Pediatric inflammatory brain diseases: a diagnostic approach

Curr Opin Rheumatol. 2014 Sep;26(5):553-61. doi: 10.1097/BOR.0000000000000092.


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.

Publication types

  • Review

MeSH terms

  • Anti-N-Methyl-D-Aspartate Receptor Encephalitis / diagnosis
  • Autoimmune Diseases of the Nervous System / diagnosis*
  • Cerebral Angiography
  • Child
  • Diagnosis, Differential
  • Encephalitis / diagnosis*
  • Encephalomyelitis, Acute Disseminated / diagnosis
  • Humans
  • Limbic Encephalitis / diagnosis
  • Neuromyelitis Optica / diagnosis
  • Vasculitis, Central Nervous System / diagnosis