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

Abstract

SummaryBackgroundBickerstaff’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 ReportThe 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.ConclusionsMR 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.

Bickerstaff brainstem encephalitis: clinical, neurophysiological, laboratory and postmortem findings of a case presenting as encephalomyelitis

“…The outcome of BBE is usually favourable especially for subjects who are positive for anti-GQ1b antibodies; they have less frequent abnormalities in their CSF and MRI scans, and they tend to recover more rapidly and have fewer residual deficits than those without the antibodies 3 4. There are very few reports of mortality from BBE in the literature; two of these were associated with pulmonary embolism, and another two were attributed to recurrence of BBE 6–9. The poor outcome in our patient, who had positive anti-GQ1b antibodies and did not have pulmonary embolism or recurrent BBE, is therefore unexpected.…”

Atypical Imaging Findings in Anti-GQ1b Brainstem Encephalitis

“…5 They include extensive high-signal intensity lesions on T2-weighted imaging, concerning the midbrain and pons, and sometimes the thalamus and basal ganglia. 5,6 One single report to date described additional involvement of the spinal cord. 7 These abnormalities usually reduce or disappear completely, after several months, even though, they might remain for longer periods, according to some reports.…”

“…7 These abnormalities usually reduce or disappear completely, after several months, even though, they might remain for longer periods, according to some reports. 6 Post-gadolinium enhancement is also usually not detected, with some authors arguing this can be present along with other autoimmunological diseases. 8 Furthermore, Roos et al 9 described a case, in which strong focal enhancement in the brainstem was detected.…”

Bickerstaff Brainstem Encephalitis with Isolated Acute Bilateral Ophthalmoplegia

“…[ 7 ] BBE is usually monophasic and self-limiting, with most patients achieving complete remission by 6 months;[ 12 ] however, individual relapses have also been described. [ 13 ]…”