Journal of Autoimmune Disorders

Description

In the spectrum of neurological disorders characterized by the detection of neural autoantibodies in serum or spinal fluid and the responsiveness to immunotherapy, autoimmune epilepsy is becoming increasingly recognized. Seizures that are frequent or medically intractable, the presence of at least one neural antibody, inflammatory changes seen on an MRI or in serum or spinal fluid, or a personal or family history of autoimmunity all raise the possibility of an autoimmune cause. Due to electrical disturbances in the brain, epilepsy is a debilitating neurological disorder characterized by seizures, sporadic electrical storms, and cognitive impairment, frequently without a known etiology or effective treatment.. Because adjunctive immunotherapy may slow, halt, or even reverse the epileptogenic process in these patients, it is important to identify an immune basis. Since its inception, para-neoplastic limbic encephalitis, which frequently manifests as seizures, has been associated with autoantibodies.

Electroencephalogram

In particular, the role of neural autoantibodies in chronic refractory epilepsy is being investigated. Modern medicine has accepted autoimmunity as a fact. Immune epilepsy was recently added to the new ILAE classification of epilepsies as one of the etiological groups. Data suggest that drug-resistant epilepsy may have an immune-mediated pathogenesis even in the absence of limbic encephalitis or cancer. Autoimmune epilepsies are clinical syndromes in which it is thought that the immune system is responsible for the pathogenesis of seizures or for neuronal injury after seizures. The rapid onset of focal seizures, encephalopathy, cognitive decline, or other focal neurological deficits, or all of these, characterize these diseases, which typically affect otherwise healthy children. When diagnosing autoimmune epilepsies, traditional neurological diagnostics lack sensitivity and specificity, so results must be viewed in the context of a clinical setting. Because conventional antiepileptic medications are typically unable to control seizures and other neurological symptoms on their own, it is essential to take into account an autoimmune etiology as soon as possible in the clinical course to ensure the prompt initiation of immunotherapy, if necessary. The autoimmune epilepsies of autoimmune encephalitis Rasmussen's encephalitis and febrile infection-related epilepsy syndrome are the subjects of this article. To better comprehend the pathogenic mechanisms, the most effective immunotherapy, and the impact of treatment on prognosis, additional research is required. Autoimmune limbic encephalitis and multifocal para-neoplastic disorders frequently present with seizures.

In the absence of syndromic encephalitis manifestations, mounting evidence points to an autoimmune cause of seizures. Recurrent seizures are the primary and persistent clinical feature of the immunologically mediated autoimmune epilepsies. A patient with epilepsy with neural autoantibodies and/or the presence of inflammatory changes on cerebrospinal fluid or magnetic resonance imaging suggests an autoimmune etiology when other etiologies have been ruled out. Depending on the particular autoimmune epilepsy syndrome that is present, immunotherapy may be extremely effective in these patients. Several autoimmune epilepsy syndromes are discussed in this chapter. Epilepsies caused by other primary autoimmune disorders will follow. The typical clinical, imaging, electroencephalogram, CSF, and serologic characteristics, as well as the pathophysiology and treatment of each syndrome, are discussed. Without standardized treatment guidelines, autoimmune epilepsy is a condition that is under-recognized. However, as these patients may continue to be refractory to conventional anti-seizure medications, it is essential to identify an underlying autoimmune etiology for epilepsy. 17.5% of epilepsy patients had an autoimmune disorder, according to a large population-based study, and the presence of an autoimmune disorder may increase the risk of epilepsy by fourfold. In these patients, it is reasonable to have a high suspicion of autoimmune epilepsy. There are no clear guidelines for the diagnosis or treatment of autoimmune epilepsy, despite the increased interest in research in recent years. Steroids, intravenous immunoglobulin cyclophosphamide, and rituximab are among the immune-modulatory therapies that have been used to control seizures in ASM-resistant patients. Autoimmune encephalitis is known to result in epilepsy.

Epileptogenesis

Autoantibodies that target neuronal intracellular or neuronal cell surface antigens are both associated with encephalitides and epilepsy. It has been demonstrated that intra-neuronal antibodies are a sign of underlying cancer. These antibodies have been shown to cause neuronal cell death through T-cells rather than directly through antibodies, as is the case with surface antigen-related autoimmunity. One of the most prevalent primary brain disorders is epilepsy. Adult-onset epilepsy is increasingly being linked to nonparaneoplastic autoimmune encephalitis. However, a cause of the syndrome can only be determined in extremely rare instances. In a subset of patients, autoantibody detection following central nervous herpes virus infection suggests a post viral etiology. We conducted a large-scale analysis of herpes virus DNA in cerebrospinal fluid samples from patients with clinical signs of autoimmune encephalitis to investigate the possibility of under recognition of post infectious autoimmunity. Seizures and epilepsy have been linked to autoimmune disease and inflammation. The central nervous system can be affected by autoimmune disorders as a syndrome or as part of a systemic disease.

Steroids, immunoglobulin’s, and other immune-modulatory therapies are options for this autoimmune injury's treatment. Patients with systemic autoimmune diseases may benefit from targeted treatments and improved outcomes if the mechanisms underlying epileptogenesis are better understood. Epilepsy risk factors for systemic autoimmune diseases have gained notoriety. Epilepsy, on the other hand, has been linked to an increased risk of systemic autoimmune diseases, according to recent research. Epilepsy and SAD may be linked by an underlying biological mechanism, such as pro-inflammatory conditions and antibodies, based on emerging data. This connection has been the subject of numerous studies because it may help clarify the various roles that auto-Abs and inflammation play in epileptogenesis. It has also been looked into whether age is a risk factor for epilepsy in SAD patients, but the results were inconsistent.