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A significant proportion of children with morphea en coup de sabre and Parry-Romberg syndrome have neuroimaging findings. Pediatr Dermatol 2012;29(6):738-48

Date

10/31/2012

Pubmed ID

23106674

Pubmed Central ID

PMC3490222

DOI

10.1111/pde.12001

Scopus ID

2-s2.0-84868159458   70 Citations

Abstract

En coup de sabre (ECDS) and Parry-Romberg syndrome (PRS) are variants of linear morphea on the head and neck that can be associated with neurologic manifestations. Intracranial abnormalities on computed tomography (CT) and magnetic resonance imaging (MRI) are present in a significant proportion of individuals with these conditions. We describe 32 children from our institution with ECDS or PRS; neuroimaging was performed in 21 cases. We also review 51 additional cases from the literature. Nineteen percent of the children at our institution with ECDS or PRS had intracranial abnormalities on MRI, half of whom were asymptomatic. Hyperintensities on T2-weighted sequences were the most common finding, present in all children with intracranial abnormalities on MRI. Seizures (13%) and headaches (9%) were the most common neurologic symptom. Neurologic symptoms were not correlated with neuroimaging abnormalities, with two asymptomatic children having marked MRI findings and only two of nine symptomatic children having an abnormal MRI. Similarly the severity of the superficial disease did not predict neurologic involvement; a child with subtle skin involvement had striking MRI findings and seizures, whereas another with a bony defect had no brain parenchymal involvement. Neurologic symptoms and neuroimaging abnormalities are found in a surprisingly substantial percentage of children with ECDS and PRS. Early recognition of neurologic involvement is necessary because it affects treatment choices. Because clinical predictors of intracranial abnormalities are poor, strong consideration should be given to obtaining an MRI before treatment initiation to assist in management decisions and establish a baseline examination.

Author List

Chiu YE, Vora S, Kwon EK, Maheshwari M

Authors

Yvonne E. Chiu MD Vice Chair, Professor in the Dermatology department at Medical College of Wisconsin
Mohit Maheshwari MD Professor in the Radiology department at Medical College of Wisconsin




MESH terms used to index this publication - Major topics in bold

Adolescent
Atrophy
Brain Diseases
Child
Child, Preschool
Epilepsy
Facial Hemiatrophy
Female
Humans
Infant
Magnetic Resonance Imaging
Male
Neuroimaging
Retrospective Studies
Scleroderma, Localized
Tomography, X-Ray Computed