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Rhomboencephalitis
  1. Jonathan Cleaver1,2,
  2. Richard James3,
  3. Claire M Rice2,4
  1. 1 Department of Neurology, Royal United Hospitals Bath NHS Foundation Trust, Bath, UK
  2. 2 Department of Neurology, North Bristol NHS Trust, Bristol, UK
  3. 3 Department of Neuroradiology, Royal United Hospitals Bath NHS Foundation Trust, Bath, UK
  4. 4 Clinical Neuroscience, Translational Health Sciences, Bristol Medical School, University of Bristol, Bristol, UK
  1. Correspondence to Dr Claire M Rice, Clinical Neuroscience, Translational Health Sciences, Bristol Medical School, University of Bristol, Bristol, UK; C.M.Rice{at}bristol.ac.uk

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.

  • infectious diseases
  • immunology
  • neuroimmunology
  • neuropathology
  • virology
  • neurovirology

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Footnotes

  • Contributors The manuscript was designed by JC and CMR. All authors contributed to drafting and revision of the manuscript for intellectual content.

  • Funding The authors have not declared a specific grant for this research from any funding agency in the public, commercial or not-for-profit sectors.

  • Competing interests None declared.

  • Patient consent for publication Not required.

  • Provenance and peer review Commissioned. Externally peer reviewed by Emma Tallantyre, Cardiff, UK.

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    Phil E M Smith Geraint N Fuller

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