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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.

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

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