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Magnetic resonance imaging findings in children with Parry-Romberg syndrome and en coup de sabre
BACKGROUND: The aim of this study was to: (i) describe the abnormalities seen on brain imaging in a group of children with en coup de sabre (EDCS) with/without Parry-Romberg syndrome (PRS); and (ii) identify clinical predictors of brain imaging abnormalities. METHODS: This was a single centre (Great...
Autores principales: | , , , , , , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
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BioMed Central
2021
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7986399/ https://www.ncbi.nlm.nih.gov/pubmed/33757522 http://dx.doi.org/10.1186/s12969-021-00512-6 |
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author | Knights, Harry Minas, Elizabeth Khan, Faraan Shaw, Lindsay Al Obaidi, Muthana Mankad, Kshitij Eleftheriou, Despina |
author_facet | Knights, Harry Minas, Elizabeth Khan, Faraan Shaw, Lindsay Al Obaidi, Muthana Mankad, Kshitij Eleftheriou, Despina |
author_sort | Knights, Harry |
collection | PubMed |
description | BACKGROUND: The aim of this study was to: (i) describe the abnormalities seen on brain imaging in a group of children with en coup de sabre (EDCS) with/without Parry-Romberg syndrome (PRS); and (ii) identify clinical predictors of brain imaging abnormalities. METHODS: This was a single centre (Great Ormond Street Hospital, London) retrospective case series of patients with ECDS/PRS seen from 2000 to 2018. We identified patients with cutaneous manifestations consistent with the clinical descriptions of ECDS/PRS. Presenting clinical, laboratory, and radiological brain findings are described. Results are expressed as medians and ranges or frequencies and percentages. Fisher’s exact test was used to identify clinical associations with magnetic resonance imaging (MRI) abnormalities. RESULTS: Fourteen patients were studied: 6 males and 8 females; median age 14 years (range 3–20). We observed neuroimaging abnormalities in 2/6 ECDS and 5/8 ECDS/PRS patients. White matter signal abnormality, dystrophic calcification, leptomeningeal enhancement, and sulcal crowding were the typical findings on brain imaging. A total of 50% of patients had no MRI abnormality despite some of these patients having neurological symptoms. The presence of seizures was significantly associated with ipsilateral enhanced white matter signalling on MRI (p < 0.05). CONCLUSIONS: In summary, we observed several distinct radiographic patterns associated with ECDS/PRS. Seizure disorder was strongly associated with the presence of ipsilateral enhanced white matter signalling. Improved neuroimaging techniques that combine morphological with functional imaging may improve the detection rate of brain involvement in children with ECDS/PRS in the future. SUPPLEMENTARY INFORMATION: The online version contains supplementary material available at 10.1186/s12969-021-00512-6. |
format | Online Article Text |
id | pubmed-7986399 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2021 |
publisher | BioMed Central |
record_format | MEDLINE/PubMed |
spelling | pubmed-79863992021-03-24 Magnetic resonance imaging findings in children with Parry-Romberg syndrome and en coup de sabre Knights, Harry Minas, Elizabeth Khan, Faraan Shaw, Lindsay Al Obaidi, Muthana Mankad, Kshitij Eleftheriou, Despina Pediatr Rheumatol Online J Research Article BACKGROUND: The aim of this study was to: (i) describe the abnormalities seen on brain imaging in a group of children with en coup de sabre (EDCS) with/without Parry-Romberg syndrome (PRS); and (ii) identify clinical predictors of brain imaging abnormalities. METHODS: This was a single centre (Great Ormond Street Hospital, London) retrospective case series of patients with ECDS/PRS seen from 2000 to 2018. We identified patients with cutaneous manifestations consistent with the clinical descriptions of ECDS/PRS. Presenting clinical, laboratory, and radiological brain findings are described. Results are expressed as medians and ranges or frequencies and percentages. Fisher’s exact test was used to identify clinical associations with magnetic resonance imaging (MRI) abnormalities. RESULTS: Fourteen patients were studied: 6 males and 8 females; median age 14 years (range 3–20). We observed neuroimaging abnormalities in 2/6 ECDS and 5/8 ECDS/PRS patients. White matter signal abnormality, dystrophic calcification, leptomeningeal enhancement, and sulcal crowding were the typical findings on brain imaging. A total of 50% of patients had no MRI abnormality despite some of these patients having neurological symptoms. The presence of seizures was significantly associated with ipsilateral enhanced white matter signalling on MRI (p < 0.05). CONCLUSIONS: In summary, we observed several distinct radiographic patterns associated with ECDS/PRS. Seizure disorder was strongly associated with the presence of ipsilateral enhanced white matter signalling. Improved neuroimaging techniques that combine morphological with functional imaging may improve the detection rate of brain involvement in children with ECDS/PRS in the future. SUPPLEMENTARY INFORMATION: The online version contains supplementary material available at 10.1186/s12969-021-00512-6. BioMed Central 2021-03-23 /pmc/articles/PMC7986399/ /pubmed/33757522 http://dx.doi.org/10.1186/s12969-021-00512-6 Text en © The Author(s) 2021 Open AccessThis article is licensed under a Creative Commons Attribution 4.0 International License, which permits use, sharing, adaptation, distribution and reproduction in any medium or format, as long as you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons licence, and indicate if changes were made. The images or other third party material in this article are included in the article's Creative Commons licence, unless indicated otherwise in a credit line to the material. If material is not included in the article's Creative Commons licence and your intended use is not permitted by statutory regulation or exceeds the permitted use, you will need to obtain permission directly from the copyright holder. To view a copy of this licence, visit http://creativecommons.org/licenses/by/4.0/. The Creative Commons Public Domain Dedication waiver (http://creativecommons.org/publicdomain/zero/1.0/) applies to the data made available in this article, unless otherwise stated in a credit line to the data. |
spellingShingle | Research Article Knights, Harry Minas, Elizabeth Khan, Faraan Shaw, Lindsay Al Obaidi, Muthana Mankad, Kshitij Eleftheriou, Despina Magnetic resonance imaging findings in children with Parry-Romberg syndrome and en coup de sabre |
title | Magnetic resonance imaging findings in children with Parry-Romberg syndrome and en coup de sabre |
title_full | Magnetic resonance imaging findings in children with Parry-Romberg syndrome and en coup de sabre |
title_fullStr | Magnetic resonance imaging findings in children with Parry-Romberg syndrome and en coup de sabre |
title_full_unstemmed | Magnetic resonance imaging findings in children with Parry-Romberg syndrome and en coup de sabre |
title_short | Magnetic resonance imaging findings in children with Parry-Romberg syndrome and en coup de sabre |
title_sort | magnetic resonance imaging findings in children with parry-romberg syndrome and en coup de sabre |
topic | Research Article |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7986399/ https://www.ncbi.nlm.nih.gov/pubmed/33757522 http://dx.doi.org/10.1186/s12969-021-00512-6 |
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