Recurrent Painful Ophthalmoplegic Neuropathy: A Diagnostic Challenge in the Pediatric Age
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Publication Date: | 2024 |
Other Authors: | , , , , |
Format: | Article |
Language: | por |
Source: | Repositórios Científicos de Acesso Aberto de Portugal (RCAAP) |
Download full: | https://doi.org/10.46531/sinapse/CC/230059/2024 |
Summary: | Recurrent painful ophthalmoplegic neuropathy (RPON) is a rare clinical entity, whose pathophysiology is not well understood. We present the case of a girl who, at 14 months of age, suddenly started right eyelid ptosis and incomplete paresis of the ipsilateral oculomotor nerve. Magnetic resonance imaging revealed a thickening of the emergence of that nerve. She was treated with corticosteroids and, over the years, presented multiple similar episodes, concluding with the diagnosis of RPON. The second case involves a 17-year-old girl with a history of episodic migraine and asymptomatic SARS-CoV-2 infection, also with right palpebral ptosis, with rapid progression to incomplete paresis of the ipsilateral oculomotor nerve. Ocular myasthenia gravis, a cranial neuralgia associated with COVID-19, and RPON were considered as hypotheses, with no recurrences to date. RPON recognition is difficult. This situation may contribute to its underestimation of prevalence, particularly in children. |
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Recurrent Painful Ophthalmoplegic Neuropathy: A Diagnostic Challenge in the Pediatric AgeNeuropatia Oftalmoplégica Dolorosa Recorrente: Um Desafio Diagnóstico na Idade PediátricaChildOphthalmoplegia/diagnosisOphthalmoplegic Migraine/ diagnosisOphthalmoplegic Migraine/ drug therapyCriançaOftalmoplegia/diagnósticoEnxaqueca Oftalmoplégica/ diagnósticoEnxaqueca Oftalmoplégica/ tratamento farmacológicoRecurrent painful ophthalmoplegic neuropathy (RPON) is a rare clinical entity, whose pathophysiology is not well understood. We present the case of a girl who, at 14 months of age, suddenly started right eyelid ptosis and incomplete paresis of the ipsilateral oculomotor nerve. Magnetic resonance imaging revealed a thickening of the emergence of that nerve. She was treated with corticosteroids and, over the years, presented multiple similar episodes, concluding with the diagnosis of RPON. The second case involves a 17-year-old girl with a history of episodic migraine and asymptomatic SARS-CoV-2 infection, also with right palpebral ptosis, with rapid progression to incomplete paresis of the ipsilateral oculomotor nerve. Ocular myasthenia gravis, a cranial neuralgia associated with COVID-19, and RPON were considered as hypotheses, with no recurrences to date. RPON recognition is difficult. This situation may contribute to its underestimation of prevalence, particularly in children.A neuropatia oftalmoplégica dolorosa recorrente (NODR) é uma entidade clínica rara, cuja fisiopatologia não está bem esclarecida. Apresentamos o caso de uma menina que, aos 14 meses, iniciou subitamente ptose palpebral direita e parésia incompleta do nervo oculomotor ipsilateral. A ressonância magnética revelou um espessamento da emergência do referido nervo. Cumpriu tratamento com corticosteróide e, ao longo dos anos, apresentou múltiplos episódios semelhantes, concluindo-se pelo diagnóstico de NDOR. O segundo caso envolve uma adolescente de 17 anos, com antecedentes de enxaqueca episódica e infeção assintomática a SARS-CoV-2, também com ptose palpebral direita, com evolução rápida para parésia incompleta do nervo oculomotor ipsilateral. Consideraram-se a miastenia gravis ocular, uma nevralgia craniana associada à COVID-19 e também a NDOR como hipóteses, não tendo havido recidivas, até ao momento. O reconhecimento da NDOR é difícil. Esta situação pode contribuir para que a sua prevalência esteja subestimada, particularmente na idade pediátrica.Portuguese Society of Neurology2024-04-04info:eu-repo/semantics/publishedVersioninfo:eu-repo/semantics/articleapplication/pdfhttps://doi.org/10.46531/sinapse/CC/230059/2024https://doi.org/10.46531/sinapse/CC/230059/2024Sinapse; Vol. 24 No. 1 (2024): January-March; 33-38Sinapse; Vol. 24 N.º 1 (2024): Janeiro-Março; 33-382184-42401645-281Xreponame:Repositórios Científicos de Acesso Aberto de Portugal (RCAAP)instname:FCCN, serviços digitais da FCT – Fundação para a Ciência e a Tecnologiainstacron:RCAAPporhttps://sinapse.pt/index.php/journal/article/view/47https://sinapse.pt/index.php/journal/article/view/47/43Copyright (c) 2024 Margarida Camacho Sampaio, Henrique Coimbra Queirós, Filipe Palavra, Rui Pedro Pais, Catarina Paiva, Carmen Costainfo:eu-repo/semantics/openAccessCamacho Sampaio, MargaridaCoimbra Queirós, HenriquePalavra, FilipePais, Rui PedroPaiva, CatarinaCosta, Carmen2024-05-11T11:10:22Zoai:sinapse.pt:article/47Portal AgregadorONGhttps://www.rcaap.pt/oai/openaireinfo@rcaap.ptopendoar:https://opendoar.ac.uk/repository/71602025-05-28T13:33:56.414760Repositórios Científicos de Acesso Aberto de Portugal (RCAAP) - FCCN, serviços digitais da FCT – Fundação para a Ciência e a Tecnologiafalse |
