Autoimmune Diseases of the Nervous System

Disorders caused by cellular or humoral immune responses primarily directed towards nervous system autoantigens. The immune response may be directed towards specific tissue components (e.g., myelin) and may be limited to the central nervous system (e.g., MULTIPLE SCLEROSIS) or the peripheral nervous system (e.g., GUILLAIN-BARRE SYNDROME).

Also Known As:

Autoimmune Diseases, Nervous System; Autoimmune Diseases, Neurologic; Autoimmune Disorders of the Nervous System; Autoimmune Disorders, Nervous System; Autoimmune Nervous System Diseases; Immune Diseases, Nervous System; Nervous System Immune Diseases; Nervous System Immune Disorders; Neurologic Autoimmune Diseases; Antibody Mediated Encephalitis; Antibody-Mediated Encephalitides; Autoimmune Disease, Neurologic; Autoimmune Encephalitides; Encephalitis, Antibody-Mediated; Encephalitis, Autoimmune; Neurologic Autoimmune Disease; Antibody-Mediated Encephalitis; Autoimmune Encephalitis; Immune Disorders, Nervous System; Nervous System Autoimmune Diseases

Networked: 1576 relevant articles (62 outcomes, 103 trials/studies)

Relationship Network

Disease Context: Research Results

Related Diseases

1.	Multiple Sclerosis
2.	Myasthenia Gravis
3.	Epilepsy (Aura)
4.	Encephalitis (Encephalitis, Rasmussen)
5.	Glioma (Gliomas)

Experts

1.	Prüss, Harald: 41 articles (01/2024 - 08/2013)
2.	Dalmau, Josep: 36 articles (01/2024 - 01/2010)
3.	Leypoldt, Frank: 30 articles (02/2024 - 01/2013)
4.	Irani, Sarosh R: 21 articles (03/2024 - 04/2016)
5.	Graus, Francesc: 21 articles (12/2023 - 01/2010)
6.	Bien, Christian G: 18 articles (02/2024 - 01/2013)
7.	Chu, Kon: 18 articles (01/2024 - 12/2014)
8.	Lee, Soon-Tae: 18 articles (01/2023 - 12/2014)
9.	Honnorat, Jérôme: 17 articles (05/2024 - 05/2012)
10.	Melzer, Nico: 16 articles (01/2024 - 04/2016)

Drugs and Biologics

Drugs and Important Biological Agents (IBA) related to Autoimmune Diseases of the Nervous System:

