Bickerstaff brainstem encephalitis

Definition:

Bickerstaff's brainstem encephalitis (BBE) is a rare immune disorder in children, characterized by progressive ophthalmoplegia, ataxia, and disturbance of consciousness. Although the etiology of BBE is unknown, it has been linked to various antecedent infections. BBE is associated with the anti-GQ1b IgG antibody, which is present in 66% of a population of patients who had BBE. An autoimmune mechanism produced by microbial infection may trigger its pathogenesis. Intravenous immunoglobulin (IVIg) is effective in immune-mediated disorders such as Fisher syndrome (FS) and Guillain-Barre syndrome (GBS). IVIg is also effective in treatment of BBE, which is similar to the clinical and immunologic features of FS or GBS. Combined therapy of IVIg and high-dose methylprednisolone is a more efficacious therapy than IVIg alone. Most patients make a clinical recovery over six months.

References:

[1]. Muneaki Matsuo, et al. Bickerstaff's brainstem encephalitis associated with IgM antibodies to GM1b and GalNAc-GD1a. J Neurol Sci. 2004 Feb 15;217(2):225-8. [Content Brief]

[2]. Nortina Shahrizaila, et al. Bickerstaff brainstem encephalitis and Fisher syndrome: anti-GQ1b antibody syndrome. J Neurol Neurosurg Psychiatry. 2013 May;84(5):576-83. [Content Brief]

[3]. Young Il Rho, et al. Overlapping Guillain-Barré syndrome and Bickerstaff's brainstem encephalitis associated with Epstein Barr virus. Korean J Pediatr. 2014 Oct;57(10):457-60. [Content Brief]

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