Rhombencephalitis entities

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Etiology

  • It refers to inflammatory diseases affecting the hindbrain (Brainstem + Cerebellum).
  • The etiologies in a research on 97 patients were as follows:
    • Unknown (n = 31)
    • MS (n = 28)
    • Behçet disease (n = 10)
    • Listeria monocytogenes (n = 9)
    • Paraneoplastic syndrome (n = 6) (3 cases associated with anti-Yo antibodies and 3 with anti-Tr antibodies),
    • EBV (n = 4)
    • MTB (n = 2)
    • Pneumococcal infection (n = 2)
    • SLE (n = 1)
    • Lymphoma (n = 1),
    • Brucella species infection (n = 1)
    • JCV (n = 1)
    • Relapsing polychondritis (n = 1).[1]



Entities

  • Ataxia (Cerebellar ataxia)
    • In 100% of patients with paraneoplastic syndromes and EBV
    • In 10% of patients with Behçet
  • Ataxia was seen in diseases that predominantly affected the cerebellum, such as subacute cerebellar degeneration and EBV encephalitis, whereas involvement of the long tracts was not noted in these patients.
  • In contrast, the long tracts were the most commonly affected structures in patients with Behçet disease, whereas ataxia was seen in only 17%. Rostral brainstem involvement has been reported in Behçet disease.
  • Low level of consciousness
    • MS and Behçet disease do not have it
    • In 17% of those with paraneoplastic syndromes
    • In 78% of patients with Listeria
  • Fever and meningeal signs
    • Present in Listeria
    • Absent in MS
    • Fever without meningeal signs in EBV


Miasms

  • EBV typically presented with ataxia + fever, and no meningeal signs, because it predominantly affects the cerebellum and not the meninges
  • Low level of consciousness + Fever, and signs of meningism were typical in Listeria infection,
  • Low level of consciousness + Fever, and signs of meningism were not observed in MS, Behçet disease, or paraneoplastic syndromes.


Related disease

Brainstem encephalitis

  1. Moragas M, Martínez-Yélamos S, Majós C, et al. Rhombencephalitis: a series of 97 patients. Medicine. 2011 Jul;90(4):256-261. DOI: 10.1097/md.0b013e318224b5af. PMID: 21694648.