1.	AntibodiesIBA 01/01/2023 - "Early diagnosis and treatment of autoimmune encephalitis after HSVE may be associated with a better outcome so that high clinical awareness and routine testing for anti-NMDAR-antibodies after HSVE seems advisable. " 04/01/2016 - "This study provides Class IV evidence that immunoadsorption combined with immunosuppression therapy is effective in patients with autoimmune encephalitis with surface antibodies." 01/01/2022 - "B-cell modulation with anti-CD79b antibodies ameliorates experimental autoimmune encephalitis in mice." 12/09/2021 - "The scale assessment was helpful in predicting the presence of antibodies to autoimmune encephalitis. " 05/01/2023 - "In contrast to that seen with other autoimmune encephalitis-related antibodies, basic studies have demonstrated that IgLON5 antibody-induced neuronal damage and degeneration are irreversible, indicative of a potential link between autoimmunity and neurodegeneration in anti-IgLON5 disease. "
2.	Intravenous Immunoglobulins (IVIG)FDA Link 01/01/2022 - "These data provide the prospective evidence regarding the efficacy of IVIG in improving the functional outcomes of autoimmune encephalitis." 01/01/2022 - "The safety and efficacy of intravenous immunoglobulin in autoimmune encephalitis." 01/01/2022 - "This single-arm, open-label study assessed the efficacy and safety of 10% intravenous IVIG treatment in newly diagnosed patients with possible autoimmune encephalitis. " 05/01/2020 - "An Eye for an Eye: A Randomized Placebo-Controlled Trial of IVIG in Antibody-Mediated Encephalitis." 12/01/2023 - "Treatment of Cognitive Deficits and Behavioral Symptoms Following COVID-19-Associated Autoimmune Encephalitis With Intravenous Immunoglobulin: A Case Report and Review of the Literature."
3.	Methylprednisolone FDA LinkGeneric 11/12/2022 - "Since the patient was diagnosed with autoimmune encephalitis, intravenous methylprednisolone was administered, which yielded a positive outcome." 06/01/2007 - "He was suspected to have autoimmune encephalitis and his symptoms improved after treated with methylprednisolone (1 g/day for 3 days). " 04/01/2024 - "Under suspicion of autoimmune encephalitis, methylprednisolone was administered. " 01/01/2023 - "We diagnosed him with autoimmune encephalitis and initiated intravenous methylprednisolone, after which, the patient's symptoms rapidly subsided." 11/01/2021 - "Initial response to IV methylprednisolone followed by recurrence of symptoms strengthened our suspicion for autoimmune encephalitis. "
4.	N-Methyl-D-Aspartate Receptors (NMDA Receptors)IBA 01/01/2024 - "The objective of this study was to elucidate the contribution of cerebrospinal fluid (CSF) antibody titers (AT) and sex to acute cerebral blood flow (CBF) in patients diagnosed with anti-N-methyl-d-aspartate receptor autoimmune encephalitis (NMDAR AE). " 09/01/2020 - "Therefore, we conducted this study to analyze the clinical features, electroencephalogram (EEG) characteristics, treatment and prognosis of autoimmune encephalitis in children with negative and positive anti-N-methyl-D-aspartate receptor (NMDAR) antibody. " 05/07/2020 - "Many studies were performed, including electroencephalogram (EEG), head CT, laboratory work, urine drug screen and lumbar puncture with cerebral spinal fluid studies, which ultimately led to the diagnosis of anti-N-methyl-D-aspartate receptor (NMDAR) autoimmune encephalitis. " 12/01/2023 - "The Management and Diagnosis of Anti-NMDA Receptor Autoimmune Encephalitis in Pregnant Women: A Case Report and Literature Review." 01/01/2023 - "Pediatric anti-NMDA-receptor autoimmune encephalitis in siblings: Developmental, Electrophysiologic, and Genetic Implications."
5.	Peptides (Polypeptides)IBA 08/16/2016 - "In addition, pretreatment with MOG35-55 peptide-pulsed Mino-DCs ameliorates clinical signs of experimental autoimmune encephalitis induced by MOG peptide injection. " 01/27/2024 - "However, whether autoimmune encephalitis involves extracellular deposition of Aβ peptides in the brain is unreported. " 08/28/2021 - "Here, we provide an overview of the recent literature about peptide-based systems tested on experimental autoimmune encephalitis (EAE) models. " 01/01/2017 - "A decoy peptide disrupting the IL-17RA-NOTCH1 interaction inhibits IL-17-induced NOTCH1 activation and attenuates Th17-mediated experimental autoimmune encephalitis (EAE). " 06/01/2014 - "Several of the major pathological CNS alterations and behavioral deficits of MS are recapitulated in the experimental autoimmune encephalitis (EAE) mouse model in which the disease process is induced by administration of myelin peptides. "
