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Rhombencephalitis (aka brainstem encephalitis)
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Rhombencephalitis (aka brainstem encephalitis)

Patient presents with diplopia, vertigo, and incoordination. Exam reveals profound horizontal eye movement abnormalities and right side ataxia.
  • MRI imaging demonstrates significant pontine inflammation and right cerebellar inflammation.
  • CSF reveals a mild pleocytosis with significantly elevated protein.
  • The umbrella diagnosis is rhombencephalitis (aka brainstem encephalitis).
Notable causes of isolated rhombencephalitis include:
  • Infections
– Listeria (treated with ampicillin, look especially for abscesses with ring enhancement) – Enterovirus 71 – HSV (although this commonly also involves supratentorial encephalitis) rarely: – EBV – CMV – HHV6
  • Autoimmune Diseases
notably: – Behçet Disease
  • Paraneoplastic Syndromes
Consider that rhombencephalitic inflammation/disease may accompany supratentorial findings in other illnesses (ie, these are less likely to have isolated rhombencephalitis), including:
  • PML, Aspergillosis, Pneumococcus, Tuberculosis, Lupus, HIV
  • ADEM, MS, Sarcoidosis
  • Radiation Necrosis
For interesting review articles, see: Curr Neurol Neurosci Rep. 2011 Dec;11(6):543-52. doi: 10.1007/s11910-011-0228-5. Radiol Bras. 2016 Sep-Oct; 49(5): 329–336.

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