Journal of Neurology

, Volume 260, Issue 9, pp 2312–2319

Brainstem encephalitis: etiologies, treatment, and predictors of outcome

  • Ik Lin Tan
  • Ellen M. Mowry
  • Sonya U. Steele
  • Carlos A. Pardo
  • Justin C. McArthur
  • Avindra Nath
  • Arun Venkatesan
Original Communication

Abstract

Brainstem encephalitis (BE) is an uncommon condition. We sought to characterize clinical presentations, etiologies, response to treatment, and predictors of outcome. We performed a retrospective review of non–HIV infected patients diagnosed with BE at Johns Hopkins Hospital (January 1997–April 2010). We characterized clinical and paraclinical features, and used regression models to assess associations with poor outcome. BE was diagnosed in 81 patients. An etiology was identified in 58 of 81 (71.6 %) of cases, most of which were confirmed or probable inflammatory/autoimmune conditions. Of the remaining 23 cases in which a specific diagnosis remained undefined, clinical presentation, CSF, neuroimaging studies, and outcomes were similar to the inflammatory/autoimmune group. Brain biopsy identified a specific diagnosis in 7 of 14 patients (50 %). Fifteen patients (18.5 %) either died or had a poor outcome. In multivariate logistic regression models, a higher CSF protein (per 5 mg/dl, OR = 1.11, 95 % CI: 1.03–1.20), a higher CSF glucose (per 5 mg/dl, OR = 1.36, 95 % CI: 1.09–1.70), and higher serum glucose (per 5 mg/dl, OR = 1.27, 95 % CI: 1.06–1.52) were independently associated with increased odds of poor outcome. Inflammatory and non-infectious conditions accounted for most cases of BE. Higher CSF protein and glucose were independently associated with poor outcome. In immunocompetent patients with BE of undefined etiology despite extensive investigation, a trial of immunosuppressive treatment may be warranted, though deterioration clinically or on magnetic resonance imaging should prompt a brain biopsy.

Keywords

Rhombencephalitis Brainstem encephalitis Brain biopsy Autoimmune Inflammatory Glucose 

Supplementary material

415_2013_6986_MOESM1_ESM.docx (13 kb)
Supplementary material 1 (DOCX 13 kb)

Copyright information

© Springer-Verlag Berlin Heidelberg 2013

Authors and Affiliations

  • Ik Lin Tan
    • 1
    • 2
  • Ellen M. Mowry
    • 2
  • Sonya U. Steele
    • 2
  • Carlos A. Pardo
    • 1
    • 2
  • Justin C. McArthur
    • 1
    • 2
  • Avindra Nath
    • 3
  • Arun Venkatesan
    • 1
    • 2
    • 4
  1. 1.Johns Hopkins Encephalitis CenterJohns Hopkins University School of MedicineBaltimoreUSA
  2. 2.Department of NeurologyJohns Hopkins University School of MedicineBaltimoreUSA
  3. 3.Section of Infections of the Nervous System, National Institute of Neurological Disorders and StrokeNational Institutes of HealthBethesdaUSA
  4. 4.Johns Hopkins Neuroimmunology and Neuroinfectious DiseasesBaltimoreUSA

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