The term rhombencephalitis refers to inflammatory diseases affecting the
hindbrain (brainstem and cerebellum). Rhombencephalitis has a wide variety of
etiologies, including infections, autoimmune diseases, and paraneoplastic
syndromes. Infection with bacteria of the genus Listeria is the
most common cause of rhombencephalitis. Primary rhombencephalitis caused by
infection with Listeria spp. occurs in healthy young adults. It
usually has a biphasic time course with a flu-like syndrome, followed by
brainstem dysfunction; 75% of patients have cerebrospinal fluid pleocytosis, and
nearly 100% have an abnormal brain magnetic resonance imaging scan. However,
other possible causes of rhombencephalitis must be borne in mind. In addition to
the clinical aspects, the patterns seen in magnetic resonance imaging can be
helpful in defining the possible cause. Some of the reported causes of
rhombencephalitis are potentially severe and life threatening; therefore, an
accurate initial diagnostic approach is important to establishing a proper early
treatment regimen. This pictorial essay reviews the various causes of
rhombencephalitis and the corresponding magnetic resonance imaging findings, by
describing illustrative confirmed cases.