Bickerstaff’s brainstem encephalitis (BBE) in childhood: rapid resolution after intravenous immunoglobulins treatment

P. Pavone, A. Le Pira, F. Greco, G. Vitaliti, P.L. Smilari, E. Parano, R. Falsaperla

Unit of Pediatrics and Pediatric Emergency, University Hospital, “Vittorio Emanuele Polyclinic”, Catania, Italy. ppavone@unict.it

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• Neurology

Three young patients with Bickerstaff’s brainstem encephalitis (BBE) are reported. Some weeks following an upper tract infection, the children after a short period of recovery, showed acute onset of symmetric weakness of the lower limbs with difficulty in standing by and walking. The distal muscle weakness had a rapid progression with involvement of the cranial nerve, and then with severe impairment of the consciousness till to coma in one of the three children. BBE is a rare and often underdiagnosed affection in childhood. Common neuro-immune pathogenesis, overlap of clinical signs and strict correlation among BBE with Fisher syndrome and Guillain-Barrè syndrome lead to think that these affections represent an unique spectrum with different central and peripheral involvement.

In these children, treatment with intravenous immunoglobulins resulted in a progressive and rapid resolution of the clinical features.

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