Immunoglobulin-responsive refractory epilepsy – 3 cases with a similar EEG pattern

Abstract

Autoimmune epilepsies are a heterogeneous group of recently described refractory epilepsies. They have been characterized by a variety of clinical, serological and radiological features. A range of anti-neuronal antibodies, which are usually found in limbic encephalitis, including antibodies to GAD,VGKC-complex antibodies,NMDA-R,amongst others, have been associated with some of these disorders. Other, antibody negative epilepsy patients had radiological features of central nervous system inflammation, inflammatory infiltrates histologically or high CSF white cell counts and oligoclonal bands. Immune therapy, including steroids and IVIg, has been used in these cases and resulted in clinical improvement. Best outcomes were associated with a short duration of the underlying illness. Long-term immune therapy has often been utilized in these cases.In this article, we describe a small cohort of patients who had a rapid, sustained response to a short course of IVIg, despite limited serological and radiological evidence for an underlying autoimmune process. This response was independent of the varying seizure phenotypes. Interestingly, all 3 patients shared a certain EEG pattern.