CNS imaging findings associated with Parry–Romberg syndrome and en coup de sabre: correlation to dermatologic and neurologic abnormalities

Derrick A. Doolittle, Vance T Lehman, Kara M. Schwartz, Lily Wong-Kisiel, Julia Lehman, Megha M Tollefson

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24 Citations (Scopus)

Abstract

Conclusions: In PRS and ECS, imaging findings often are bilateral and often do not progress, regardless of cutaneous disease activity. Findings are inconsistently associated with clinical abnormalities.

Methods: We retrospectively reviewed patients younger than 50 years at our institution over a 16-year interval who had clinical diagnosis of PRS and ECS by a skin or facial subspecialist. Two neuroradiologists evaluated available imaging and characterized CNS imaging findings.

Introduction: Parry–Romberg syndrome (PRS) and en coup de sabre (ECS) are variants of morphea. Although numerous findings on central nervous system (CNS) imaging of PRS and ECS have been reported, the spectrum and frequency of CNS imaging findings and relation to cutaneous and neurologic abnormalities have not been fully characterized.

Results: Eighty-eight patients with PRS or ECS were identified (62 women [70.4 %]; mean age 28.8 years). Of the 43 patients with CNS imaging, 19 (44 %) had abnormal findings. The only finding in 1 of these 19 patients was lateral ventricle asymmetry; of the other 18, findings were bilateral in 11 (61 %), ipsilateral to the side of facial involvement in 6 (33 %), and contralateral in 1 (6 %). Sixteen patients had serial imaging examinations over an average of 632 days; 13 (81 %) had stable imaging findings, and 3 (19 %) had change over time. Of six patients with progressive cutaneous findings, five (83 %) had stable imaging findings over time. Among the 23 patients with clinical neurologic abnormality and imaging, 12 (52 %) had abnormal imaging findings. All seven patients with seizures (100 %) had abnormal imaging studies.

Original languageEnglish (US)
Pages (from-to)21-34
Number of pages14
JournalNeuroradiology
Volume57
Issue number1
DOIs
StatePublished - 2014

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Nervous System Malformations
Central Nervous System
Skin Abnormalities
Localized Scleroderma
Skin
Lateral Ventricles
Skin Diseases
Seizures

Keywords

  • Cerebral atrophy
  • En coup de sabre
  • Encephalomalacia
  • Hemifacial atrophy
  • Parry–Romberg syndrome

ASJC Scopus subject areas

  • Radiology Nuclear Medicine and imaging
  • Clinical Neurology
  • Cardiology and Cardiovascular Medicine
  • Medicine(all)

Cite this

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title = "CNS imaging findings associated with Parry–Romberg syndrome and en coup de sabre: correlation to dermatologic and neurologic abnormalities",
abstract = "Conclusions: In PRS and ECS, imaging findings often are bilateral and often do not progress, regardless of cutaneous disease activity. Findings are inconsistently associated with clinical abnormalities.Methods: We retrospectively reviewed patients younger than 50 years at our institution over a 16-year interval who had clinical diagnosis of PRS and ECS by a skin or facial subspecialist. Two neuroradiologists evaluated available imaging and characterized CNS imaging findings.Introduction: Parry–Romberg syndrome (PRS) and en coup de sabre (ECS) are variants of morphea. Although numerous findings on central nervous system (CNS) imaging of PRS and ECS have been reported, the spectrum and frequency of CNS imaging findings and relation to cutaneous and neurologic abnormalities have not been fully characterized.Results: Eighty-eight patients with PRS or ECS were identified (62 women [70.4 {\%}]; mean age 28.8 years). Of the 43 patients with CNS imaging, 19 (44 {\%}) had abnormal findings. The only finding in 1 of these 19 patients was lateral ventricle asymmetry; of the other 18, findings were bilateral in 11 (61 {\%}), ipsilateral to the side of facial involvement in 6 (33 {\%}), and contralateral in 1 (6 {\%}). Sixteen patients had serial imaging examinations over an average of 632 days; 13 (81 {\%}) had stable imaging findings, and 3 (19 {\%}) had change over time. Of six patients with progressive cutaneous findings, five (83 {\%}) had stable imaging findings over time. Among the 23 patients with clinical neurologic abnormality and imaging, 12 (52 {\%}) had abnormal imaging findings. All seven patients with seizures (100 {\%}) had abnormal imaging studies.",
keywords = "Cerebral atrophy, En coup de sabre, Encephalomalacia, Hemifacial atrophy, Parry–Romberg syndrome",
author = "Doolittle, {Derrick A.} and Lehman, {Vance T} and Schwartz, {Kara M.} and Lily Wong-Kisiel and Julia Lehman and Tollefson, {Megha M}",
year = "2014",
doi = "10.1007/s00234-014-1448-6",
language = "English (US)",
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TY - JOUR

