Rhomboencephalitis

Abstract

Rhomboencephalitis—inflammation of the brainstem and cerebellum—has myriad clinical presentations including encephalopathy, cranial neuropathies, long tract signs and cerebellar dysfunction and is associated with significant morbidity and mortality. There are a variety of potential underlying causes that respond variably to treatment, including infections, parainfective syndromes, inflammatory disorders including autoimmune encephalitis and paraneoplastic syndromes. Here, we review its clinical presentation and outline a practical approach to its investigation, aiming to facilitate prompt diagnosis and confirmation of the underlying cause, to start appropriate management early and optimise the clinical outcome.

A Case of Fulminant Listeria Rhombencephalitis with Brainstem Abscesses in a 37-Year-Old Immunocompetent Patient: From Vestibular Neuritis to Ondine's Curse

“…This case comprises several interesting clinical findings, that add favourably to the existing literature. Firstly being, that it is very unusual for a fit healthy 37-year-old to develop such a severe LM infection, as well as the multiple unfortunate co-infections (SARS-CoV-2, Gardnerella vaginalis, Mycoplasma hominis and Staphylococcus aureus) 1,8 .…”

“…In the literature a retrograde spread of LM through the trigeminal nerve has been proposed earlier via damaged oropharyngeal mucosa or periodontal tissue. Another former study commented on the assumption of entrance of the pathogen via the vagus nerve through enteric neurons in the walls of the gastrointestinal tract 1,3,9 . Next, the radiological phenomenon of spread via white matter fibre has been reported previously, although, without involvement of cranial nerves 10 .…”

Weakness in the intensive care unit

“…Cranial nerves 3 and 4 (midbrain), 5, 6, 7 and 8 (pons) and 9, 10, 11 and 12 (medulla) potentially cause diplopia, facial or jaw weakness as well as dysarthria, dysphonia, dysphagia and tongue weakness 44 45…”

Tratamento empírico para romboencefalite por Listeria Monocytogenes

“…O exame de líquor cefalorraquidiano (LCR) pode ter alterações diversas a depender da etiologia, mas geralmente mostram sinais inflamatórios como aumento de leucócitos e/ou hiperproteinorraquia. Neoplasias, doença vasculares e reações medicamentosas estão entre os diagnósticos diferenciais[1][2][3][4] .A Listeria monocytogenes é uma bactéria ubíqua pode ser encontrada na água, no solo e em fezes, chamando-nos a atenção para a grande possibilidade de contaminação, principalmente após a industrialização da produção alimentar.Tabela 1. Principais causas de encefalite do tronco cerebral.…”