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- Title
Unilateral Neuroimaging Findings in Pediatric Craniofacial Scleroderma: Parry-Romberg Syndrome and En Coup de Sabre.
- Authors
Seese, Ronald R.; Glaser, Daniel; Furtado, Andre; Thakkar, Kavita; Torok, Kathryn S.
- Abstract
Objective: Parry-Romberg syndrome (PRS) and en coup de sabre (ECDS) are subtypes of craniofacial localized scleroderma. Systematic analyses of central nervous system imaging findings and their clinical associations in children are lacking. Here, we aim to characterize neuroimaging findings and associated neurological symptoms in these conditions. Methods: Neuroimaging and neurological symptoms of children evaluated at our institution with a diagnosis of PRS or ECDS were retrospectively reviewed. Laterality, location, stability, and number of lesion(s) were evaluated, as was the presence of susceptibility lesion(s) and contrast enhancement. History of seizures or headaches was noted. Results: From 2003 to 2019, 80 patients with PRS or ECDS were followed at our institution. Neuroimaging was completed in 73 and found to be abnormal in 25. In 12 (48%) of these 25 cases, headaches and/or seizures were present. In the vast majority of these cases (22/25, 88%), lesions were ipsilateral to skin findings. White matter was involved in 19 (76%) patients. MRI abnormalities preceded a rheumatological diagnosis in 7 (28%). Susceptibility lesions were noted in 11 (44%), and 8 (73%) of these patients endorsed a history of headaches. Most lesions were in the supratentorial compartment, did not enhance, and were stable at 1-year follow up imaging. Of those with progression, susceptibility findings were present at baseline. Conclusions: Neuroimaging findings in pediatric PRS and ECDS are often supratentorial, stable, unilateral, and ipsilateral to skin findings, and they can precede cutaneous findings.
- Subjects
NEUROLOGICAL disorders; SCLERODERMA (Disease); BRAIN imaging; SYMPTOMS; CENTRAL nervous system; SYNDROMES
- Publication
Journal of Child Neurology, 2020, Vol 35, Issue 11, p753
- ISSN
0883-0738
- Publication type
Article
- DOI
10.1177/0883073820931253