Pharyngeal-cervical-brachial variant of Guillain-Barré syndrome: a rare cause of acute bulbar dysfunction in children

Detalhes bibliográficos
Autor(a) principal: Coelho, Joana
Data de Publicação: 2014
Outros Autores: Azevedo, Sara, Furtado, Fátima, Abecasis, Francisco, Quintas, Sofia, Levy, António
Tipo de documento: Artigo
Idioma: eng
Título da fonte: Scientia Medica (Porto Alegre. Online)
Texto Completo: https://revistaseletronicas.pucrs.br/scientiamedica/article/view/18310
Resumo: AIMS: To report a case of pharyngeal-cervical-brachial variant of Guillain-Barré syndrome, which is characterized by rapidly progressive bulbar palsy with upper limb, neck and oropharyngeal involvement. It is a rare disorder in childhood and most cases have been described in adolescents.CASE DESCRIPTION: A seven year-old-boy presented with dysarthria, hoarseness, dysphagia, facial diplegia and bilateral progressive upper limb weakness. These symptoms started two weeks after a gastrointestinal infection. Nerve conduction studies were compatible with an acute demyelinating polyneuropathy in the upper extremities. Anti-ganglioside antibodies in the serum (anti-GT1a, GD1a, GQ1b) were positive and Campylobacter jejuni was isolated from stools. The patient was treated with intravenous immunoglobulin and needed ventilatory support during the first 12 days of admission. He was discharged at day 15 showing improvement of his neurological deficits. He fully recovered after eleven months of follow-up.CONCLUSIONS: Although pharyngeal-cervical-brachial variant of Guillain-Barré syndrome is uncommon in children, it should be considered in a child with acute bulbar dysfunction because a timely diagnosis allows the early institution of therapeutic measures that can be lifesaving.
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spelling Pharyngeal-cervical-brachial variant of Guillain-Barré syndrome: a rare cause of acute bulbar dysfunction in childrenPharyngeal-cervical-brachial variant of Guillain-Barré syndrome: a rare cause of acute bulbar dysfunction in childrenBULBAR PALSYPROGRESSIVECHILDHOODGUILLAIN-BARRE SYNDROMEPARALISIA BULBAR PROGRESSIVACRIANÇASÍNDROME DE GUILLAIN- BARRÉAIMS: To report a case of pharyngeal-cervical-brachial variant of Guillain-Barré syndrome, which is characterized by rapidly progressive bulbar palsy with upper limb, neck and oropharyngeal involvement. It is a rare disorder in childhood and most cases have been described in adolescents.CASE DESCRIPTION: A seven year-old-boy presented with dysarthria, hoarseness, dysphagia, facial diplegia and bilateral progressive upper limb weakness. These symptoms started two weeks after a gastrointestinal infection. Nerve conduction studies were compatible with an acute demyelinating polyneuropathy in the upper extremities. Anti-ganglioside antibodies in the serum (anti-GT1a, GD1a, GQ1b) were positive and Campylobacter jejuni was isolated from stools. The patient was treated with intravenous immunoglobulin and needed ventilatory support during the first 12 days of admission. He was discharged at day 15 showing improvement of his neurological deficits. He fully recovered after eleven months of follow-up.CONCLUSIONS: Although pharyngeal-cervical-brachial variant of Guillain-Barré syndrome is uncommon in children, it should be considered in a child with acute bulbar dysfunction because a timely diagnosis allows the early institution of therapeutic measures that can be lifesaving.