Extensive linear scleroderma en coup de sabre with exertion-induced hemiplegic migraine.
En coup de sabre
Hemiplegic
Linear
Localized
Migraine
Scleroderma
Journal
Multiple sclerosis and related disorders
ISSN: 2211-0356
Titre abrégé: Mult Scler Relat Disord
Pays: Netherlands
ID NLM: 101580247
Informations de publication
Date de publication:
Jan 2020
Jan 2020
Historique:
received:
11
04
2019
revised:
29
09
2019
accepted:
18
10
2019
pubmed:
2
11
2019
medline:
20
11
2020
entrez:
1
11
2019
Statut:
ppublish
Résumé
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.
Identifiants
pubmed: 31670009
pii: S2211-0348(19)30446-8
doi: 10.1016/j.msard.2019.101457
pii:
doi:
Types de publication
Case Reports
Langues
eng
Sous-ensembles de citation
IM
Pagination
101457Informations de copyright
Copyright © 2019 Elsevier B.V. All rights reserved.
Déclaration de conflit d'intérêts
Declaration of Competing Interest The authors have no conflicts of interest to disclose.