Differential Diagnosis of a Patient With Rhomboencephalitis

Rhomboencephalitis is a rare and severe infection that frequently presents as progressive brainstem dysfunction. Most cases reported in the medical literature are associated with a positive and specific microbial etiology that is clearly documented by blood, cerebrospinal fluid (CSF), or brain tissue culture. However, some patients with rhomboencephalitis have a negative culture workup, which can lead to a delay in treatment initiation as a differential diagnosis is made. Because a high mortality rate has been associated with a delay in antibiotic therapy for patients with rhomboencephalitis, an improved index of suspicion is needed for early diagnosis. Recently, the authors cared for a patient with a culture-negative rhomboencephalitis that was successfully treated with empiric antibiotic management. The patient was a 36-year-old woman with symptoms of fever, nausea, and dysphagia. Imaging revealed a right-sided intra-axial contrast-enhancing lesion at the pontomedullary junction, but an exhaustive laboratory and imaging workup failed to reveal a definite etiology. The patient was then given a 6-week course of meropenem, linezolid, and acyclovir to cover possible pathogens, and she ultimately made a complete neurological recovery.