***Variante faringo-cérvico-braquial da síndrome de Guillain-Barré: uma causa rara de disfunção bulbar aguda em crianças*** OBJETIVOS: Relatar um caso da variante faringo-cervico-braquial da síndrome de Guillain-Barré, que se caracteriza por paralisia bulbar rapidamente progressiva com envolvimento dos membros superiores, pescoço e região orofaríngea. É um diagnóstico raro na criança, ocorrendo a maioria dos casos em adolescentes.DESCRIÇÃO DO CASO: Um menino de sete anos de idade iniciou com queixas de disartria, disfonia, disfagia, diplegia facial e fraqueza muscular progressiva dos membros superiores. Estes sintomas surgiram duas semanas após uma infeção gastrointestinal. Os estudos eletrofisiológicos foram compatíveis com polineuropatia aguda desmielinizante nos membros superiores. Os anticorpos anti-gangliosídeo no plasma (anti-GT1a, GD1a, GQ1b) foram positivos e Campylobacter jejuni foi isolado nas fezes. O paciente foi tratado com imunoglobulina endovenosa e necessitou de suporte ventilatório durante os primeiros 12 dias. Teve alta no 15º dia com melhora dos sintomas neurológicos. Recuperou-se totalmente após 11 meses de seguimento.CONCLUSÕES: Apesar da variante faringo-cervico-braquial ser pouco frequente em idade pediátrica, é um diagnóstico que deve ser considerado perante uma criança com disfunção bulbar aguda, pois a identificação precoce permite instituir rapidamente medidas terapêuticas que podem evitar a morte.Editora da PUCRS - ediPUCRS2014-11-14info:eu-repo/semantics/articleinfo:eu-repo/semantics/publishedVersionRelato de caso raro ou de especial interesseapplication/pdfhttps://revistaseletronicas.pucrs.br/scientiamedica/article/view/1831010.15448/1980-6108.2014.4.18310Scientia Medica; Vol. 24 No. 4 (2014); 381-384Scientia Medica; v. 24 n. 4 (2014); 381-3841980-61081806-556210.15448/1980-6108.2014.4reponame:Scientia Medica (Porto Alegre. Online)instname:Pontifícia Universidade Católica do Rio Grande do Sul (PUCRS)instacron:PUC_RSenghttps://revistaseletronicas.pucrs.br/scientiamedica/article/view/18310/12343Coelho, JoanaAzevedo, SaraFurtado, FátimaAbecasis, FranciscoQuintas, SofiaLevy, Antónioinfo:eu-repo/semantics/openAccess2018-08-09T03:09:16Zoai:ojs.revistaseletronicas.pucrs.br:article/18310Revistahttps://revistaseletronicas.pucrs.br/scientiamedica/PUBhttps://revistaseletronicas.pucrs.br/scientiamedica/oaiscientiamedica@pucrs.br || editora.periodicos@pucrs.br1980-61081806-5562opendoar:2018-08-09T03:09:16Scientia Medica (Porto Alegre. Online) - Pontifícia Universidade Católica do Rio Grande do Sul (PUCRS)false
dc.title.none.fl_str_mv Pharyngeal-cervical-brachial variant of Guillain-Barré syndrome: a rare cause of acute bulbar dysfunction in children
Pharyngeal-cervical-brachial variant of Guillain-Barré syndrome: a rare cause of acute bulbar dysfunction in children
title Pharyngeal-cervical-brachial variant of Guillain-Barré syndrome: a rare cause of acute bulbar dysfunction in children
spellingShingle Pharyngeal-cervical-brachial variant of Guillain-Barré syndrome: a rare cause of acute bulbar dysfunction in children
Coelho, Joana
BULBAR PALSY
PROGRESSIVE
CHILDHOOD
GUILLAIN-BARRE SYNDROME
PARALISIA BULBAR PROGRESSIVA
CRIANÇA
SÍNDROME DE GUILLAIN- BARRÉ
title_short Pharyngeal-cervical-brachial variant of Guillain-Barré syndrome: a rare cause of acute bulbar dysfunction in children
title_full Pharyngeal-cervical-brachial variant of Guillain-Barré syndrome: a rare cause of acute bulbar dysfunction in children
title_fullStr Pharyngeal-cervical-brachial variant of Guillain-Barré syndrome: a rare cause of acute bulbar dysfunction in children
title_full_unstemmed Pharyngeal-cervical-brachial variant of Guillain-Barré syndrome: a rare cause of acute bulbar dysfunction in children
title_sort Pharyngeal-cervical-brachial variant of Guillain-Barré syndrome: a rare cause of acute bulbar dysfunction in children
