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.

Clinical spectrum and outcomes of patients with encephalitis requiring intensive care

“…High CSF protein levels were identified to be independently associated with outcome, as suggested in two previous studies on brainstem encephalitis and West Nile encephalitis . Elevated CSF protein levels on admission may indeed reflect a more severe inflammatory reaction, which may in turn translate into greater CNS dysfunction and brain injury.…”

Paediatric brainstem encephalitis associated with glial and neuronal autoantibodies

“…Several entities including infectious, autoinflammatory, and paraneoplastic syndromes have been reported. A cross‐sectional study at a single hospital in the USA identified inflammatory/autoimmune aetiology in 54 (67%) of 81 adult patients with brainstem encephalitis, but these patients were not tested for any specific antibodies (Ab) . However, it is clear that antibodies can diffuse into the area postrema, which lacks a blood brain barrier, as in neuromyelitis optica, and other brainstem regions may also be permeable, as inferred recently from rodent studies .…”

A case of brainstem encephalitis treated with plasmapheresis

“…Biopsies were made of the brain stem, cerebellum, cerebellum, and thalamus. Only one patient developed dysarthria after brain biopsy, and no mortality associated with brain biopsy was reported [2]. We did not perform brain biopsy in our case.…”

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Brain stem encephalitis is a rare disease, which can be triggered by infection, with an unclear etiology, in which infectious and autoimmune mechanisms are thought to play a role in the pathogenesis [1]. Major clinical symptoms are ataxia, muscle weakness and ocular and bulbar dysfunction [2]. Its etiology can vary from infection to autoimmune reasons.

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“…It may also be caused by neurobehcets, Bickerstaff's encephalitis, autoimmune diseases such as multiple sclerosis and paraneoplastic reasons [3][4][5]. With those in mind, the underlying etiology is unclear in most cases [2]. Treatment is based on etiology.…”