Listeria rhombencephalitis

Listeria rhombencephalitis is a particular form of listerial encephalitis that affects primarily the hindbrain (brainstem and cerebellum). Listeria monocytogenes is cited as the most common etiology for rhombencephalitis, typically seen in the elderly, and resulting in significant morbidity and mortality. 

Please refer to the article on rhombencephalitis for a general discussion of that entity.

The majority of listerial rhombencephalitis reported cases occurred in previously healthy adults 2,3. In contrast listerial cerebritis is rare and encountered mostly in the setting of profound immune compromise 4

Encephalitis caused by Listeria are often misdiagnosed initially due a nonspecific prodromal (fever, headache, nausea and vomiting) and unusual meningeal signs 1-3.

Patients with rhombencephalitis develop lately a spectrum of brainstem symptomatology that could show multiple cranial nerve palsies (it occurs in ~75% of all cases), cerebellar and long-tract motor and sensory deficits. Symptoms such as ataxia, nystagmus, vertigo, facial numbness, dysphagia, persistent hiccuping, and respiratory failure (~41%) may be present 3

CSF study 1:

  • increased leukocyte count (usually due polymorphonuclear cells)
  • increased protein 
  • normal glucose levels

It is difficult to isolate the agent: the bacillus can be cultured from blood in 61% and from CSF in 41% 3.

Aetiology

Listeria monocytogenes is an anaerobic and gram-positive bacillus.

Beam-hardening artifacts limit the visualization of the brainstem on CT images (initial CT of the brain often gives normal results) and MRI is the imaging modality of choice for the assessment of patients with suspected pathology in that anatomic location. Protocol should include contrast. 

Subcortical abscesses in the thalamus, pons and medulla are common in rhombencephalitis caused by this bacteria 2,3

MRI
  • T1: hypo to isointense lesion
  • T2/FLAIR: hyperintense
  • DWI: hyperintense/hypointense on ADC map
  • T1 C+:     
    • linear cranial nerve enhancement
    • ring enhancement (abscess)
    • heterogeneous if extensive inflammation

It is reported to have a high mortality (~50%) and frequent serious sequelae for survivors (~60%) 3

Survival is strongly related to the timely use of appropriate antibiotics: usually ampicillin or penicillin combined with gentamicin for a period of at least six weeks 2

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Article information

rID: 32682
Section: Pathology
Synonyms or Alternate Spellings:
  • Listeria rhomboencephalitis

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