- Dr. Mercè Falip, MD
- Epilepy Unit, Hospital Universitari de Bellvitge, Spain.
- Dr. Mar Carreño, MD, PhD
- Epilepsy Unit, Hospital Clinic, Barcelona, Spain.
Special Issue Introduction
As is defined by the International League Against Epilepsy (ILAE), epilepsy is considered a chronic disease of the brain. Several brain insults can induce epileptogenesis. Epileptogenesis is the process by which a brain network that was previously normal is functionally altered toward increased seizure susceptibility, thus having an enhanced probability to generate spontaneous recurrent seizures. The occurrence of epileptogenesis after acquired brain insults in humans has been most extensively studied after stroke, traumatic brain injury and status epilepticus.
It is estimated that epilepsy of unknown aetiology constitutes one-third of all epilepsies in adults. A subgroup of these patients may have immune-mediated aetiology. There is nowadays little information about the rate of epileptogenesis after an autoimmune (limbic or non limbic) encephalitis (AE). However, it is well known that the risk of epilepsy is higher for disorders in which the antigens are intracellular (often T cell-mediated) compared with disorders in which the antigens are on the cell surface (antibody-mediated).
On the other hand, evidence exists now for a two-way relationship between inflammation and epilepsy, where inflammation may be the cause and/or a consequence of epilepsy. Inflammation appears to have a mixed role in immune/inflammatory reactions in the central nervous system (CNS), in some cases offering protection, and in some deleterious effects. In deleterious instances, neuronal dysfunction or damage in some diseases appears to be the direct result of inflammatory reactions caused by antibodies directed at targets within the CNS and intracellular or surface antigens in different types of encephalitis. Some patients with systemic lupus erythematosus (SLE) or primary antiphospolipid antibodies or other systemic autoimmune diseases develop acute symptomatic seizures (ASS) in the context of acute inflammation and epilepsy over time. It has been suggested that some systemic autoantibodies (anti-DNA, anti-cardiolipin and anti-La) can cross-react with some receptors (NMDA receptor and GABA or GABA receptors) blocking their normal function. However, as mentioned above, inflammation may also have a beneficial effect on CNS diseases. Further, inflammation may promote the infiltration of neuroprotective lymphocytes and macrophages into the epileptic region. The net effect of inflammatory processes may be determined by the nature of cytokines released and duration of inflammation.
For this special issue of the journal, we request submission of manuscripts concerning clinical, immunological, electrophysiological and neuroimaging characteristics of patients with ASS in the context of systemic autoimmune disease or patients who suffered an AE. Studies concerning epileptogenic mechanism and biomarkers in these patients are also welcomed.
Submissions especially (but not exclusively) on the following topics are welcomed:
Long-term evolution of patients suffering an AE with ASS and incidence of epilepsy after suffering an AE;
Biomarkers (serologic, neurophysiologic or in neuroimage) of epileptogenesis in patients with AE or with seizures in the context of a systemic autoimmune diseases;
Treatment response in patients with AE and seizures in the context of a systemic autoimmune encephalitis specially focusing in epileptogenesis;
Treatment response in patients with drug resistant autoimmune epilepsy (Epilepsy +GAD ab, Epilepsy in patient with SLE…).
KeywordsLimbic encephalitis, autoimmune encephalitis, systemic autoimmune disease, epilepsy, acute symptomatic seizure, neuronal antibodies, systemic autoautibodies
Submission Deadline31 Mar 2020