dc.title.none.fl_str_mv |
Recurrent Painful Ophthalmoplegic Neuropathy: A Diagnostic Challenge in the Pediatric Age Neuropatia Oftalmoplégica Dolorosa Recorrente: Um Desafio Diagnóstico na Idade Pediátrica |
title |
Recurrent Painful Ophthalmoplegic Neuropathy: A Diagnostic Challenge in the Pediatric Age |
spellingShingle |
Recurrent Painful Ophthalmoplegic Neuropathy: A Diagnostic Challenge in the Pediatric Age Camacho Sampaio, Margarida Child Ophthalmoplegia/diagnosis Ophthalmoplegic Migraine/ diagnosis Ophthalmoplegic Migraine/ drug therapy Criança Oftalmoplegia/diagnóstico Enxaqueca Oftalmoplégica/ diagnóstico Enxaqueca Oftalmoplégica/ tratamento farmacológico |
title_short |
Recurrent Painful Ophthalmoplegic Neuropathy: A Diagnostic Challenge in the Pediatric Age |
title_full |
Recurrent Painful Ophthalmoplegic Neuropathy: A Diagnostic Challenge in the Pediatric Age |
title_fullStr |
Recurrent Painful Ophthalmoplegic Neuropathy: A Diagnostic Challenge in the Pediatric Age |
title_full_unstemmed |
Recurrent Painful Ophthalmoplegic Neuropathy: A Diagnostic Challenge in the Pediatric Age |
title_sort |
Recurrent Painful Ophthalmoplegic Neuropathy: A Diagnostic Challenge in the Pediatric Age |
author |
Camacho Sampaio, Margarida |
author_facet |
Camacho Sampaio, Margarida Coimbra Queirós, Henrique Palavra, Filipe Pais, Rui Pedro Paiva, Catarina Costa, Carmen |
author_role |
author |
author2 |
Coimbra Queirós, Henrique Palavra, Filipe Pais, Rui Pedro Paiva, Catarina Costa, Carmen |
author2_role |
author author author author author |
dc.contributor.author.fl_str_mv |
Camacho Sampaio, Margarida Coimbra Queirós, Henrique Palavra, Filipe Pais, Rui Pedro Paiva, Catarina Costa, Carmen |
dc.subject.por.fl_str_mv |
Child Ophthalmoplegia/diagnosis Ophthalmoplegic Migraine/ diagnosis Ophthalmoplegic Migraine/ drug therapy Criança Oftalmoplegia/diagnóstico Enxaqueca Oftalmoplégica/ diagnóstico Enxaqueca Oftalmoplégica/ tratamento farmacológico |
topic |
Child Ophthalmoplegia/diagnosis Ophthalmoplegic Migraine/ diagnosis Ophthalmoplegic Migraine/ drug therapy Criança Oftalmoplegia/diagnóstico Enxaqueca Oftalmoplégica/ diagnóstico Enxaqueca Oftalmoplégica/ tratamento farmacológico |
description |
Recurrent painful ophthalmoplegic neuropathy (RPON) is a rare clinical entity, whose pathophysiology is not well understood. We present the case of a girl who, at 14 months of age, suddenly started right eyelid ptosis and incomplete paresis of the ipsilateral oculomotor nerve. Magnetic resonance imaging revealed a thickening of the emergence of that nerve. She was treated with corticosteroids and, over the years, presented multiple similar episodes, concluding with the diagnosis of RPON. The second case involves a 17-year-old girl with a history of episodic migraine and asymptomatic SARS-CoV-2 infection, also with right palpebral ptosis, with rapid progression to incomplete paresis of the ipsilateral oculomotor nerve. Ocular myasthenia gravis, a cranial neuralgia associated with COVID-19, and RPON were considered as hypotheses, with no recurrences to date. RPON recognition is difficult. This situation may contribute to its underestimation of prevalence, particularly in children. |
publishDate |
2024 |
dc.date.none.fl_str_mv |
2024-04-04 |
dc.type.status.fl_str_mv |
info:eu-repo/semantics/publishedVersion |
dc.type.driver.fl_str_mv |
info:eu-repo/semantics/article |
format |
article |
status_str |
publishedVersion |
dc.identifier.uri.fl_str_mv |
https://doi.org/10.46531/sinapse/CC/230059/2024 https://doi.org/10.46531/sinapse/CC/230059/2024 |
url |
https://doi.org/10.46531/sinapse/CC/230059/2024 |
dc.language.iso.fl_str_mv |
por |
language |
por |
dc.relation.none.fl_str_mv |
https://sinapse.pt/index.php/journal/article/view/47 https://sinapse.pt/index.php/journal/article/view/47/43 |
dc.rights.driver.fl_str_mv |
info:eu-repo/semantics/openAccess |
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openAccess |
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application/pdf |
dc.publisher.none.fl_str_mv |
Portuguese Society of Neurology |
publisher.none.fl_str_mv |
Portuguese Society of Neurology |
dc.source.none.fl_str_mv |
Sinapse; Vol. 24 No. 1 (2024): January-March; 33-38 Sinapse; Vol. 24 N.º 1 (2024): Janeiro-Março; 33-38 2184-4240 1645-281X reponame:Repositórios Científicos de Acesso Aberto de Portugal (RCAAP) instname:FCCN, serviços digitais da FCT – Fundação para a Ciência e a Tecnologia instacron:RCAAP |
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