism by dedicated brain FDG-PET/CT may serve as an early biomarker for discriminating anti-NMDA receptor encephalitis from other AE. Resolution of lateral and medial occipital hypometabolism may correlate with improved neurologic status in anti-NMDA receptor encephalitis." 06/01/2022 - "This study examined neurovascular coupling and cognitive function in anti-NMDAR encephalitis patients to identify prognostic biomarkers, reveal potential pathogenic mechanisms, and provide clues to possible therapeutic strategies. " 01/01/2021 - "The current study provides a comprehensive view of characteristic multimodal network dysfunction in anti-NMDAR encephalitis, which is crucial to establish new diagnostic biomarkers and promising therapeutic targets for the disease." 10/05/2023 - "The T1-weighted/T2-weighted ratio as a biomarker of anti-NMDA receptor encephalitis." 07/22/2023 - "Elevated blood and cerebrospinal fluid biomarkers of microglial activation and blood‒brain barrier disruption in anti-NMDA receptor encephalitis."
10.	N-Methylaspartate (NMDA)IBA 02/01/2020 - "This study aimed to investigate brain white matter (WM) changes and their relationship to cognition in patients with anti-N-methyl-D-aspartate (anti-NMDA) receptor encephalitis. " 01/01/2022 - "Anti-N-methyl-D-aspartate (anti-NMDA) receptor encephalitis is a condition that was only identified relatively recently. " 01/01/2022 - "Impact statement Patients with anti-N-methyl-D-aspartate (anti-NMDA) receptor encephalitis are often left with permanent memory impairments. " 01/01/2022 - "Background: Patients with postacute anti-N-methyl-D-aspartate (anti-NMDA) receptor encephalitis are often left with permanent memory impairments. " 01/01/2021 - "Anti-N-methyl-D-aspartate (anti-NMDA) receptor encephalitis is an immune-mediated syndrome that is still under-recognised, with grave consequences if not treated early. "

Therapies and Procedures

1.	Immunotherapy 06/02/2021 - "Immunotherapy for anti-NMDAR encephalitis is effective for many patients, but recovery is prolonged and relapses can occur." 01/01/2020 - "Re-enforced first-line immunotherapy is effective in managing anti-NMDAR encephalitis in the acute phase. " 01/01/2019 - "Immunotherapy is an effective treatment for anti-NMDA receptor encephalitis. " 06/18/2022 - "All patients underwent immunotherapy as first-line treatment for anti-NMDAR encephalitis. " 02/01/2022 - "As a strategy to protect embryonic neurons, our findings may support the efficacy of early immunotherapy for anti-NMDAR encephalitis in pregnancy."
2.	Therapeutics 01/01/2019 - "Early second-line therapy is associated with improved episodic memory in anti-NMDA receptor encephalitis." 09/01/2011 - "[Dramatic improvement in two cases of anti-NMDA receptor encephalitis after immunomodulating therapy]." 06/01/2022 - "The case report highlights the risk factors, disease course, and treatment modalities of anti-NMDA receptor encephalitis with special emphasis on the subsect of patients who may not respond to first-line therapies." 03/01/2022 - "MDs are a core feature of anti NMDAR encephalitis, particularly in the paediatric age group, understanding and characterization of which, is the key to early diagnosis and effective therapy." 12/01/2021 - "Successful treatment with immunoadsorption therapy in four patients with severe and refractory anti-N-methyl-D-aspartate receptor encephalitis."
3.	Plasma Exchange 01/01/2019 - "Efficacy of Therapeutic Plasma Exchange in Patients with Severe Refractory Anti-NMDA Receptor Encephalitis." 04/01/2023 - "This review provides an overview of the use of therapeutic plasma exchange including the principle and mechanisms, the evidence, initial time, efficiency and complications in anti-NMDAR encephalitis." 04/01/2023 - "However, several aspects of treatment remain incompletely clarified, and treatment strategies are still heterogeneous, especially with regard to therapeutic plasma exchange in anti-NMDAR encephalitis. " 04/01/2023 - "Therapeutic plasma exchange in anti-N-methyl-D-aspartate receptor encephalitis." 08/01/2021 - "This report highlights the role of plasma exchange in anti-NMDA receptor encephalitis in pediatric patients. "
4.	Plasmapheresis 12/01/2016 - "Successful treatment of anti-NMDA receptor encephalitis with a prompt ovarian tumour removal and prolonged course of plasmapheresis: A case report." 01/01/2019 - "These cases show that anti-NMDAR encephalitis is a treatable condition and its early recognition together with appropriate treatment (immunotherapy/plasmapheresis) are essential for a favorable evolution. " 08/01/2018 - "This report describes a Successful treatment of anti-NMDA receptor encephalitis with early teratoma removal and plasmapheresis. " 08/01/2018 - "Successful treatment of anti-NMDA receptor encephalitis with early teratoma removal and plasmapheresis: A case report." 01/01/2014 - "A successful case of anti-NMDAR encephalitis without tumor treated with a prolonged regimen of plasmapheresis."
5.	Immunomodulation 01/01/2016 - "Anti-NMDAR encephalitis requires prolonged immunomodulatory therapy. " 07/07/2023 - "The aim of this study was to evaluate the effectiveness of intra-thecal methotrexate as immunomodulatory therapy for refractory anti-NMDA receptor encephalitis. " 07/07/2023 - "Intra-thecal methotrexate may be a potentially effective and relatively safe escalation option for immunomodulatory therapy of refractory anti-NMDA receptor encephalitis. " 03/01/2018 - "Immunomodulatory therapy has shown some therapeutic benefits in patients with anti-N-methyl-D-aspartate receptor (anti-NMDAR) encephalitis. " 03/01/2023 - "Although the primary treatment for catatonia associated with anti-N-methyl-D-aspartate receptor encephalitis is immunomodulatory therapy paired with benzodiazepines, electroconvulsive therapy has been shown to be an effective and safe adjuvant treatment that is especially useful for management of excited catatonia."