Rhombencephalitis entities

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).

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

• Involvement of long tracts (spino-thalamic and corticospinal tracts and posterior column) was
  • In 70% of Behçet patients
  • In 17% of paraneoplastic syndromes
  • No 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