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

dc.contributor.authorMartins, M
dc.contributor.authorQuintas, S
dc.contributor.authorCoelho, J
dc.contributor.authorSantos, T
dc.contributor.authorLevy, A
dc.date.accessioned2019-12-06T16:06:47Z
dc.date.available2019-12-06T16:06:47Z
dc.date.issued2019
dc.description.abstractWe 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.pt_PT
dc.description.versioninfo:eu-repo/semantics/publishedVersionpt_PT
dc.identifier.citationMult Scler Relat Disord, 37, 101457 2019 Oct 18[Online ahead of print]pt_PT
dc.identifier.doi10.1016/j.msard.2019.101457pt_PT
dc.identifier.issn2211-0356
dc.identifier.urihttp://hdl.handle.net/10400.10/2339
dc.language.isoengpt_PT
dc.peerreviewedyespt_PT
dc.publisherElsevierpt_PT
dc.subjectSclerodermapt_PT
dc.subjectMigrainept_PT
dc.titleExtensive Linear Scleroderma en Coup De Sabre With Exertion-Induced Hemiplegic Migrainept_PT
dc.typejournal article
dspace.entity.typePublication
oaire.citation.conferencePlaceAmsterdampt_PT
oaire.citation.titleMultiple Sclerosis and Related Disorderspt_PT
rcaap.rightsclosedAccesspt_PT
rcaap.typearticlept_PT

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