Pharyngeal-cervical-brachial variant of Guillain-Barré syndrome: a rare cause of acute bulbar dysfunction in children
Autor(a) principal: | |
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Data de Publicação: | 2014 |
Outros Autores: | , , , , |
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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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 |
_version_ |
1809101750605447168 |