6.	Adrenal Cortex Hormones (Corticosteroids)IBA 01/01/2023 - "Although she was initially treated with corticosteroids for presumed sero-negative autoimmune encephalitis, her symptoms persisted. " 01/01/2021 - "Autoimmune encephalitis was identified by brain biopsy and the patient recovered with corticosteroids treatment. " 12/01/2004 - "The patient was dismissed with the primary diagnosis of autoimmune encephalitis of the brainstem and put on oral corticosteroids. " 01/01/1996 - "Specifically, the hypothalamic-pituitary-adrenal (HPA) system seems crucial because (a) the inflammatory response is accompanied by HPA activation; (b) animal models with an inherited HPA defect are prone to developing experimental autoimmune encephalitis; and (c) most important, corticosteroids are still the most widely used treatment. " 09/01/2018 - "A subacute onset, rapidly progressive painful, pure motor quadriparesis; Ganglionopathy in elderly and autoimmune encephalitis with ill sustained or no response to corticosteroids merits consideration of paraneoplastic etiology."
7.	Glatiramer Acetate (Copaxone)FDA Link 01/01/2014 - "Glatiramer acetate (GA, Copaxone, Copolymer-1) is an FDA approved drug for the treatment of MS and it is very effective in suppressing neuroinflammation in experimental autoimmune encephalitis (EAE), an animal model of MS. Although this drug was designed to inhibit pathogenic T cells, the exact mechanism of EAE/MS suppression by GA is still not well understood. " 01/01/2001 - "Glatiramer acetate has been shown to be effective in preventing and suppressing experimental autoimmune encephalitis (EAE), the animal model of multiple sclerosis (MS). " 11/01/2003 - "Glatiramer acetate (GA; Copaxone, also known as Copolymer 1 or Cop-1), a copolymer of amino acids, is very effective in the suppression of experimental autoimmune encephalitis (EAE), the animal model for multiple sclerosis (MS), in various species including primates. "
8.	AutoantibodiesIBA 01/01/2022 - "Autoimmune encephalitis and central nervous system (CNS) demyelinating autoantibodies in the CSF also showed positive results. " 01/01/2022 - "Although the exact mechanisms remain elusive, autoantibodies derived from patients with autoimmune encephalitis pose a unique tool to study pathogenesis of neuropsychiatric states. " 07/01/2021 - "This study aimed to assess the role of Tregs among patients with autoimmune encephalitis (AE) with different autoantibody types and to evaluate their association with clinical features. " 01/01/2021 - "Background: In our previous single-center study of autoimmune encephalitis (AE) related autoantibody test results we found positivity in 60 patients out of 1,034 with suspected AE from 2012 through 2018 as part of a Hungarian nationwide program. " 12/01/2019 - "Single-center study of autoimmune encephalitis-related autoantibody testing in Hungary."
9.	Proteins (Proteins, Gene)FDA Link 05/09/2024 - "Autoimmune encephalitis due to glial fibrillar acidic protein (GFAP) astrocytopathy is a rare cause of subacute neuropsychiatric changes. " 10/01/2023 - "To ascertain the clinical characteristics of pediatric patients with contactin-associated protein-like 2 (CASPR2) antibody-associated autoimmune encephalitis (AEs). " 10/01/2023 - "Contactin-associated protein-like 2 antibody-associated autoimmune encephalitis in children: case reports and systematic review of literature." 01/01/2023 - "Anti-contactin-associated protein 2 (CASPR2)-related autoimmune encephalitis (AE) is more common in adults than in children. " 01/01/2023 - "Clinical spectrum of contactin-associated protein 2 autoimmune encephalitis in children."
10.	Leucine (L-Leucine)FDA Link 01/01/2022 - "The First Case of Familiar Anti-leucine-rich Glioma-Inactivated1 Autoimmune Encephalitis: A Case Report and Literature Review." 11/01/2023 - "Our study aimed to investigate the utility of 18 F-FDG PET imaging in diagnosing and monitoring patients with anti-leucine-rich glioma-inactivated 1 antibody autoimmune encephalitis (anti-LGI1 AE). " 12/01/2024 - "Leucine-rich glioma inactivated 1 (LGI1) antibody-related autoimmune encephalitis is easily misdiagnosed clinically because of its complex and diverse clinical manifestations. " 05/01/2024 - "Relapses occur in 15%-25% of patients with leucine-rich glioma-inactivated 1 antibody (LGI1-Ab) autoimmune encephalitis and may cause additional disability. " 04/17/2023 - "A faciobrachial dystonic seizure was witnessed during his hospitalisation, ultimately leading to a diagnosis of leucine-rich glioma-inactivated 1 (LGI-1) autoimmune encephalitis. "

