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Pediatric autoimmune encephalitis: Recognition and diagnosis

Marienke A A M de Bruijn1, Arlette L Bruijstens1, Anna E M Bastiaansen1

  • 1From the Department of Neurology (M.A.A.M.d.B., A.L.B., A.E.M.B., A.v.S., P.A.E.S.S., M.J.T.), Department of Immunology (M.W.J.S.), and Department of Pediatric Neurology (R.F.N.), Erasmus MC University Medical Center, Rotterdam; Haga Hospital (A.v.S.), The Hague; and Sophia Children's Hospital (R.F.N.), Rotterdam, the Netherlands. A.v.S. is currently working at Medisch Centrum Haaglanden, The Hague.

Neurology(R) Neuroimmunology & Neuroinflammation
|February 13, 2020
PubMed

Insights

Autoimmune encephalitis (AIE) and acute disseminated encephalomyelitis (ADEM) are rare in children, with incidence rates of 1.54 and 2.49 per million, respectively. Critical review and specialized consultation are vital for diagnosing pediatric autoimmune neurological disorders.

Area of Science:

  • Pediatric Neurology
  • Neuroimmunology
  • Epidemiology

Background:

  • Autoimmune encephalitis (AIE) and acute disseminated encephalomyelitis (ADEM) are inflammatory neurological disorders affecting children.
  • Accurate diagnosis is crucial for timely and effective treatment, yet diagnostic challenges persist in pediatric autoimmune neurological disorders.
  • Understanding the incidence and diagnostic pitfalls is essential for improving patient outcomes.

Purpose of the Study:

  • To determine the incidence of AIE and ADEM in a pediatric population.
  • To validate existing clinical criteria for diagnosing AIE in children.
  • To identify diagnostic challenges and pitfalls in pediatric autoimmune and inflammatory neurological disorders.

Main Methods:

  • Retrospective cohort study of 113 children with suspected autoimmune or inflammatory neurological disorders.
  • Inclusion of three patient categories: antibody-mediated AIE, ADEM, and suspected autoimmune etiology.
  • Validation of AIE diagnostic guidelines using baseline and follow-up clinical data; review of patient files and final diagnoses.

Main Results:

  • The incidence of antibody-mediated AIE was 1.54/million and ADEM was 2.49/million children.
  • The current AIE diagnostic guideline proved useful in the pediatric cohort.
  • Diagnostic errors occurred, with 22% of initially suspected autoimmune/inflammatory cases lacking supporting evidence upon review.

Conclusions:

  • Antibody-mediated AIE and ADEM are the most common autoimmune/inflammatory encephalitides in children; other forms are rare.
  • Existing diagnostic criteria for AIE are applicable to pediatric cases.
  • Careful data review, comprehensive workup, and consultation with specialized centers are recommended for children with nonspecific neurological symptoms suggestive of autoimmune or inflammatory conditions.
Abstract

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