Autoimmune and inflammatory epilepsies

• Published in: Epilepsia (Copenhagen) Vol. 53; no. s4; pp. 58 - 62
• Main Author: Nabbout, Rima
• Format: Journal Article Conference Proceeding
• Language: English
• Published: Oxford, UK Blackwell Publishing Ltd 01.09.2012; Wiley-Blackwell; Wiley Subscription Services, Inc
• Subjects: Adult; Anti-Inflammatory Agents - therapeutic use; Anticonvulsants. Antiepileptics. Antiparkinson agents; Autoantibodies - immunology; Autoimmune diseases; Autoimmune Diseases - complications; Autoimmune Diseases - drug therapy; Biological and medical sciences; Child; Encephalitis - complications; Encephalitis - drug therapy; Epilepsy; Epilepsy - drug therapy; Epilepsy - etiology; Epileptic encephalopathies; FIRES; Headache. Facial pains. Syncopes. Epilepsia. Intracranial hypertension. Brain oedema. Cerebral palsy; Humans; Idiopathic hemiconvulsion-hemiplegia syndrome; Immunologic Factors - therapeutic use; Medical sciences; N-Methyl-d-aspartate antibodies; Nervous system (semeiology, syndromes); Neurology; Neuropharmacology; Pharmacology. Drug treatments; Receptors, N-Methyl-D-Aspartate - immunology; Steroids; Voltage-gated potassium channel antibodies; Autoimmunity; Steroid; FIRES; Nervous system diseases; Motor system disorder; Epilepsy; Idiopathic; Inorganic element; N-Methyl-D-aspartate antibodies; Cerebral disorder; Idiopathic hemiconvulsion-hemiplegia syndrome; Voltage-gated potassium channel antibodies; Encephalopathy; Central nervous system disease; Voltage; NMDA; Hemiplegia; Epileptic encephalopathies; Neurological disorder; Potassium; Steroids
• ISSN: 0013-9580; 1528-1167
• DOI: 10.1111/j.1528-1167.2012.03614.x

Summary The role of immunity and inflammation in epilepsy have long been suggested by the anticonvulsant activity of steroids in some infancy and childhood epilepsies. The role of fever and infection in exacerbating seizures due to possible proinflammatory molecules, the increased frequency of seizures in systemic autoimmune diseases like systemic lupus erythematous, and, recently, the detection of autoantibodies in some unexplained epilepsies reinforced the causal place of immunity and inflammation in epilepsies with unknown etiology. In this article, we summarize epilepsies where clinical and biologic data strongly support the pathogenic role of autoantibodies (e.g., limbic encephalitides, N‐methyl‐d‐aspartate [NMDA] encephalitis) and epilepsies where immune‐mediated inflammation occurs, but the full pathogenic cascade is either not clear (e.g., Rasmussen’s encephalitis) or only strongly hypothesized (idiopathic hemiconvulsion–hemiplegia syndrome [IHHS] and fever‐induced refractory epilepsy in school‐aged children [FIRES]). We emphasize the electroclinical features that would help to diagnose these conditions, allowing early immunomodulating therapy. Finally, we raise some questions that remain unclear regarding diagnosis, mechanisms, and future therapies.