Bickerstaff's Brainstem Encephalitis
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Bickerstaff's brainstem encephalitis (BBE) is an immune disorder of unknown aetiology. It is characterised by:
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- Acute, progressive cranial nerve dysfunction.
- Associated cerebellar ataxia.
- Coma.
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].
Epidemiology
- It is very rare and mostly reported in adults; however, cases affecting children have also been reported[3].
- 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].
Presentation
- Acute diplopia.
- Ataxia.
- 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.
Differential diagnosis[7]
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.
Investigations
- 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.
Associated diseases
- A large number of patients have associated Guillain-Barré syndrome, suggesting that the two disorders are closely related[8].
- Miller Fisher's syndrome (ophthalmoplegia, ataxia and absent reflexes)[10].
Management
Success has been achieved with treatment with steroids plus double filtration plasmapheresis[11]. It has also been achieved with immunoglobulin therapy[12]. However, there are no randomised controlled trials of immunomodulatory therapy[13].
Prognosis
Although the initial presentation is severe, there is usually a good outcome with complete resolution[14]. Cases of recurrent BBE have been reported[9].
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.