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Magnetic resonance imaging findings in children with Parry-Romberg syndrome and en coup de sabre.

Authors :
Knights H
Minas E
Khan F
Shaw L
Al Obaidi M
Mankad K
Eleftheriou D
Source :
Pediatric rheumatology online journal [Pediatr Rheumatol Online J] 2021 Mar 23; Vol. 19 (1), pp. 42. Date of Electronic Publication: 2021 Mar 23.
Publication Year :
2021

Abstract

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.<br />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.<br />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).<br />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.

Details

Language :
English
ISSN :
1546-0096
Volume :
19
Issue :
1
Database :
MEDLINE
Journal :
Pediatric rheumatology online journal
Publication Type :
Academic Journal
Accession number :
33757522
Full Text :
https://doi.org/10.1186/s12969-021-00512-6