Bickerstaff's brainstem encephalitis with overlapping Guillain-Barré syndrome: Usefulness of sequential nerve conduction studies.

Autor: Fong CY; Division of Paediatric Neurology, Department of Paediatrics, Faculty of Medicine, University of Malaya, Kuala Lumpur, Malaysia. Electronic address: cyfong@ummc.edu.my., Aung HWW; Division of Paediatric Neurology, Department of Paediatrics, Faculty of Medicine, University of Malaya, Kuala Lumpur, Malaysia; Paediatric Neurology Unit, Yangon Children Hospital, Yangon, Myanmar., Khairani A; Division of Paediatric Neurology, Department of Paediatrics, Faculty of Medicine, University of Malaya, Kuala Lumpur, Malaysia; Division of Paediatric Neurology, National Brain Centre, East Jakarta, Jakarta, Indonesia., Gan CS; Division of Paediatric Intensive Care, Department of Paediatrics, Faculty of Medicine, University of Malaya, Kuala Lumpur, Malaysia., Shahrizaila N; Division of Neurology, Faculty of Medicine, University of Malaya, Kuala Lumpur, Malaysia., Goh KJ; Division of Neurology, Faculty of Medicine, University of Malaya, Kuala Lumpur, Malaysia.
Jazyk: angličtina
Zdroj: Brain & development [Brain Dev] 2018 Jun; Vol. 40 (6), pp. 507-511. Date of Electronic Publication: 2018 Feb 17.
DOI: 10.1016/j.braindev.2018.02.001
Abstrakt: Bickerstaff's brainstem encephalitis (BBE) is a rare immune-mediated disorder characterized by ophthalmoplegia, ataxia and disturbance of consciousness, which may overlap with Guillain-Barré syndrome (GBS) if there is additional limb weakness. We report a 7-month-old boy presented with ophthalmoplegia followed by a rapidly ascending paralysis of all four limbs and disturbance of consciousness. The initial impression was BBE with overlapping GBS. This was supported by sequential nerve conduction study (NCS) findings compatible with an acute inflammatory demyelinating polyneuropathy (AIDP). He received intravenous pulse methylprednisolone, intravenous immunoglobulin and plasmapharesis with complete clinical recovery after 6 weeks of illness and improved NCS findings from week 16. This is the first case of paediatric BBE with overlapping GBS with an AIDP subtype of GBS. It expands the clinical spectrum of this condition in children. Our case highlights the importance of sequential NCS in paediatric BBE with overlapping GBS for accurate electrophysiological diagnosis and prognosis particularly if the first NCS findings are not informative.
(Copyright © 2018 The Japanese Society of Child Neurology. Published by Elsevier B.V. All rights reserved.)
Databáze: MEDLINE
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