Nationwide survey of patients in Japan with Bickerstaff brainstem encephalitis: epidemiological and clinical characteristics

Abstract

BBE is a rare disorder but accounts for a major proportion of brainstem encephalitis. BBE consists of typical and atypical cases. Typical BBE has similar neurological and serological features to Fisher syndrome and shows good recovery whereas atypical BBE is characterised by delayed recovery, negative anti-GQ1b antibodies, and abnormal CSF and brain MRI findings with other possible pathogeneses.

Ambiguous value of anti-ganglioside IgM autoantibodies in Guillain-Barré syndrome and its variants

“…Diagnoses of GBS [15], FS [16], and BBE [17] were based on published criteria. Control sera from 22 enteritis patients without subsequent neurological deficits from whom C. jejuni had been isolated were also included.…”

Blood–brain barrier destruction determines Fisher/Bickerstaff clinical phenotypes: an in vitro study

“…BBE is a rare disorder probably affecting no more than one individual per 1 000 000 9. An autopsy case of a BBE patient demonstrated perivascular lymphocytic infiltration with perivascular oedema in the brainstem, suggesting that disruption of BBB may be pathogenic in the development of BBE 10.…”

Atypical Bickerstaff brainstem encephalitis: ataxic hypersomnolence without ophthalmoplegia

“…BBE is rare, with reported annualised incidence of approximately one patient per million 4. Understanding the clinical spectrum of BBE continues to evolve and relies on systematic review of published reports.…”

“…The discovery three decades later that most patients presenting with this clinical triad carry autoantibodies against GQ1b ganglioside has reinforced the view that so called Bickerstaff brainstem encephalitis (BBE) forms a clinicopathological continuum with Fisher syndrome (FS) 2 3. Current diagnostic criteria for BBE require the development of progressive, symmetrical ophthalmoplegia 4 5. However, reports of ‘incomplete’ FS in patients with no, minimal or unilateral ophthalmoplegia,3 6 suggests that BBE may present with variable degrees of ophthalmoplegia.…”