View clinical trials related to Autoimmune Encephalitis.
Filter by:Anti-leucine-rich glioma-inactivated 1 (LGI1) encephalitis has been increasingly identified as the second most common type of autoimmune encephalitis after anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis. It presents with acute or subacute onset of epileptic seizures, anterograde amnesia, behavior disturbances, sleep disorders and hyponatremia. In most patients with anti-LGI1 encephalitis, immunotherapy is successful in treating the encephalitis. However, relapses, chronic epilepsy, cognitive declines and psychiatric problems have been reported in some cases. So far, prospective studies to evaluate its clinical outcomes still remain limited. In this project, the investigators will use clinical features and advanced paraclinical examinations to prospectively investigate the clinical outcomes and the associated factors in patients with anti-LGI1 encephalitis.
Autoimmune encephalitis (AE) is an immune-mediated brain disorder characterized by varied clinical manifestations that correlate with specific types of antibodies.Typical symptoms include acute behavioral changes, psychosis, seizures, memory deficits, dyskinesias, speech impairments, and autonomic and respiratory dysregulation.While the majority of patients respond well to immunotherapeutic agents, a significant proportion remains resistant to initial and secondary-line immunotherapies.Minocycline, a semisynthetic tetracycline, is notably used for the central nervous system due to its lipophilic characteristics and its capacity to penetrate the blood-brain barrier. While the primary neuroprotective focus of minocycline in the central nervous system remains unknown, the primary effects of minocycline include the inhibition of microglial activation, mitigation of apoptosis, and reduction in reactive oxygen species generation.Protective effect has been observed in hypoxic injury, ischemic stroke, amyotrophic lateral sclerosis, traumatic spinal cord injury, multiple sclerosis, Parkinson's disease, and Huntington's disease.Can minocycline offer a protective role in AE? Consequently, we proposed a randomized, controlled trial to investigate the efficacy of minocycline in AE.
The goal of this retrospective observational study is to compare brain fluorodeoxyglucose-positron emission tomography (FDG-PET) of patients with autoimmune encephalitis, normal controls and patients with Alzheimer's disease (AD). The main question it aims to answer is: •is there a specific pattern of brain metabolism in patients with autoimmune encephalitis Participants data and images will be retrospectively collected from hospital records, and FDG-PET images will be analyzed by means of statistical parametric mapping (SPM). Controls will be selected from validated public databases.
Autoimmune encephalitis (AE) and paraneoplastic neurological syndromes (PNS) are rare neuroimmune syndromes with a wide range of clinical presentation but without pathognomonic clinical sign facilitating the diagnosis. A lot of differential diagnoses are possible such as neurodegenerative diseases or viral infections. Although rare the diagnosis of AE or PNS is essential because despite severe neurological symptoms, patients can be cured by appropriate immunotherapy. Autoantibodies highly specific of AE and PNS has been described in the serum and cerebrospinal fluid of the patients and can be used as biomarkers of the disease. Their presence can predict an autoimmune origin and in many cases a good prognosis after immunotherapy. However, if some autoantibodies are now well-characterized and industrial kits have been developed to detect them, in numerous cases of highly suspect AE or PNS no specific autoantibodies are identified leading frequently to an inappropriate treatment. Furthermore, as the mechanisms of AE and PNS is still unknown, treatments are not optimal and in some cases inefficient. There is no prognosis biomarker able to predict the patient's sensitivity to immunotherapy and there are only few clues to know how the immune system can provoke the neuropsychiatric symptoms observed in the patients.
Autoimmune encephalitis is brain inflammation caused by the immune system mistakenly reacting against proteins in the brain. The commonest form is called NMDAR-antibody encephalitis (N-methyl-D-aspartate receptor antibody encephalitis), a rare condition which mainly affects children and young people and causes difficulties in memory, thinking and mental health which can have significant long-term impacts on education, employment and quality of life. In this project we will use advanced magnetic resonance imaging (MRI) to measure changes in the structure, function and chemistry of the brains of children and young people who are in early recovery from NMDAR-antibody encephalitis and other forms of immune-mediated encephalitis. We will investigate if MRI measurements in patients differ from those in healthy people, and if they can help predict patient outcome one year later, assessed by tests of memory, thinking, mental health and functioning in daily life.
