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Relapsing-Remitting Severe Bickerstaff's Brainstem Encephalitis - Case Report and Literature Review

Case Reports

Relapsing-Remitting Severe Bickerstaff's Brainstem Encephalitis - Case Report and Literature Review

Zuzanna Tyrakowska et al. Pol J Radiol.


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.


Figure 1
Figure 1
Following MRI in a 59-year-old patient with Bickerstaff encephalitis (FLAIR axial images). Admission MRI showed an irregular hyperintensity area in the dorsal pons spreading towards the medulla (A). MRI repeated after 5 months showed a significant regression of changes (B). After 1.5 year MRI showed recurrence of the previously stated lesions, with involvement of the pons, cerebral and cerebellar peduncles (C). Subsequent MRI showed a considerable increase in the extension of hyperintensity involving the brain stem and spreading towards the hemispheres of the cerebellum (D).
Figure 2
Figure 2
Following MRI in a patient with Bickerstaff encephalitis; T1-weighted images after contrast enhancement. In initial MRI, a small central area slightly enhancing after injection of the contrast medium (A). Regression of changes in the brainstem and no significant enhancement lesion in MRI after 5 months (B). MRI after 1.5 year revealed irregular areas of enhancement after injection of the contrast medium (C). An intensive, irregular area with strong enhancement after injection of the contrast in a subsequent MRI (D).
Figure 3
Figure 3
Initial single voxel proton MR spectroscopy showed the correct proportions of the main metabolites (NAA/Cr, Cho/Cr and mI/Cr) in the changed area, with the presence of lactate and lipid bands (A). Control MRS in the second episode after 1.5 year showed a slight reduction of NAA/Cr and an increase in Cho/Cr and mI/Cr with still present lactate and lipids (B). Subsequent MRS showed a considerable progressive reduction of NAA/Cr and a clear increase in Cho/Cr and mI/Cr with a significant increase of lactate and lipid peaks (C).

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