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Brainstem encephalitis: etiologies, treatment, and predictors of outcome

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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.

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Acknowledgments

Financial disclosure: Ellen M. Mowry: Funding NIH K23NS067055; National MS Society RG4407A2. Receipt of free study medication from Teva Neurological. Justin C. McArthur: Grants from NIH, Biogen-Idec, payment from lectures, speakers’ bureau in various universities, book royalties, stock option from Gliamed. Avindra Nath: Consultant to Biogen Idec and Diogenix. Arun Venkatesan: Grants from NIH, HHMI, Maryland Stem Cell Research Foundation, National Multiple Sclerosis Society. Ik Lin Tan, Sonya Steele, Carlos Pardo-Villamizar report no financial disclosure.

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On behalf of all authors, the corresponding author states that there is no conflict of interest.

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Correspondence to Arun Venkatesan.

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Tan, I.L., Mowry, E.M., Steele, S.U. et al. Brainstem encephalitis: etiologies, treatment, and predictors of outcome. J Neurol 260, 2312–2319 (2013). https://doi.org/10.1007/s00415-013-6986-z

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  • DOI: https://doi.org/10.1007/s00415-013-6986-z

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