Rhombencephalitis is an umbrella word for a group of inflammatory illnesses that mostly affect the base of the brain (cerebellum and brainstem). Rhombencephalitis has a wide range of causes, some of which are serious and even fatal if not treated promptly. The medulla, cerebellum, and pons make up the hindbrain. Furthermore, the words “brainstem encephalitis” and “rhombencephalitis” are frequently used interchangeably. Infections, inflammatory disorders, and paraneoplastic syndromes are all potential causes of brainstem encephalitis.
Listeria monocytogenes is an important bacterium that causes illness in a wide variety of people, including babies, those with impaired immune systems, pregnant women, the elderly, and less frequently healthy people. Contrary to the invasive form of the illness, Listeria-associated gastroenteritis has a significantly shorter incubation period. Listeria rhombencephalitis, often known as LRE, is an extremely uncommon form of encephalitis that affects the hindbrain and can cause a wide range of neurological symptoms. Prompt antibiotic treatment is needed to prevent excessive mortality and morbidity.
The many causes of rhombencephalitis lead to a wide range of symptoms. Headache, nausea/vomiting, fever, and unexplained neurological symptoms are classic signs of a viral infection. As a result, patients exhibit a wide variety of brainstem symptoms, including cranial nerve palsies, impairments in cerebellar function, and motor and sensory deficiencies further down the spinal cord. Rhomboencephalitis, also known as inflammation of the brainstem and cerebellum, can manifest in a variety of ways in a patient’s body, including, cranial neuropathies, encephalopathy, long tract symptoms, and cerebellar dysfunction.
Rhombencephalitis is a syndrome that describes inflammatory illnesses of the hindbrain. In medical terms, the midbrain, also known as the mesencephalon, is not considered to be a member of the rhombencephalon; yet, the terms RE and brainstem encephalitis are sometimes used interchangeably.
Three groups of etiologies for RE mostly exist such as autoimmune, infectious, and paraneoplastic diseases. The three most frequent causes of infectious RE are listeria and herpes viruses. The most frequent autoimmune etiology of RE is Behçet disease (BD), while relapsing polychondritis and systemic lupus erythematosus are also implicated in a few isolated cases.
The most frequent source of contagious RE is listeria. Listeria RE mostly affects healthy young adults. It typically has a biphasic course, beginning with a flu-like symptom and progressing to brainstem dysfunction. Ampicillin is the primary treatment in this situation. Enterovirus 71 is the second most prevalent cause of RE, and there is no treatment. The third most typical viral factor causing RE is the herpes simplex virus (HSV).
The most prevalent cause of autoimmunity is Behcet’s illness. Only 25% of patients who receive corticosteroids and immunosuppressive medications mostly recover fully. The third type of RE is brought on by paraneoplastic conditions. MRIs of the brain are often normal; there is typically a pleocytosis in CSF, but the protein levels are typically normal in this case. Antibodies against neurons are typically present. The prognosis is poor and treatment is marginally helpful.
The clinicians generally suggest initial therapy with acyclovir and ampicillin in cases of Listeria infectious causes. MRI findings, culture results, PCR findings, and antibody study findings are frequently taken into consideration while making adjustments to antibiotic treatment.