Journal of Autoimmune Disorders

Description

Although many neurologists are familiar with the clinical signs and symptoms of limbic encephalitides or anti-N-methyl-D-aspartate receptor epilepsy, the autoimmune causes of sub-acute and chronic epilepsies remain poorly understood. Additionally, the pediatric population's subtleties and differences in presentation limit diagnosis and present a challenge to clinicians. Due to the uncertainty surrounding the selection of suitable candidates for testing and immunomodulation, it is likely that many clinicians do not test for autoimmune disorders in the absence of an acute encephalitic picture. The definition of epilepsy in relation to autoimmune mechanisms has been expanded as a result of recent developments. To the best of our knowledge, autoimmune epilepsy is a subset of autoimmune encephalitis in which epilepsy and seizures are the primary presenting symptoms. Drug-resistant epilepsies are now increasingly being linked to autoimmune epilepsy. However, it is still difficult to identify affected individuals, particularly in the pediatric population. As more people with epilepsy are tested for antibodies to neuronal proteins and more antibodies are discovered, our knowledge of autoimmune epilepsy continues to grow.  

Drug-Resistant Epilepsies

The clinical features that are most frequently associated with positive antibody testing in epilepsy are discussed in this article, as are the scales that are currently available to screen patients for antibody testing and immunotherapy response. Numerous neuronal antibodies, including those that are anti-NMDAR, anti-AMPAR, anti-GABA-AR, anti-GABA-BR, and so on, were found to be linked to AE-related epilepsy and autoimmune encephalitis. There are still gaps in epidemiological evidence regarding the development of AE to epilepsy, the combination of AE and seizures, and the positive rate of antibodies in epileptic patients. Early health care management, therapeutic strategies and decisions, and prognosis prediction all benefit from an understanding of the epidemiology of AE and AE-related epilepsy. Sarcoidosis, systemic lupus erythematosus, and other multisystem inflammatory diseases, as well as para-neoplastic neurologic disorders, acute disseminated encephalomyelitis, Rasmussen encephalitis, paratyphoid neuropathy, and autoimmune encephalitis all have epilepsy as one of the major clinical manifestations. Seizures are more common in people with autoimmune diseases than in the general population. CSF protein and lactate are two parameters that frequently change after seizures but are not specific for the etiology of seizures. Although rare, pleocytosis and CSF-specific oligoclonal bands should be considered indicators of infectious or immune-mediated epilepsy. If autoimmune etiology is suspected in new-onset seizures or status epilepticus, CSF analysis is especially important.

There are currently no clinically useful biomarkers in CSF that can be used to evaluate neuronal damage or refractory epilepsy, but this is an important area of research for the future. EEG and neuroimaging tests are routinely performed on epileptic patients who have just had a seizure or epilepsy that has just been diagnosed. First, these help determine whether an epilepsy diagnosis can be made. Second, they aid in the classification and etiology of epilepsy. However, test results may not be conclusive, and clinical conditions may necessitate additional laboratory tests, such as CSF analysis. The purpose of this review is to find out how seizures affect CSF results and when CSF analysis can be used to diagnose seizures and epilepsy. In the beginning, changes to the CSF routine and additional parameters in seizure patients are discussed. Second, various clinical settings for CSF analysis are investigated. The differential diagnosis of epilepsy and autoimmune-associated seizures is then given special attention. The recognition of seizures during autoimmune encephalitis is growing.

Multisystem Inflammatory Diseases

It has been reported that standard antiepileptic medication treatment for these seizures does not work well. Additionally, refractory SE and status epilepticus typically appear during the acute phase of AIE. Indeed, new-onset RSE is a diagnostic for AIE and a strong early predictor of AIE. Epilepsy is one of the most common chronic brain disorders, affecting 50 million people worldwide. Antiepileptic medications come in a variety of strengths and can be combined due to their distinct mechanisms of action. However, one third of patients do not stop having seizures. Despite the growing recognition of chronic autoimmune epilepsy, little is known about its clinical and electrographic features. We present a patient undergoing diagnostic stereo-electroencephalography who is now seizure-free thanks to immunotherapy and was found to have multifocal perisylvian epilepsy with distinctive electrographic features. The patient's GluR3B antibodies target glutamate's central AMPA receptor, the brain's primary excitatory neurotransmitter. The patient's GluR3B peptide antibodies bind to neural and T cells, cause ROS production, and rapidly kill them. Multiple neurological impairments, onchocerca volvulus malnutrition, war-induced trauma, and other insults are all associated with Nodding Syndrome fatal pediatric epilepsy of unknown etiology.

A group of syndromes known as autoimmune encephalitis includes subacute onsets of amnesia, confusion, and frequently prominent seizures. The range of autoimmune encephalitis is getting wider. Antivoltage-gated potassium channel antibodies have been linked to the onset of a new, unexplained seizure disorder in patients with autoimmune limbic encephalitis symptoms in recent retrospective studies. Anti-VGKC-complex antibodies have low titers in the sera of some patients with isolated seizure syndromes, such as drug-resistant epilepsy and temporal lobe epilepsy. Because adjunctive immunotherapy may improve these patients' clinical conditions it is essential to identify an immune basis. Seizures are a hallmark of epilepsy, a debilitating neurological disorder. Although structural, metabolic, genetic, or infectious factors are frequently identified as causes of epilepsy, the etiology of a significant number of patients is unknown. Emerging data have revealed an autoimmune cause in patients with previous epilepsy of unknown etiology. The diagnosis of autoimmune encephalitis, which frequently manifests as seizures, relies on the detection of autoantibodies that target neural proteins.