3. Bickerstaff's Brainstem Encephalitis

Bickerstaff's Brainstem Encephalitis

This PatientPlus article is written for healthcare professionals so the language may be more technical than the condition leaflets. You may find the abbreviations list helpful.

This disease is notifiable in the UK.

Bickerstaff's brainstem encephalitis (BBE) is an immune disorder of unknown aetiology. It is characterised by:

There is a clinical continuum between BBE and Fisher's syndrome.[1]

Bickerstaff reported eight patients who, in addition to acute ophthalmoplegia (diplopia) and ataxia, showed drowsiness, extensor plantar responses or hemisensory loss.[2]

  • Very rare. Mostly reported in adults but cases affecting children have also been reported.[3]
  • Very often follows a preceding illness, and an association with certain infections, including cytomegalovirus, Campylobacter jejuni, typhoid fever and Mycoplasma pneumoniae, has been documented.[4][5][6]

Multiple sclerosis.
Behçet's disease.
Lyme disease.
Progressive multifocal leukoencephalopathy.
Sarcoidosis.
Whipple's disease.
Listeria rhombencephalitis.
Vasculitis due to systemic lupus erythematosus (SLE).
Acute disseminated encephalomyelitis .

  • One review of 62 patients found positive serum anti-GQ1b immunoglobulin G (IgG) antibody in 66%, and brain abnormality on MRI scan in 30% of patients.[4][8]
  • The presence of anti-GQ1b antibodies and an abnormal brain MRI scan can help to support its diagnosis but absence of anti-GQ1b antibodies and a normal MRI scan result do not exclude the diagnosis, which remains based on clinical criteria and exclusion of other aetiologies.[9]
  • Electrodiagnostic study results suggested peripheral motor axonal degeneration.
  • A large number of patients have associated Guillain-Barré syndrome, suggesting that the two disorders are closely related.[10]
  • Miller Fisher's syndrome[11] (ophthalmoplegia, ataxia and absent reflexes).

Success has been achieved with treatment with steroids plus double filtration plasmapheresis[12] and also with immunoglobulin therapy.[13] However, there are no randomised controlled trials of immunomodulatory therapy.[14]

Although the initial presentation is severe, there is usually a good outcome with complete resolution.[15] Cases of recurrent Bickerstaff's brainstem encephalitis (BBE) have been reported.[9]

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Further reading & references

  1. Ito M, Kuwabara S, Odaka M, et al; Bickerstaff's brainstem encephalitis and Fisher syndrome form a continuous spectrum : Clinical analysis of 581 cases. J Neurol. 2008 Feb 18;.
  2. Bickerstaff ER, Cloake PC; Mesencephalitis and rhombencephalitis. Br Med J. 1951 Jul 14;4723:77-81.
  3. Fargas A, Roig M, Vazquez E, et al; Brainstem involvement in a child with ophthalmoplegia, ataxia, areflexia syndrome. Pediatr Neurol. 1998 Jan;18(1):73-5.
  4. Steer AC, Starr M, Kornberg AJ; Bickerstaff brainstem encephalitis associated with Mycoplasma pneumoniae infection. J Child Neurol. 2006 Jun;21(6):533-4.
  5. Kanzaki A, Yabuki S, Yuki N; Bickerstaff's brainstem encephalitis associated with cytomegalovirus infection. J Neurol Neurosurg Psychiatry. 1995 Feb;58(2):260-1.
  6. Hussain AM, Flint NJ, Livsey SA, et al; Bickerstaff's brainstem encephalitis related to Campylobacter jejuni gastroenteritis. J Clin Pathol. 2007 Oct;60(10):1161-2. Epub 2007 May 18.
  7. Falini A, Kesavadas C, Pontesilli S, et al; Differential diagnosis of posterior fossa multiple sclerosis lesions--neuroradiological aspects. Neurol Sci. 2001 Nov;22 Suppl 2:S79-83.
  8. Odaka M, Yuki N, Yamada M, et al; Bickerstaff's brainstem encephalitis: clinical features of 62 cases and a subgroup associated with Guillain-Barre syndrome. Brain. 2003 Oct;126(Pt 10):2279-90. Epub 2003 Jul 7.
  9. Sharma V, Chan YC, Ong, et al; Bickerstaff's brainstem encephalitis: can it recur? J Clin Neurosci. 2006 Feb;13(2):277-9. Epub 2006 Jan 26.
  10. Yuki N, Wakabayashi K, Yamada M, et al; Overlap of Guillain-Barre syndrome and Bickerstaff's brainstem encephalitis. J Neurol Sci. 1997 Jan;145(1):119-21.
  11. Yuki N; Bickerstaff's brainstem encephalitis and Fisher syndrome: their relationship and treatment. Rinsho Shinkeigaku. 2004 Nov;44(11):802-4.
  12. Fujisato H, Amemiya M, Hayashi Y, et al; Treatment with steroids and double filtration plasmapheresis for a case of anti-GQ1b antibody-positive Bickerstaff's encephalitis. Ther Apher. 1999 Feb;3(1):72-4.
  13. Fox RJ, Kasner SE, Galetta SL, et al; Treatment of Bickerstaff's brainstem encephalitis with immune globulin. J Neurol Sci. 2000 Sep 15;178(2):88-90.
  14. Overell JR, Hsieh ST, Odaka M, et al; Treatment for Fisher syndrome, Bickerstaff's brainstem encephalitis and related disorders. Cochrane Database Syst Rev. 2007 Jan 24;(1):CD004761.
  15. Berkowicz T, Siger-Zajdel M, Zaleski K, et al; Bickerstaff's brainstem encephalitis -- an analysis of clinical and MRI findings. Neurol Neurochir Pol. 2006 Jan-Feb;40(1):16-21.
Original Author: Dr Colin Tidy Current Version:
Last Checked: 26/10/2010 Document ID: 1283  Version: 22 © EMIS

Disclaimer: This article is for information only and should not be used for the diagnosis or treatment of medical conditions. EMIS has used all reasonable care in compiling the information but make no warranty as to its accuracy. Consult a doctor or other health care professional for diagnosis and treatment of medical conditions. For details see our conditions.

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