author Coelho, Joana
author_facet Coelho, Joana
Azevedo, Sara
Furtado, Fátima
Abecasis, Francisco
Quintas, Sofia
Levy, António
author_role author
author2 Azevedo, Sara
Furtado, Fátima
Abecasis, Francisco
Quintas, Sofia
Levy, António
author2_role author
author
author
author
author
dc.contributor.author.fl_str_mv Coelho, Joana
Azevedo, Sara
Furtado, Fátima
Abecasis, Francisco
Quintas, Sofia
Levy, António
dc.subject.por.fl_str_mv BULBAR PALSY
PROGRESSIVE
CHILDHOOD
GUILLAIN-BARRE SYNDROME
PARALISIA BULBAR PROGRESSIVA
CRIANÇA
SÍNDROME DE GUILLAIN- BARRÉ
topic BULBAR PALSY
PROGRESSIVE
CHILDHOOD
GUILLAIN-BARRE SYNDROME
PARALISIA BULBAR PROGRESSIVA
CRIANÇA
SÍNDROME DE GUILLAIN- BARRÉ
description AIMS: To report a case of pharyngeal-cervical-brachial variant of Guillain-Barré syndrome, which is characterized by rapidly progressive bulbar palsy with upper limb, neck and oropharyngeal involvement. It is a rare disorder in childhood and most cases have been described in adolescents.CASE DESCRIPTION: A seven year-old-boy presented with dysarthria, hoarseness, dysphagia, facial diplegia and bilateral progressive upper limb weakness. These symptoms started two weeks after a gastrointestinal infection. Nerve conduction studies were compatible with an acute demyelinating polyneuropathy in the upper extremities. Anti-ganglioside antibodies in the serum (anti-GT1a, GD1a, GQ1b) were positive and Campylobacter jejuni was isolated from stools. The patient was treated with intravenous immunoglobulin and needed ventilatory support during the first 12 days of admission. He was discharged at day 15 showing improvement of his neurological deficits. He fully recovered after eleven months of follow-up.CONCLUSIONS: Although pharyngeal-cervical-brachial variant of Guillain-Barré syndrome is uncommon in children, it should be considered in a child with acute bulbar dysfunction because a timely diagnosis allows the early institution of therapeutic measures that can be lifesaving.
publishDate 2014
dc.date.none.fl_str_mv 2014-11-14
dc.type.driver.fl_str_mv info:eu-repo/semantics/article
info:eu-repo/semantics/publishedVersion
Relato de caso raro ou de especial interesse
format article
status_str publishedVersion
dc.identifier.uri.fl_str_mv https://revistaseletronicas.pucrs.br/scientiamedica/article/view/18310
10.15448/1980-6108.2014.4.18310
url https://revistaseletronicas.pucrs.br/scientiamedica/article/view/18310
identifier_str_mv 10.15448/1980-6108.2014.4.18310
dc.language.iso.fl_str_mv eng
language eng
dc.relation.none.fl_str_mv https://revistaseletronicas.pucrs.br/scientiamedica/article/view/18310/12343
dc.rights.driver.fl_str_mv info:eu-repo/semantics/openAccess
eu_rights_str_mv openAccess
dc.format.none.fl_str_mv application/pdf
dc.publisher.none.fl_str_mv Editora da PUCRS - ediPUCRS
publisher.none.fl_str_mv Editora da PUCRS - ediPUCRS
dc.source.none.fl_str_mv Scientia Medica; Vol. 24 No. 4 (2014); 381-384
Scientia Medica; v. 24 n. 4 (2014); 381-384
1980-6108
1806-5562
10.15448/1980-6108.2014.4
reponame:Scientia Medica (Porto Alegre. Online)
instname:Pontifícia Universidade Católica do Rio Grande do Sul (PUCRS)
instacron:PUC_RS
instname_str Pontifícia Universidade Católica do Rio Grande do Sul (PUCRS)
instacron_str PUC_RS
institution PUC_RS
reponame_str Scientia Medica (Porto Alegre. Online)
collection Scientia Medica (Porto Alegre. Online)
repository.name.fl_str_mv Scientia Medica (Porto Alegre. Online) - Pontifícia Universidade Católica do Rio Grande do Sul (PUCRS)
repository.mail.fl_str_mv scientiamedica@pucrs.br || editora.periodicos@pucrs.br
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