Bickerstaff brainstem encephalitis

Bickerstaff brainstem encephalitis is a rare inflammatory disorder of the central nervous system,[3] first described by Edwin Bickerstaff in 1951.[4][5] It may also affect the peripheral nervous system, and has features in common with both Miller Fisher syndrome and Guillain–Barré syndrome.[2]

Bickerstaff brainstem encephalitis
Other namesBBE, Bickerstaff’s encephalitis; Bickerstaff’s syndrome.
SymptomsDrowsiness, gait disturbances, and diplopia.[1]
Diagnostic methodAnamnestic, clinical, and radiological features.[2]
Differential diagnosisGuillain-Barré syndrome and Miller-Fisher syndrome.[2]

Signs and symptoms


The most frequent initial symptoms of Bickerstaff brainstem encephalitis are drowsiness, gait disturbances, and diplopia. Throughout the course of the disorder, almost every patient develops ataxia and external ophthalmoplegia. Most patients have disturbances of consciousness such as stupor, drowsiness, or coma. Babinki’s sign, facial weakness, pupil abnormalities, bulbar palsy, and nystagmus are also commonly found. Symptoms tend to develop fairly quickly, within one to two days. There have been reports of dysesthesias and limb weakness as the presenting features of Bickerstaff brainstem encephalitis.[1]



The clinical features and course of the condition, the associated auto-antibodies against relevant antigens, and the response to treatment, all suggest that Bickerstaff brainstem encephalitis is an autoimmune disease. However, each of these criteria fails to fit a substantial proportion of patients, and there is no single test or feature which is diagnostic of Bickerstaff brainstem encephalitis. It is, therefore, possible that a proportion of cases are due to other causes, such as infection or lymphoma, but remain undiagnosed. It is also possible that there is more than one autoimmune disease that can cause an illness that would currently be diagnosed as Bickerstaff's. There is certainly overlap between Guillain–Barré syndrome, Miller Fisher syndrome and Bickerstaff brainstem encephalitis, as well as other conditions associated with anti-ganglioside antibodies such as chronic ophthalmoplegia with anti-GQ1b antibody and the pharyngo-cervico-brachial variant of GBS.[6]



Anti-GQ1b antibodies have been found in two-thirds of patients with this condition.[7] This antibody is also found in almost all cases of Miller Fisher syndrome. The EEG is often abnormal, but shows only slow wave activity, which also occurs in many other conditions, and so is of limited value in diagnosis. Similarly, raised CSF protein levels and pleocytosis are frequent but non-specific. It was originally thought[5] that raised CSF protein without pleocytosis ('albuminocytological dissociation') was a characteristic feature, as it is in Guillain–Barré syndrome, but this has not been supported in more recent work.[7] In only 30% of cases is an MRI brain scan abnormal. Nerve conduction studies may show an axonal polyneuropathy.[8]



Most patients reported in the literature have been given treatments suitable for autoimmune neurological diseases, such as, plasmapheresis and/or intravenous immunoglobulin, and most have made a good recovery.[7] The condition is too rare for controlled trials to have been undertaken.[9]



The first cases of bickerstaff brainstem encephalitis were reported in 1951 by Cloake and Bickerstaff under the name “Mesencephalitis and rhombencephalitis”.[4] Edwin Bickerstaff named the disease “brainstem encephalitis” in 1957.[10] The disorder has been known as Bickerstaff’s brainstem encephalitis ever since 1978 when Edwin Bickerstaff wrote a review in the Handbook of Clinical Neurology under the name “Brain stem encephalitis (Bickerstaff’s encephalitis)”.[11]

See also



  1. ^ a b Berkowicz, Tomasz; Selmaj, Krzysztof (2009). "Bickerstaff's Brainstem Encephalitis". Encyclopedia of Neuroscience. Berlin, Heidelberg: Springer Berlin Heidelberg. pp. 377–381. doi:10.1007/978-3-540-29678-2_615. ISBN 978-3-540-23735-8. Retrieved December 2, 2023.
  2. ^ a b c d Abide, Zakaria; Sif Nasr, Karima; Kaddouri, Soufiane; Edderai, Meryem; Elfenni, Jamal; Salaheddine, Tarik (2023). "Bickerstaff brainstem encephalitis: A case report". Radiology Case Reports. 18 (8). Elsevier BV: 2704–2706. doi:10.1016/j.radcr.2023.04.038. ISSN 1930-0433. PMC 10250568. PMID 37304311.
  3. ^ "Bickerstaff's Brainstem Encephalitis. BBE information. Patient". 21 August 2014. Retrieved 2017-04-08.
  4. ^ a b Bickerstaff, E. R.; Cloake, P. C. P. (July 14, 1951). "Mesencephalitis and Rhombencephalitis". BMJ. 2 (4723): 77–81. doi:10.1136/bmj.2.4723.77. ISSN 0959-8138. PMC 2069534. PMID 14848512.
  5. ^ a b Bickerstaff ER (1957). "Brain-stem encephalitis: further observations on a grave syndrome with benign prognosis". Br Med J. 1 (5032): 1384–7. doi:10.1136/bmj.1.5032.1384. PMC 1973653. PMID 13436795.
  6. ^ Kaida, K.; Ariga, T.; Yu, R. K (February 24, 2009). "Antiganglioside antibodies and their pathophysiological effects on Guillain-Barre syndrome and related disorders--A review". Glycobiology. 19 (7). Oxford University Press (OUP): 676–692. doi:10.1093/glycob/cwp027. ISSN 0959-6658. PMC 2688390. PMID 19240270.
  7. ^ a b c Odaka M (2003). "Bickerstaff's brainstem encephalitis: clinical features of 62 cases and a subgroup associated with Guillain–Barré syndrome". Brain. 126 (10). OUP: 2279–90. doi:10.1093/brain/awg233. PMID 12847079.
  8. ^ Cleaver, Jonathan; James, Richard; Chohan, Gurjit; Lyons, Paul (2020). "Bickerstaff's brainstem encephalitis associated with anti-GM1 and anti-GD1a antibodies". BMJ Case Reports. 13 (9). BMJ: e236545. doi:10.1136/bcr-2020-236545. ISSN 1757-790X. PMC 7511636. PMID 32948528.
  9. ^ Shahrizaila, N.; Yuki, N. (September 15, 2012). "Bickerstaff brainstem encephalitis and Fisher syndrome: anti-GQ1b antibody syndrome". Journal of Neurology, Neurosurgery & Psychiatry. 84 (5). BMJ: 576–583. doi:10.1136/jnnp-2012-302824. ISSN 0022-3050. Retrieved December 2, 2023.
  10. ^ Bickerstaff, E. R. (June 15, 1957). "Brain-stem Encephalitis". BMJ. 1 (5032): 1384–1390. doi:10.1136/bmj.1.5032.1384. ISSN 0959-8138. PMC 1973653. PMID 13436795.
  11. ^ Bickerstaff, Edwin (1978). "Brain stem encephalitis (Bickerstaff's encephalitis)". Handbook of Clinical Neurology: Infections of the nervous system, part 2 (34 ed.). Elsevier. Retrieved 2 December 2023.