Bickerstaff's brainstem encephalitisis an acute, progressive cranial nerve dysfunction associated with ataxia and coma. It may result in apnoea and a reversible brain death picture.
No structural brain damage has been demonstrated.
Treatment:
- most patients can be managed by immunotherapy, using plasmapheresis or intra-venous immunoglobulin either singly or in combination
- those patients who fulfil the diagnostic criteria of BBE usually have a good prognosis
- optimal effective treatment has not been established
- intravenous immunoglobulin has proved as effective as plasma exchange, while combined treatment with methylprednisolone and intravenous immunoglobulin is controversial
Notes:
- Fisher syndrome (FS) and Bickerstaff's brainstem encephalitis (BBE) have been classified as clinical variants of Guillain-Barre syndrome (GBS)
- both conditions have the clinical findings of ophthalmoplegia and ataxia.
- FS with central lesion is difficult to be differentiated from BBE with peripheral lesion, and vice versa
- overlap of clinical findings among FS, BBE and classical GBS indicate that they may form a continuous spectrum with different peripheral and central involvement
- in 2008, a new eponymic terminology 'Fisher-Bickerstaff syndrome' (FBS) was proposed to help to diagnose the unclassified patients that had ophthalmoplegia and ataxia with preserved tendon reflexes and clear consciousness, and those overlapping cases with FS and BBE
Reference:
- Winer JB. Bickerstaff's encephalitis and the Miller Fisher syndrome. J Neurol Neurosurg Psychiatry 2001; 71: 433-435
- Ito M, Kuwabara S, Odaka M, Misawa S, Koga M, Hirata K, et al. Bickerstaff's brainstem encephalitis and Fisher syndrome form a continuous spectrum: clinical analysis of 581 cases. J Neurol 2008; 255: 674-682.
- Kuitwaard K, van Koningsveld R, Ruts L, Jacobs BC, van Doorn PA. Recurrent Guillain-Barre syndrome. J Neurol Neurosurg Psychiatry 2009; 80: 56-59.