Autoimmune encephalitis are characterized by the subacute development of memory deficits, altered mental status, and psychiatric symptoms, generally in association with anti-neuronal antibodies. Two main groups of autoimmune encephalitis may be distinguished based on the location of the targeted antigen: 1) Intracellular antigens, in which the antibodies are thought not to be pathogenic, and the disorders are usually strongly associated with cancer, constituting therefore paraneoplastic neurological syndromes; 2) Synaptic proteins and surface receptors, in which the antibodies are pathogenic and the frequency of cancer is variable depending on the antibody and the demographic characteristics of the patient. Encephalitis with antibodies against N-methy-D-aspartate receptor is the most common autoimmune encephalitis, being even more frequent than infectious etiologies. It is characterized by subacute onset of memory deficits, psychiatric symptoms, speech dysfunction, seizures, movement disorders, decreased level of consciousness, dysautonomia and central hypoventilation. Nearly 50% of women with anti-NMDAR encephalitis have an ovarian teratoma, while associated tumors in elderly patients are usually carcinomas. In contrast, most cases in children and young men are non-paraneoplastic. Recently, herpes-simplex encephalitis has been described as another trigger of NMDAR encephalitis. Conversely, for the vast majority of the non-paraneoplastic autoimmune encephalitis, no acquired triggers have been described so far. In addition to acquired susceptibility, genetic predisposition may also be important in the pathogenesis of autoimmune encephalitis. The human leukocyte antigen (HLA) is the genetic factor most frequently associated with autoimmune diseases, and it has been already linked to a few autoimmune encephalitis, such as anti-leucine rich glioma inactivated 1 (LGI1), contactin-associated protein-like 2 (CASPR2), IgLON5, and glutamic acid decarboxylase 65 (GAD65) encephalitis. However, no HLA association has been reported for NMDAR encephalitis, suggesting that in this condition, and likely in others, non-HLA loci might be involved in the pathogenesis as well. Genome-wide association studies (GWAS) are useful tools to identify variants at genomic loci that are associated with complex diseases, and in particular, to detect associations between single-nucleotide polymorphisms (SNPs) and diseases. The aim of the study is to detect genetic variants in NMDAR encephalitis and other autoimmune encephalitis.
To compare the efficacy and safety of NPB-01 in patients with autoimmune encephalitis refractory to steroid pulse therapy using steroid pulse therapy as a control.
Autoimmune encephalitis (AE) are characterized by subacute onset of memory deficits, altered mental status or psychiatric symptoms, frequently associated with seizures, inflammatory cerebrospinal fluid and in cases with prominent limbic involvement, typical magnetic resonance imaging. Several autoantibodies (Abs) may be detected in AE, although its detection is not mandatory to establish a diagnosis. These Abs mainly recognize different synaptic and cell-surface proteins in the central nervous system, and are thought to be pathogenic as they alter the normal location or function of its antigens. Paraneoplastic neurological syndromes (PNS) are immune-mediated, remote complications of cancer. The clinical presentation is highly diverse, from central nervous system disorders (limbic encephalitis, cerebellar ataxia) to peripheral neuropathies and neuromuscular junction diseases. Two different kinds of Abs are associated with PNS: a first group known as onconeural Abs, which recognize intracellular antigens and are thought not to be pathogenic; and a second one whose targeted synaptic and cell-surface antigens shared with some non-paraneoplastic AE. The primary trigger of the immune response is unknown for most of AE. In addition to acquired susceptibility such as herpes simplex encephalitis, genetic predisposition may also be important in the pathogenesis of AE. Human leukocyte antigen (HLA) is the genetic factor most frequently associated with autoimmune diseases, due to its genetic complexity and key role in the adaptive immune response. Others and we already described the HLA haplotypes associated with three types different of AE: anti-leucine-rich glioma inactivated 1 (LGI1), anti-contactin-associated protein-like 2 (CASPR2), and anti-glutamic acid decarboxylase (GAD). Nevertheless, the genetic predisposition of many other AE has not been investigated yet. Cancer is considered as the trigger of the immune response that lead to PNS development, as the neural antigens recognized by the onconeural Abs are also expressed by tumor cells. Nevertheless, it is still unknown why some patients develop PNS and others do not, even if they present the same histological type of tumor, suggesting that some particular, maybe genetic, characteristics of the patients may play a role in this susceptibility. Furthermore, there is already evidence that, for those neurological diseases that may appear either as PNS or as non-paraneoplastic autoimmune disorder (i.e. Lambert-Eaton myasthenic syndrome), HLA profiles are not the same.
Determine the difference in the modified Rankin score at 16 weeks in participants with anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis treated with "first-line" immunomodulatory therapies provided as standard-of-care, and either inebilizumab (investigational agent) or placebo.
Herpes Simplex Virus encephalitis is the most common infectious encephalitis, with an estimated annual incidence of 1 / 250,000 to 1 / 500,000 in industrialized countries. Despite a widely used antiviral treatment, the prognosis remains poor with a mortality of 5 to 20% and a considerable morbidity rate. One of the contributing factors of bad prognosis is the development of encephalitis mediated by autoantibodies, most often directed against NMDA receptors, in the weeks following viral encephalitis. The description of this pathology is recent, the pathophysiology of this process remains poorly understood, and the management of these patients is not yet codified.