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Table 4 Acute and chronic diseases involving the brainstem

From: Brainstem dysfunction in critically ill patients

Causes of brainstem dysfunction

Acute primary insult

 Vascular injury

  Ischemic: thrombotic or cardio-embolic, lacunar ischemia due to small vessel disease, vasculitis

  Hemorrhage

 Inflammatory

  Multiple sclerosis (MS)

  Acute disseminated encephalomyelitis (ADEM)

  Neuromyelitis optica (NMO) (anti-MOG, anti-AQP4 antibodies, or seronegative types)

  Birkenstaff encephalitis (anti-ganglioside GQ1b antibodies)

  Behcet disease and rarely other autoimmune disease (lupus, neuro-sarcoidosis)

  Langerhans cell histiocytosis

 Traumatic: direct or indirect injury

 Metabolic: central pontine myelinolysis

 Infectious: rhombencephalitis, abscess, Listeria monocytogenes and enterovirus 68 and 71, followed by herpes simplex viruses and tuberculosis, Epstein-Barr virus (EBV), and human herpesvirus 6 (HHV6)

 Paraneoplastic (anti-neuronal NMDA, AMPA, GABA, CASPR2, Hu, Ma2, Ri, Yo, CV2, amphiphysin, Lgi1,glycine, mGluR1/5, VGKC/VGCC, GAD antibodies)

Chronic primary insult

 Tumoural

 Degenerative/atrophic injury

  1. MRI magnetic resonance imaging, TDM tomodensitometry, CSF cerebrospinal fluid, ECG electrocardiogram
  2. MRI results according to etiologies:
  3. Vascular injury: diffusion and FLAIR-weighted sequence hyperintensity restricted to a vascular territory
  4. Hemorrhage: SWI/T2* sequence hypointensity
  5. Inflammatory: diffuse or multifocal white matter lesions on T2- and FLAIR-weighted sequences, with or without contrast enhancement
  6. Inflammatory NMO (MRI of optical nerve and medullary MRI): extensive and confluent myelitis on more than three vertebrae and optical neuritis with possible contrast enhancement
  7. Traumatic injury: hyperintensity on diffusion sequence, diffuse axonal injuries on DTI (diffusion tensor imaging) sequence, hemorrhage lesions on T2*/SWI
  8. Metabolic: T2 hyperintensity specifically involves the central pons
  9. Infectious: abscess/nodes with contrast enhancement
  10. Paraneoplastic: limbic encephalitis with temporal diffusion and FLAIR hyperintensity
  11. Tumor: mass with possible necrosis, contrast enhancement and oedema revealed by a FLAIR hyperintensity around tumor
  12. Degenerative injury: brain and brainstem atrophy (colibri sign)