title: Brainstem encephalitis: etiologies, treatment, and predictors of outcome.
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.
authors.affiliation: Johns Hopkins Encephalitis Center, Johns Hopkins University School of Medicine, Baltimore, MD 21287, USA.
authors.forename: Ik Lin
authors.initials: IL
authors.lastname: Tan
authors.forename: Ellen M
authors.initials: EM
authors.lastname: Mowry
authors.forename: Sonya U
authors.initials: SU
authors.lastname: Steele
authors.forename: Carlos A
authors.initials: CA
authors.lastname: Pardo
authors.forename: Justin C
authors.initials: JC
authors.lastname: McArthur
authors.forename: Avindra
authors.initials: A
authors.lastname: Nath
authors.forename: Arun
authors.initials: A
authors.lastname: Venkatesan
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D001933:Brain Stem / Q000473:pathology*; D002648:Child; D002675:Child, Preschool; D004660:Encephalitis / Q000175:diagnosis / Q000209:etiology / Q000628:therapy; D005260:Female; D006801:Humans; D007223:Infant; D008297:Male; D008875:Middle Aged; D012189:Retrospective Studies; D016896:Treatment Outcome; D055815:Young Adult