Extensive Linear Scleroderma en Coup De Sabre With Exertion-Induced Hemiplegic Migraine

Detalhes bibliográficos
Autor(a) principal: Martins, M
Data de Publicação: 2019
Outros Autores: Quintas, S, Coelho, J, Santos, T, Levy, A
Tipo de documento: Artigo
Idioma: eng
Título da fonte: Repositório Científico de Acesso Aberto de Portugal (Repositórios Cientìficos)
Texto Completo: http://hdl.handle.net/10400.10/2339
Resumo: We report the case of a 9-year-old girl with linear scleroderma en coup de sabre (LSCS) who developed progressive white matter involvement, presenting as intractable hemiplegic migraine-like attacks induced by exercise. After a period of severely aggressive course, clinical and radiological stabilization was achieved under immunosuppressant treatment. Intrathecal synthesis of IgG and lymphocytic pleocytosis provided indirect evidence of a chronic inflammatory process of the central nervous system. We discuss the possible immunopathogenic mechanisms responsible for the neurocutaneous involvement in LSCS, favouring the hypothesis of an autoimmune and inflammatory vasculopathy. The singular occurrence of hemiplegic migraine triggered by exertion add further insight to the currently unknown pathogenesis of scleroderma disorder. In addition, we highlight the importance of intensive immunosuppression approaches in selected cases, contrasting with the classic benign course of LCSC.
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spelling Extensive Linear Scleroderma en Coup De Sabre With Exertion-Induced Hemiplegic MigraineSclerodermaMigraineWe report the case of a 9-year-old girl with linear scleroderma en coup de sabre (LSCS) who developed progressive white matter involvement, presenting as intractable hemiplegic migraine-like attacks induced by exercise. After a period of severely aggressive course, clinical and radiological stabilization was achieved under immunosuppressant treatment. Intrathecal synthesis of IgG and lymphocytic pleocytosis provided indirect evidence of a chronic inflammatory process of the central nervous system. We discuss the possible immunopathogenic mechanisms responsible for the neurocutaneous involvement in LSCS, favouring the hypothesis of an autoimmune and inflammatory vasculopathy. The singular occurrence of hemiplegic migraine triggered by exertion add further insight to the currently unknown pathogenesis of scleroderma disorder. In addition, we highlight the importance of intensive immunosuppression approaches in selected cases, contrasting with the classic benign course of LCSC.ElsevierRepositório do Hospital Prof. Doutor Fernando FonsecaMartins, MQuintas, SCoelho, JSantos, TLevy, A2019-12-06T16:06:47Z2019-01-01T00:00:00Z2019-01-01T00:00:00Zinfo:eu-repo/semantics/publishedVersioninfo:eu-repo/semantics/articleapplication/pdfhttp://hdl.handle.net/10400.10/2339engMult Scler Relat Disord, 37, 101457 2019 Oct 18[Online ahead of print]2211-035610.1016/j.msard.2019.101457metadata only accessinfo:eu-repo/semantics/openAccessreponame:Repositório Científico de Acesso Aberto de Portugal (Repositórios Cientìficos)instname:Agência para a Sociedade do Conhecimento (UMIC) - FCT - Sociedade da Informaçãoinstacron:RCAAP2022-09-20T15:53:01ZPortal AgregadorONG
dc.title.none.fl_str_mv Extensive Linear Scleroderma en Coup De Sabre With Exertion-Induced Hemiplegic Migraine
title Extensive Linear Scleroderma en Coup De Sabre With Exertion-Induced Hemiplegic Migraine
spellingShingle Extensive Linear Scleroderma en Coup De Sabre With Exertion-Induced Hemiplegic Migraine
Martins, M
Scleroderma
Migraine
title_short Extensive Linear Scleroderma en Coup De Sabre With Exertion-Induced Hemiplegic Migraine
title_full Extensive Linear Scleroderma en Coup De Sabre With Exertion-Induced Hemiplegic Migraine
title_fullStr Extensive Linear Scleroderma en Coup De Sabre With Exertion-Induced Hemiplegic Migraine
title_full_unstemmed Extensive Linear Scleroderma en Coup De Sabre With Exertion-Induced Hemiplegic Migraine
title_sort Extensive Linear Scleroderma en Coup De Sabre With Exertion-Induced Hemiplegic Migraine
author Martins, M
author_facet Martins, M
Quintas, S
Coelho, J
Santos, T
Levy, A
author_role author
author2 Quintas, S
Coelho, J
Santos, T
Levy, A
author2_role author
author
author
author
dc.contributor.none.fl_str_mv Repositório do Hospital Prof. Doutor Fernando Fonseca
dc.contributor.author.fl_str_mv Martins, M
Quintas, S
Coelho, J
Santos, T
Levy, A
dc.subject.por.fl_str_mv Scleroderma
Migraine
topic Scleroderma
Migraine
description We report the case of a 9-year-old girl with linear scleroderma en coup de sabre (LSCS) who developed progressive white matter involvement, presenting as intractable hemiplegic migraine-like attacks induced by exercise. After a period of severely aggressive course, clinical and radiological stabilization was achieved under immunosuppressant treatment. Intrathecal synthesis of IgG and lymphocytic pleocytosis provided indirect evidence of a chronic inflammatory process of the central nervous system. We discuss the possible immunopathogenic mechanisms responsible for the neurocutaneous involvement in LSCS, favouring the hypothesis of an autoimmune and inflammatory vasculopathy. The singular occurrence of hemiplegic migraine triggered by exertion add further insight to the currently unknown pathogenesis of scleroderma disorder. In addition, we highlight the importance of intensive immunosuppression approaches in selected cases, contrasting with the classic benign course of LCSC.
publishDate 2019
dc.date.none.fl_str_mv 2019-12-06T16:06:47Z
2019-01-01T00:00:00Z
2019-01-01T00:00:00Z
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 http://hdl.handle.net/10400.10/2339
url http://hdl.handle.net/10400.10/2339
dc.language.iso.fl_str_mv eng
language eng
dc.relation.none.fl_str_mv Mult Scler Relat Disord, 37, 101457 2019 Oct 18[Online ahead of print]
2211-0356
10.1016/j.msard.2019.101457
dc.rights.driver.fl_str_mv metadata only access
info:eu-repo/semantics/openAccess
rights_invalid_str_mv metadata only access
eu_rights_str_mv openAccess
dc.format.none.fl_str_mv application/pdf
dc.publisher.none.fl_str_mv Elsevier
publisher.none.fl_str_mv Elsevier
dc.source.none.fl_str_mv reponame:Repositório Científico de Acesso Aberto de Portugal (Repositórios Cientìficos)
instname:Agência para a Sociedade do Conhecimento (UMIC) - FCT - Sociedade da Informação
instacron:RCAAP
instname_str Agência para a Sociedade do Conhecimento (UMIC) - FCT - Sociedade da Informação
instacron_str RCAAP
institution RCAAP
reponame_str Repositório Científico de Acesso Aberto de Portugal (Repositórios Cientìficos)
collection Repositório Científico de Acesso Aberto de Portugal (Repositórios Cientìficos)
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repository.mail.fl_str_mv
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