T1 - CNS imaging findings associated with Parry–Romberg syndrome and en coup de sabre

T2 - correlation to dermatologic and neurologic abnormalities

AU - Doolittle, Derrick A.

AU - Lehman, Vance T

AU - Schwartz, Kara M.

AU - Wong-Kisiel, Lily

AU - Lehman, Julia

AU - Tollefson, Megha M

PY - 2014

Y1 - 2014

N2 - Conclusions: In PRS and ECS, imaging findings often are bilateral and often do not progress, regardless of cutaneous disease activity. Findings are inconsistently associated with clinical abnormalities.Methods: We retrospectively reviewed patients younger than 50 years at our institution over a 16-year interval who had clinical diagnosis of PRS and ECS by a skin or facial subspecialist. Two neuroradiologists evaluated available imaging and characterized CNS imaging findings.Introduction: Parry–Romberg syndrome (PRS) and en coup de sabre (ECS) are variants of morphea. Although numerous findings on central nervous system (CNS) imaging of PRS and ECS have been reported, the spectrum and frequency of CNS imaging findings and relation to cutaneous and neurologic abnormalities have not been fully characterized.Results: Eighty-eight patients with PRS or ECS were identified (62 women [70.4 %]; mean age 28.8 years). Of the 43 patients with CNS imaging, 19 (44 %) had abnormal findings. The only finding in 1 of these 19 patients was lateral ventricle asymmetry; of the other 18, findings were bilateral in 11 (61 %), ipsilateral to the side of facial involvement in 6 (33 %), and contralateral in 1 (6 %). Sixteen patients had serial imaging examinations over an average of 632 days; 13 (81 %) had stable imaging findings, and 3 (19 %) had change over time. Of six patients with progressive cutaneous findings, five (83 %) had stable imaging findings over time. Among the 23 patients with clinical neurologic abnormality and imaging, 12 (52 %) had abnormal imaging findings. All seven patients with seizures (100 %) had abnormal imaging studies.

AB - Conclusions: In PRS and ECS, imaging findings often are bilateral and often do not progress, regardless of cutaneous disease activity. Findings are inconsistently associated with clinical abnormalities.Methods: We retrospectively reviewed patients younger than 50 years at our institution over a 16-year interval who had clinical diagnosis of PRS and ECS by a skin or facial subspecialist. Two neuroradiologists evaluated available imaging and characterized CNS imaging findings.Introduction: Parry–Romberg syndrome (PRS) and en coup de sabre (ECS) are variants of morphea. Although numerous findings on central nervous system (CNS) imaging of PRS and ECS have been reported, the spectrum and frequency of CNS imaging findings and relation to cutaneous and neurologic abnormalities have not been fully characterized.Results: Eighty-eight patients with PRS or ECS were identified (62 women [70.4 %]; mean age 28.8 years). Of the 43 patients with CNS imaging, 19 (44 %) had abnormal findings. The only finding in 1 of these 19 patients was lateral ventricle asymmetry; of the other 18, findings were bilateral in 11 (61 %), ipsilateral to the side of facial involvement in 6 (33 %), and contralateral in 1 (6 %). Sixteen patients had serial imaging examinations over an average of 632 days; 13 (81 %) had stable imaging findings, and 3 (19 %) had change over time. Of six patients with progressive cutaneous findings, five (83 %) had stable imaging findings over time. Among the 23 patients with clinical neurologic abnormality and imaging, 12 (52 %) had abnormal imaging findings. All seven patients with seizures (100 %) had abnormal imaging studies.

KW - Cerebral atrophy

KW - En coup de sabre

KW - Encephalomalacia

KW - Hemifacial atrophy

KW - Parry–Romberg syndrome

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JO - Neuroradiology

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