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Linear scleroderma en coup de sabre presenting with seizures

Scleroderma is a rare connective tissue disorder categorized into systemic sclerosis and localized scleroderma, also called morphea. Linear scleroderma of the scalp, also called en coup de sabre, is infrequently associated with neurologic symptoms. We describe a case of linear scleroderma en coup de...

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Detalles Bibliográficos
Autores principales: Nguyen, Kevin, Atty, Corrine, Ree, Alexander
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Elsevier 2020
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7484537/
https://www.ncbi.nlm.nih.gov/pubmed/32952756
http://dx.doi.org/10.1016/j.radcr.2020.08.011
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author Nguyen, Kevin
Atty, Corrine
Ree, Alexander
author_facet Nguyen, Kevin
Atty, Corrine
Ree, Alexander
author_sort Nguyen, Kevin
collection PubMed
description Scleroderma is a rare connective tissue disorder categorized into systemic sclerosis and localized scleroderma, also called morphea. Linear scleroderma of the scalp, also called en coup de sabre, is infrequently associated with neurologic symptoms. We describe a case of linear scleroderma en coup de sabre in a 28-year-old female presenting with seizures and characteristic cutaneous lesions. Imaging findings over a course of 7 years demonstrated waxing and waning signal changes. MR perfusion and spectroscopic imaging, demonstrating decreased cerebral blood volume, increased mean transit time, and decreased metabolites, was performed during a time of progressing radiological and clinical findings. Comparison with other reports in the literature supported several clinical and imaging findings that while not pathognomic, highly suggest the diagnosis of linear scleroderma en coup de sabre. Hyperintense signal on T2W magentic resonance imaging and contrast enhancement on computed tomography and magnetic resonance imaging have been the most commonly described imaging findings. To our knowledge, no previous description of spectroscopic or perfusion imaging of linear scleroderma en coup de sabre have been reported. It is our hope that this report may add MRS and magnetic resonance perfusion findings to a growing knowledge of this rare entity.
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spelling pubmed-74845372020-09-17 Linear scleroderma en coup de sabre presenting with seizures Nguyen, Kevin Atty, Corrine Ree, Alexander Radiol Case Rep Case Report Scleroderma is a rare connective tissue disorder categorized into systemic sclerosis and localized scleroderma, also called morphea. Linear scleroderma of the scalp, also called en coup de sabre, is infrequently associated with neurologic symptoms. We describe a case of linear scleroderma en coup de sabre in a 28-year-old female presenting with seizures and characteristic cutaneous lesions. Imaging findings over a course of 7 years demonstrated waxing and waning signal changes. MR perfusion and spectroscopic imaging, demonstrating decreased cerebral blood volume, increased mean transit time, and decreased metabolites, was performed during a time of progressing radiological and clinical findings. Comparison with other reports in the literature supported several clinical and imaging findings that while not pathognomic, highly suggest the diagnosis of linear scleroderma en coup de sabre. Hyperintense signal on T2W magentic resonance imaging and contrast enhancement on computed tomography and magnetic resonance imaging have been the most commonly described imaging findings. To our knowledge, no previous description of spectroscopic or perfusion imaging of linear scleroderma en coup de sabre have been reported. It is our hope that this report may add MRS and magnetic resonance perfusion findings to a growing knowledge of this rare entity. Elsevier 2020-09-03 /pmc/articles/PMC7484537/ /pubmed/32952756 http://dx.doi.org/10.1016/j.radcr.2020.08.011 Text en © 2020 The Authors. Published by Elsevier Inc. on behalf of University of Washington. http://creativecommons.org/licenses/by-nc-nd/4.0/ This is an open access article under the CC BY-NC-ND license (http://creativecommons.org/licenses/by-nc-nd/4.0/).
spellingShingle Case Report
Nguyen, Kevin
Atty, Corrine
Ree, Alexander
Linear scleroderma en coup de sabre presenting with seizures
title Linear scleroderma en coup de sabre presenting with seizures
title_full Linear scleroderma en coup de sabre presenting with seizures
title_fullStr Linear scleroderma en coup de sabre presenting with seizures
title_full_unstemmed Linear scleroderma en coup de sabre presenting with seizures
title_short Linear scleroderma en coup de sabre presenting with seizures
title_sort linear scleroderma en coup de sabre presenting with seizures
topic Case Report
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7484537/
https://www.ncbi.nlm.nih.gov/pubmed/32952756
http://dx.doi.org/10.1016/j.radcr.2020.08.011
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