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Bickerstaff's brainstem encephalitis (BBE) is an immune disorder of unknown aetiology. It is characterised by:
- Acute, progressive cranial nerve dysfunction.
- Associated cerebellar ataxia.
There is a clinical continuum between BBE and Fisher's syndrome.
Bickerstaff reported eight patients who, in addition to acute ophthalmoplegia (diplopia) and ataxia, showed drowsiness, extensor plantar responses or hemisensory loss.
- It is very rare and mostly reported in adults; however, cases affecting children have also been reported.
- Very often it follows an illness, and an association with certain infections, including cytomegalovirus, Campylobacter jejuni, typhoid fever and Mycoplasma pneumoniae, has been documented.[4, 5, 6]
- Acute diplopia.
- Pyramidal tract paralysis.
- Disturbance of consciousness.
- Headache is common.
- Progressive, symmetrical ophthalmoplegia, ataxia and either disturbance of consciousness or hyperreflexia.
- Facial palsy, extensor plantar reflex, pupillary abnormality, nystagmus and bulbar palsy.
- It may result in apnoea and a reversible brain death picture.
Progressive multifocal leukoencephalopathy.
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.
- 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.
- Miller Fisher's syndrome (ophthalmoplegia, ataxia and absent reflexes).
Success has been achieved with treatment with steroids plus double filtration plasmapheresis.It has also been achieved with immunoglobulin therapy. However, there are no randomised controlled trials of immunomodulatory therapy.
Further reading and references
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.
Bickerstaff ER, Cloake PC; Mesencephalitis and rhombencephalitis. Br Med J. 1951 Jul 144723:77-81.
Lin JJ, Hsia SH, Wang HS, et al; Clinical variants of Guillain-Barre syndrome in children. Pediatr Neurol. 2012 Aug47(2):91-6. doi: 10.1016/j.pediatrneurol.2012.05.011.
Steer AC, Starr M, Kornberg AJ; Bickerstaff brainstem encephalitis associated with Mycoplasma pneumoniae infection. J Child Neurol. 2006 Jun21(6):533-4.
Kanzaki A, Yabuki S, Yuki N; Bickerstaff's brainstem encephalitis associated with cytomegalovirus infection. J Neurol Neurosurg Psychiatry. 1995 Feb58(2):260-1.
Hussain AM, Flint NJ, Livsey SA, et al; Bickerstaff's brainstem encephalitis related to Campylobacter jejuni gastroenteritis. J Clin Pathol. 2007 Oct60(10):1161-2. Epub 2007 May 18.
Falini A, Kesavadas C, Pontesilli S, et al; Differential diagnosis of posterior fossa multiple sclerosis lesions--neuroradiological aspects. Neurol Sci. 2001 Nov22 Suppl 2:S79-83.
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 Oct126(Pt 10):2279-90. Epub 2003 Jul 7.
Sharma V, Chan YC, Ong, et al; Bickerstaff's brainstem encephalitis: can it recur? J Clin Neurosci. 2006 Feb13(2):277-9. Epub 2006 Jan 26.
Yuki N; Bickerstaff's brainstem encephalitis and Fisher syndrome: their relationship and treatment. Rinsho Shinkeigaku. 2004 Nov44(11):802-4.
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 Feb3(1):72-4.
Fox RJ, Kasner SE, Galetta SL, et al; Treatment of Bickerstaff's brainstem encephalitis with immune globulin. J Neurol Sci. 2000 Sep 15178(2):88-90.
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.
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-Feb40(1):16-21.