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J Neurol (2013) 260:23122319 DOI 10.1007/s00415-013-6986-z
ORIGINAL COMMUNICATION
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
Received: 4 March 2013 / Revised: 22 May 2013 / Accepted: 28 May 2013 / Published online: 9 June 2013 Springer-Verlag Berlin Heidelberg 2013
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 nonHIV infected patients diagnosed with BE at Johns Hopkins Hospital (January 1997April 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 identied in 58 of 81 (71.6 %) of cases, most of which were conrmed or probable inammatory/autoimmune conditions. Of the remaining 23 cases in which a specic diagnosis remained undened, clinical presentation, CSF, neuroimaging studies, and outcomes were similar to the inammatory/auto-immune group. Brain biopsy identied a specic 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: 1.031.20), a higher CSF glucose (per 5 mg/dl, OR = 1.36, 95 % CI: 1.091.70), and higher serum glucose (per 5 mg/dl, OR = 1.27, 95 % CI:1.061.52) were independently associated with increased odds of poor outcome. Inammatory 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 undened 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 Inammatory Glucose
Introduction
Encephalitis affects 1 in 10,000 individuals yearly, often with devastating neurologic consequences [1]. Challenges in the management of patients with encephalitis include identifying the causative agent and dening prognosis. In several large studies, infectious agents comprise the majority of cases in which a cause is identied. Little, however, is known about the typical etiologies of brainstem encephalitis (BE). Several entities, including Listeria rhombencephalitis, Bickerstaffs brainstem...