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Child with Suspected Autoimmune Encephalitis

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Symptom-Based Approach to Pediatric Neurology

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Abstract

Autoimmune encephalitis (AIE) is one of the more common causes of pediatric-onset encephalitis and typically manifests as subacute encephalopathy with associated neurological and/or psychiatric signs and symptoms. Neurological manifestations may include new-onset seizures (often refractory to typical anti-seizure medications), movement disorders, cognitive or developmental regression, and dysautonomia. Psychiatric symptoms, including mood alterations, are more prominently noted in young adults. Cerebrospinal fluid (CSF) testing and magnetic resonance imaging (MRI) may provide evidence of an inflammatory process, but normal testing does not exclude the possibility of an AIE. As opposed to other forms of pediatric encephalitis, an AIE is uniquely responsive to treatment with immunotherapy. In cases of probable AIE, early effective treatment is recommended once important disease mimics are effectively ruled out.

This chapter will review the epidemiology, clinical presentation, evaluation, diagnosis, treatment, and prognosis of pediatric patients diagnosed with an AIE. We will focus on the most common AIEs in children: anti-N-methyl-D-aspartate (NMDA)-receptor encephalitis and acute disseminated encephalomyelitis (ADEM).

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Authors and Affiliations

  1. Department of Neurology, Division of Pediatric Neurology, University of Virginia, VA, USA

    Jenny Joseph & J. Nicholas Brenton

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  1. Jenny Joseph
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  2. J. Nicholas Brenton
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Correspondence to J. Nicholas Brenton .

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Editors and Affiliations

  1. Department of Pediatrics, University of Texas Health Science Center, San Antonio, TX, USA

    Deepak M. Kamat

  2. Pediatrics and Neurology, Central Michigan University Children’s Hospital of Michigan, Detroit, MI, USA

    Lalitha Sivaswamy

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Joseph, J., Brenton, J.N. (2022). Child with Suspected Autoimmune Encephalitis. In: Kamat, D.M., Sivaswamy, L. (eds) Symptom-Based Approach to Pediatric Neurology . Springer, Cham. https://doi.org/10.1007/978-3-031-10494-7_32

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  • DOI: https://doi.org/10.1007/978-3-031-10494-7_32

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