(Bickerstaff syndrome)

Prevalence: unknown. Very rare form of post-infectious encephalitis combining external ophthalmoplegia, ataxia, areflexia of the lower limbs and disorders of consciousness. Onset: a few days after an infection of the upper respiratory tract or gastroenteritis. Involved germs are most often the CMV, Mycoplasma pneumoniae or Campylobacter jejuni. Very often presence of antiganglioside antibodies, especially anti-GQ1b.

Clinical signs:

-        onset by symmetric and progressive external ophthalmoplegia

-         flaccid and progressive tetraplegia in 50 %

-        facial paresis, nystagmus, ptosis

-        bulbar palsy.

-        consciousness: varies from drowsiness to deep coma.

Can present a clinical picture of 'locked-in' syndrome or even apparent brain death.

Management: symptomatic treatment, immunoglobulins, sometimes plasmapheresis.

Evolution: full resolution in 6 months in 50% of cases; if not, neurological sequelae of varying severity.

Differential diagnosis: Guillain-Barre syndrome‚ and Miller-Fisher syndrome

Anesthetic implications:

during the critical period: neurological patient with consciousness and swallowing disorders; after healing: avoid succinylcholine for several months (presence of diffuse ACh fetal postsynaptic receptors following the prolonged denervation).

References : 

-        Battaglia F, Franques J, Somma-Mauvais H, Roche P-H.  
Tableau d’encéphalite de Bickerstaff et hypertension intracrânienne. 
Revue Neurologique 2011 ; 167 : 164-8.

-         Jones GD, Wilmshurst JM, Sykes K, Murdoch IA. 
Guillain-Barre syndrome: delayed diagnosis following anaesthesia. 
Paediatr Anaesth 1999; 9:539-42.

Updated: November 2019