PEDIATRIC AND HERITABLE DISORDERS: Edited by Polly J. FergusonPediatric inflammatory brain diseases a diagnostic approachVan Mater, HeatherAuthor Information Division of Pediatric Rheumatology, Department of Pediatrics, Duke University, Durham, North Carolina, USA Correspondence to Heather Van Mater, Division of Pediatric Rheumatology, Duke University Medical Center, 2301 Erwin Road, DUMC Box 3212, Durham, NC 27710, USA. Tel: +1 919 684 6575; fax: +919 684 6616; e-mail: [email protected] Current Opinion in Rheumatology: September 2014 - Volume 26 - Issue 5 - p 553-561 doi: 10.1097/BOR.0000000000000092 Buy Metrics Abstract Purpose of review Autoimmune-mediated inflammatory brain diseases represent a rapidly evolving area of medicine. The expanding spectrum of disease challenges providers as they strive to accurately diagnosis and treat children presenting with new onset neuropsychiatric deficits. This review examines recently published studies on primary central nervous system vasculitis and autoimmune encephalitis and utilizes key distinguishing features to guide a diagnostic approach. Recent findings The spectrum of inflammatory brain disease continues to expand with the recognition of variable disease phenotypes and targets of the immune system. Providers are often overwhelmed by the heterogeneity in clinical features and the lack of sensitivity and specificity of diagnostic testing. Recent scientific advances have enhanced our ability to diagnose these conditions and provide great promise for successful treatment in even severely affected children. Summary We are beginning to recognize the brain's susceptibility to autoimmunity and the broad spectrum of phenotypes associated with these conditions. Differentiating the various types of inflammatory brain disease remains challenging and benefits from a systematic approach. © 2014 Lippincott Williams & Wilkins, Inc.