Rhombencephalitis: pictorial essay

Abstract

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.

Neuromyelitis Optica Spectrum Disorders: Spectrum of MR Imaging Findings and Their Differential Diagnosis

“…It has a wide diversity of causes, such as infectious, paraneoplastic, and autoimmune disorders. The most common infectious agent of infectious rhombencephalitis is Listeria monocytogenes, followed by enterovirus 71 and the herpes family viruses (78). Listeria rhombencephalitis occurs in healthy adults with a typical biphasic presentation that may affect even the area postrema (Fig 17).…”

“…Listeria rhombencephalitis occurs in healthy adults with a typical biphasic presentation that may affect even the area postrema (Fig 17). The MR imaging appearance of Listeria rhombencephalitis may be distinguishable from that of NMOSD when abscess formation or intra-axial trigeminal nerve involvement is observed (78,79).…”

Two cases of listeria rhombencephalitis

“…Indeed, gadolinium-enhanced MRI usually shows abnormalities which aid in diagnosis [3] , [4] , [13] and should be performed in patients with brainstem or cerebellar signs and a non-diagnostic CT scan. Characteristic MRI changes include T2 and FLAIR hyperintensity in the brainstem and cerebellum [17] , which was seen in the second case, and ring enhancing lesions, principally in LRE [1] , [13] , which were seen in the first case. Computed tomography is less useful but may show hypodense areas in the brainstem [3] , [7] .…”

Neuromyelitis optica spectrum disorder presenting as rhomboencephalitis

“…In most paraneoplastic cases, small cell lung cancer is the underlying malignancy. Isolated cases of systemic lupus erythematosus causing rhomboencephalitis have also been reported 1. NMOSD causing rhombocephalitis is uncommon.…”