| Literature DB >> 29459060 |
Choong Yi Fong1, Hnin Wint Wint Aung2, Arie Khairani3, Chin Seng Gan4, Nortina Shahrizaila5, Khean Jin Goh5.
Abstract
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.Entities:
Keywords: Acute inflammatory demyelinating polyneuropathy; Bickerstaff’s brainstem encephalitis; Gullain-Barré syndrome; Nerve conduction study
Mesh:
Year: 2018 PMID: 29459060 DOI: 10.1016/j.braindev.2018.02.001
Source DB: PubMed Journal: Brain Dev ISSN: 0387-7604 Impact factor: 1.961