Neuroimmune disorders of the central nervous system in children in the molecular era
Immune mediated disorders of the CNS in children are a complex group of demyelinating, inflammatory, parainfectious and postinfectious disorders with heterogeneous pathobiological mechanisms and clinical manifestations, often associated with fundamental derangement in immune regulation. In this Review, we aim to provide an update on our knowledge of neuroimmune disorders and highlight areas of research that are priorities for improving clinical management. We outline the clinical features of neuroimmune disorders, the current approaches to their treatment and new approaches in development. We then consider the pathological features, including biomarkers, pathological mechanisms and genetics, and discuss the value of immune assays in clinical investigation and basic research. On the basis of current knowledge and techniques, we propose four research priorities: rigorous and consistent collection of core clinical data, cooperative investigation of treatments, development of biological assays and genetic studies. These priorities should help us to achieve the shared goal of precision medicine for neuroimmune disorders. However, multicentre research and the creation of clinical consortia for these rare disorders will be necessary, and we hope that this Review serves as a call to action that is timely given current exciting advances in neuroimmune therapeutics.In this Review, the authors discuss all aspects of immune mediated disorders of the CNS in children, from the clinical features and treatment to pathological mechanisms and biomarkers, and outline priorities for collaborative research to develop precision medicine for these disorders.Key pointsNeuroimmune disorders are more prevalent than previously appreciated.Advances in assay development, genetic research, neuroimaging and clinical phenotyping have led to better characterization of the different neuroimmune disorders.Immune signatures have not been identified for all neuroimmune disorders; diagnosis in patients with these disorders relies on the clinical features of acquired neuroinflammation.Prompt diagnosis and immunomodulatory therapy have improved clinical outcomes from neuroimmune disorders, but formal consensus based treatment protocols or protocols based on evidence from clinical trials are yet to be established.Collaborative research into the pathobiological mechanisms of neuroimmune disorders will further refine relevant treatment strategies, including targeting of key biological pathways.
Nature Reviews Neurology