Induction of Central Nervous System Disease by the Adaptive Immune Response

Over the last years it has become clear that many neurological diseases of the central nervous system (CNS) are induced by an adaptive immune response directed against molecules expressed on CNS-resident cells. Prototypic examples are anti- N-methyl-D-aspartate receptor (NMDAR) encephalitis which is induced by an immune response against the NMDAR expressed on neurons or neuromyelitis optica (NMO) in which the disease is induced by antibodies directed against aquaporin-4 expressed on astrocytes. There are many more examples in which it has become clear that a specific adaptive immune response mediated by T or/and B cells is leading to CNS disease. Often the symptoms of the induced disease are not easily interpreted as caused by an immune mediated disease. Beside classical neurological symptoms like ataxia, vision disturbance and motor or sensory symptoms, these can include cognitive disturbances, behavioral abnormalities or/and epileptic seizures. Although much has been learned regarding the pathophysiology of prototypic examples of this group of disorders, there are still major holes in the understanding of disease biology. This may be due to the fact that these disorders are rare diseases, and the treatment approaches are still very limited. The research topic includes contributions addressing the target molecules of this group of disorders, the molecular mapping of the relevant epitopes, the analysis of the adaptive immune response (T cell, B cells, antibodies) driving disease, the effector mechanisms such as complement activation cascades, genetic and genomic regulation, as well as environmental triggers. The research topic will also include contributions addressing diagnostic criteria and methods as well as therapeutic approaches. The ultimate goal of the research topic is an improved pathophysiological understanding of immune mediated CNS disorders, and better diagnostic criteria and treatment approaches.