Revision 2 for 'Rhombencephalitis'

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Rhomboencephalitis

Rhombencephalitis (RE) corresponds to inflammatory diseases affecting the hindbrain (brainstem and cerebellum) and has a wide variety of etiologies including infections, autoimmune diseases, and paraneoplastic syndromes.

Terminology

It is important to remember that both terms "rhombencephalitis" and "brainstem encephalitis”, despite being used as interchangeable terms, are anatomically different, once the first includes the cerebellum besides brainstem. 


Epidemiology

Clinical presentation


Pathology

Infectious causes

  • Viral
    • Listeria monocytogenes: the most common cause of infectious RE
    • enterovirus 71: is probably the second most common 
    • herpes simplex virus (HSV): is the third most common
      • 80% are caused by HSV1 
      • 20% are caused by HSV2
      • Epstein-Barr virus (EBV)
    • human herpesvirus 6 (HHV6) 
    • Flaviviruses (e.g. West Nile virus and Japanese encephalitis virus)
  • Bacterial
    • Mycobacterium tuberculosis 
    • Rickettsia, Borrelia burgdoferi, Salmonela typhi, Legionella bozemanii and Mycoplasma pneumoniae are agents that rarely cause encephalitis, but can involve the brainstem. 

Autoimmune

  • Behçet disease is the most common autoimmune etiology to promote RE. 

Paraneoplastic causes


Radiographic features


Beam-hardening artifacts limit the visualization of the brainstem on CT images and MRI is the imaging modality of choice for the assessment of patients with suspected pathology in that anatomic location. Protocol should include contrast. 
MRI
    T1: hypo-isointense lesion.
    T2/FLAIR: hyperintense.
    DWI: hyperintense / hypointense on ADC map.
    Spectroscopy: allows differentiate abscess (peak lactate and aa + little increase perfusion) from tumor
    T1 C+:     linear cranial nerve enhancement
        Ring enhancement (abscess)
        heterogeneous if extensive inflammation.


Treatment and prognosis

RE require early diagnosis and treatment to avoid neurological sequelae 

History and etymology

It was first reported by Edwin Bickerstaff and Philip Cloake in 1951 2


Differential diagnosis

  • brainstem tumor

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