Therapies and Procedures

1.	Immunotherapy 05/01/2024 - "Rapid and accurate diagnosis of autoimmune encephalitis encourages prompt initiation of immunotherapy toward improved patient outcomes. " 01/19/2024 - "Early identification of autoimmune encephalitis and timely administration of immunotherapy may lead to a better outcome." 01/01/2020 - "Immunotherapy is the mainstay of treatment in autoimmune encephalitides. " 01/01/2022 - "This study aimed to determine the clinical characteristics and evaluate the efficacy of immunotherapy and the long-term prognosis of severe autoimmune encephalitis (AE) in China. " 12/01/2021 - "Autoimmune encephalitis was diagnosed by cerebrospinal fluid examination, and the patient was cured and discharged after a period of immunotherapy."
2.	Therapeutics 02/05/2022 - "Introduction: For more than a decade, NMDAR autoimmune encephalitis has been studied and treated as a neurological condition, and good results have been achieve through immune therapies. " 06/01/2022 - "Observational data suggest that B-cell-depleting therapies are effective for antibody-mediated autoimmune encephalitis. " 01/01/2024 - "B-cell targeted therapies in autoimmune encephalitis: mechanisms, clinical applications, and therapeutic potential." 11/01/2023 - "The choice of therapy depends on the type and severity of autoimmune encephalitis, as well as the presence of an underlying tumour." 01/01/2023 - "Therapy response in seronegative versus seropositive autoimmune encephalitis."
3.	Plasma Exchange 02/26/2023 - "Further detection of Cerebrospinal fluid Japanese B encephalitis virus Polymerase Chain Reaction positive, serum autoimmune encephalitis antibody showed CASPR-2 antibody positive (1:320), the patient's condition gradually improved after plasma exchange treatment. " 06/01/2024 - "Suspected autoimmune encephalitis: A retrospective study of patients referred for therapeutic plasma exchange." 01/01/2021 - "To determine the clinical and antibody response after therapeutic plasma exchange (TPE) in patients with severe refractory antibody-associated autoimmune encephalitis (AE). " 01/01/2021 - "Clinical efficacy of plasma exchange in patients with autoimmune encephalitis." 12/01/2016 - "Immunoadsorption or plasma exchange in the treatment of autoimmune encephalitis: a pilot study."
4.	Immunomodulation 01/01/2022 - "Immunomodulation in the acute phase of autoimmune encephalitis." 12/03/2019 - "However, the symptoms were later deteriorated, we considered the possibility of autoimmune encephalitis and, consequently, conducted an immunomodulatory therapy. " 03/01/2017 - "He received successive immunomodulatory therapy for autoimmune encephalitis and psychiatric medication. " 12/03/2019 - "Herein, we report the case of a patient suspected with autoimmune encephalitis after brain MRI and treated with immunomodulation therapy. " 01/01/2021 - "Early immunomodulatory therapies should be carefully considered if criteria of probable autoimmune psychosis or possible autoimmune encephalitis are fulfilled, as it may prevent long-term disabilities. "
5.	Aftercare (After-Treatment) 01/01/2016 - "Autoimmune encephalitis may relapse, so follow-up care is important. " 01/01/2020 - "The cerebral glucose metabolism was evaluated by FDG-PET/CT during acute-to-subacute stage of autoimmune encephalitis and after treatment. " 01/01/2023 - "Few cases of autoimmune encephalitis with immunotherapy have been described in the literature and optimal clinical management of these events as well as patient's immune-mediated response after treatment suspension is still unclear. " 01/01/2022 - "Daratumumab allowed a substantial clinical improvement in all patients, as measured by modified Rankin Scale (mRS; before treatment: mRS =5, n = 7; after treatment: median mRS =4, range = 0-5), Clinical Assessment Scale in Autoimmune Encephalitis (from median 21 to 3 points, n = 5), Inflammatory Neuropathy Cause and Treatment disability score (from 7 to 0 points, n = 1), and Quantitative Myasthenia Gravis score (from 16 to 8 points, n = 1). " 10/01/2021 - "Accelerated Course of Alveolar Echinococcosis After Treatment With Steroids in a Patient With Autoimmune Encephalitis."