Recurrent Painful Ophthalmoplegic Neuropathy: A Diagnostic Challenge in the Pediatric Age

Bibliographic Details
Main Author: Camacho Sampaio, Margarida
Publication Date: 2024
Other Authors: Coimbra Queirós, Henrique, Palavra, Filipe, Pais, Rui Pedro, Paiva, Catarina, Costa, Carmen
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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spelling 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
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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
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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
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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
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institution RCAAP
reponame_str Repositórios Científicos de Acesso Aberto de Portugal (RCAAP)
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