Background: Bickerstaff's brainstem encephalitis (BBE) is a very rare disease of the central nervous system. Aetiology of the disease is auto-immunological. However, it is not entirely understood. Clinically BBE manifests in progressive ophthalmoplegia, ataxia and consciousness disturbances. Clinical symptoms are usually preceded by an unidentified infection of the upper respiratory tract. Usually, the disease has one phase, but individual relapses have also been described. Despite quite severe clinical symptoms, the prognosis is usually good.
Case report: The article presents a case of a patient with relapsing-remitting severe BBE. The case is presented due to the relapsing-remitting clinical course of the disease that resulted in patient's death, rarely described in the literature. We also present the results of subsequent MR scans in the course of the disease, so far described only in individual reports. It is also the first report in the world's literature presenting the results of series of MR spectroscopy (MRS) examinations in the course of BBE.
Conclusions: MR examination is an important component in BBE diagnostics, allowing to differentiate atypical cases and place them under special supervision due to the possibility of the severe clinical course. MR also facilitates differentiation between Miller-Fisher Syndrome (MFS) and BBE in cases of diagnostic doubts. Adding MRS and MRI to the protocol allows us to define the nature of morphological changes more accurately in patients with suspected or diagnosed BBE.
Keywords: Brain Stem; Encephalitis; Magnetic Resonance Imaging; Magnetic Resonance Spectroscopy; Miller Fisher Syndrome.
Conflict of interest statement
The authors declare that they have no conflict of interest.
[Bickerstaff's brainstem encephalitis -- an analysis of clinical and MRI findings].Neurol Neurochir Pol. 2006 Jan-Feb;40(1):16-21. Neurol Neurochir Pol. 2006. PMID: 16463217 Polish.
A pediatric case of Bickerstaff's brainstem encephalitis.Korean J Pediatr. 2014 Dec;57(12):542-5. doi: 10.3345/kjp.2014.57.12.542. Epub 2014 Dec 31. Korean J Pediatr. 2014. PMID: 25653689 Free PMC article.
An atypical Bickerstaff's brainstem encephalitis with involvement of spinal cord.Neuroradiol J. 2016 Oct;29(5):396-9. doi: 10.1177/1971400916665383. Epub 2016 Aug 18. Neuroradiol J. 2016. PMID: 27540012 Free PMC article.
Bickerstaff's brainstem encephalitis: clinical features of 62 cases and a subgroup associated with Guillain-Barré syndrome.Brain. 2003 Oct;126(Pt 10):2279-90. doi: 10.1093/brain/awg233. Epub 2003 Jul 7. Brain. 2003. PMID: 12847079 Review.
[Bickerstaff's brainstem encephalitis and Fisher syndrome: their relationship and treatment].Rinsho Shinkeigaku. 2004 Nov;44(11):802-4. Rinsho Shinkeigaku. 2004. PMID: 15651296 Review. Japanese.
- Berkowicz T, Siger-Zajdel M, Zaleski K, et al. Zapalenie pnia mózgu Bickerstaffa – analiza przebiegu klinicznego i obrazu rezonansu magnetycznego. Neurol Neuroch Pol. 2006;40(1):16–21. [in Polish] - PubMed
- Sharma V, Chan YC, Ong, et al. Bickerstaff’s brainstem encephalitis: Can it recur? J Clin Neurosci. 2006;13(2):277–79. - PubMed
- Yuki N. Fisher syndrome and Bickerstaff brainstem encephalitis (Fisher-Bickerstaff syndrome) J Neuroimmunol. 2009;215(1–2):1–9. - PubMed