Bickerstaff Brainstem Encephalitis and overlapping Guillain-Barré syndrome in children: Report of two cases and review of the literature

A. Michev, P. Musso, T. Foiadelli, C. Trabatti, A. Lozza, D. Franciotta, A. M. Simoncelli, S. Savasta

Research output: Contribution to journalArticle

Abstract

Bickerstaff Brainstem Encephalitis (BBE) is a rare autoimmune encephalitis, characterized by acute ophthalmoplegia, ataxia and altered state of consciousness. Together with Guillan-Barrè Syndrome (GBS) and Miller–Fisher Syndrome, it forms a spectrum of post-infectious demyelinating diseases. Overlapping forms between BBE and GBS (BBE/GBS) are described in patients with lower limbs weakness and typical signs of BBE, suggesting a combined involvement of Central and Peripheral Nervous System (PNS), but only few reported cases are focused on pediatric population. We reviewed all cases of pediatric BBE in the literature, to determine if any patient showed features suggestive for BBE/GBS. Data analysis focused on the diagnostic tests performed (e.g. anti-GQ1b antibodies), neuroimaging and nerve conduction studies (NCS). Further attention was given to the therapeutic management and to patients’ outcome. We additionally present two previously unreported pediatric cases. Our review retrieved 19 cases of BBE/GBS, only 2 of which were originally and correctly diagnosed by the authors. The prevalence was higher in male subjects (ratio 3:1) and median age at diagnosis was 8 years. Anti-GQ1b were positive in 46% of the patients, while NCS were altered in 64%. Only 25% of the patients that underwent brain MRI showed abnormal findings. The incidence of BBE/GBS has been underrated in the past, mostly due to an underestimation of the PNS involvement. We therefore suggest to investigate all patients with a clinical picture suggestive of BBE/GBS through electroencephalogram, NCS, brain and spine MRI in order to promptly achieve the correct diagnosis.

Original languageEnglish
JournalEuropean Journal of Paediatric Neurology
DOIs
Publication statusAccepted/In press - Jan 1 2018

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Encephalitis
Brain Stem
Neural Conduction
Peripheral Nervous System
Pediatrics
Ophthalmoplegia
Brain
Demyelinating Diseases
Ataxia
Consciousness
Routine Diagnostic Tests
Neuroimaging
Communicable Diseases
Electroencephalography
Anti-Idiotypic Antibodies
Lower Extremity
Spine
Central Nervous System
Incidence
Population

Keywords

  • Anti-GQ1b
  • Bickerstaff Brainstem Encephalitis
  • Guillain-Barré syndrome
  • Nerve conduction studies
  • Pediatric

ASJC Scopus subject areas

  • Pediatrics, Perinatology, and Child Health
  • Clinical Neurology

Cite this

@article{efef919e20e44d6e9ad39539d5faa1aa,
title = "Bickerstaff Brainstem Encephalitis and overlapping Guillain-Barr{\'e} syndrome in children: Report of two cases and review of the literature",
abstract = "Bickerstaff Brainstem Encephalitis (BBE) is a rare autoimmune encephalitis, characterized by acute ophthalmoplegia, ataxia and altered state of consciousness. Together with Guillan-Barr{\`e} Syndrome (GBS) and Miller–Fisher Syndrome, it forms a spectrum of post-infectious demyelinating diseases. Overlapping forms between BBE and GBS (BBE/GBS) are described in patients with lower limbs weakness and typical signs of BBE, suggesting a combined involvement of Central and Peripheral Nervous System (PNS), but only few reported cases are focused on pediatric population. We reviewed all cases of pediatric BBE in the literature, to determine if any patient showed features suggestive for BBE/GBS. Data analysis focused on the diagnostic tests performed (e.g. anti-GQ1b antibodies), neuroimaging and nerve conduction studies (NCS). Further attention was given to the therapeutic management and to patients’ outcome. We additionally present two previously unreported pediatric cases. Our review retrieved 19 cases of BBE/GBS, only 2 of which were originally and correctly diagnosed by the authors. The prevalence was higher in male subjects (ratio 3:1) and median age at diagnosis was 8 years. Anti-GQ1b were positive in 46{\%} of the patients, while NCS were altered in 64{\%}. Only 25{\%} of the patients that underwent brain MRI showed abnormal findings. The incidence of BBE/GBS has been underrated in the past, mostly due to an underestimation of the PNS involvement. We therefore suggest to investigate all patients with a clinical picture suggestive of BBE/GBS through electroencephalogram, NCS, brain and spine MRI in order to promptly achieve the correct diagnosis.",
keywords = "Anti-GQ1b, Bickerstaff Brainstem Encephalitis, Guillain-Barr{\'e} syndrome, Nerve conduction studies, Pediatric",
author = "A. Michev and P. Musso and T. Foiadelli and C. Trabatti and A. Lozza and D. Franciotta and Simoncelli, {A. M.} and S. Savasta",
year = "2018",
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T2 - Report of two cases and review of the literature

AU - Michev, A.

AU - Musso, P.

AU - Foiadelli, T.

AU - Trabatti, C.

AU - Lozza, A.

AU - Franciotta, D.

AU - Simoncelli, A. M.

AU - Savasta, S.

PY - 2018/1/1

Y1 - 2018/1/1

N2 - Bickerstaff Brainstem Encephalitis (BBE) is a rare autoimmune encephalitis, characterized by acute ophthalmoplegia, ataxia and altered state of consciousness. Together with Guillan-Barrè Syndrome (GBS) and Miller–Fisher Syndrome, it forms a spectrum of post-infectious demyelinating diseases. Overlapping forms between BBE and GBS (BBE/GBS) are described in patients with lower limbs weakness and typical signs of BBE, suggesting a combined involvement of Central and Peripheral Nervous System (PNS), but only few reported cases are focused on pediatric population. We reviewed all cases of pediatric BBE in the literature, to determine if any patient showed features suggestive for BBE/GBS. Data analysis focused on the diagnostic tests performed (e.g. anti-GQ1b antibodies), neuroimaging and nerve conduction studies (NCS). Further attention was given to the therapeutic management and to patients’ outcome. We additionally present two previously unreported pediatric cases. Our review retrieved 19 cases of BBE/GBS, only 2 of which were originally and correctly diagnosed by the authors. The prevalence was higher in male subjects (ratio 3:1) and median age at diagnosis was 8 years. Anti-GQ1b were positive in 46% of the patients, while NCS were altered in 64%. Only 25% of the patients that underwent brain MRI showed abnormal findings. The incidence of BBE/GBS has been underrated in the past, mostly due to an underestimation of the PNS involvement. We therefore suggest to investigate all patients with a clinical picture suggestive of BBE/GBS through electroencephalogram, NCS, brain and spine MRI in order to promptly achieve the correct diagnosis.

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