, Volume 57, Issue 1, pp 21–34 | Cite as

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

  • Derrick A. Doolittle
  • Vance T. LehmanEmail author
  • Kara M. Schwartz
  • Lily C. Wong-Kisiel
  • Julia S. Lehman
  • Megha M. Tollefson
Diagnostic Neuroradiology



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.


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.


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.


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.


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



Central nervous system


Computed tomography


Computed tomographic angiography


En coup de sabre




Magnetic resonance angiography


Magnetic resonance imaging


Parry–Romberg syndrome


Ethical Standards and Patient Consent

We declare all human and animal studies have been approved by the Institutional Review Board and have therefore been performed in accordance with the ethical standards laid down in the 1964 Declaration of Helsinki and its later amendments. We declare that all patients gave informed consent prior to inclusion in this study.

Conflict of interest

We declare that we have no conflict of interest.


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Copyright information

© Springer-Verlag Berlin Heidelberg 2014

Authors and Affiliations

  • Derrick A. Doolittle
    • 1
  • Vance T. Lehman
    • 1
    Email author
  • Kara M. Schwartz
    • 1
  • Lily C. Wong-Kisiel
    • 4
  • Julia S. Lehman
    • 2
    • 3
  • Megha M. Tollefson
    • 5
  1. 1.Department of RadiologyMayo ClinicRochesterUSA
  2. 2.Department of Clinical Dermatology, Division of Dermatopathology and Cutaneous ImmunologyMayo ClinicRochesterUSA
  3. 3.Department of Laboratory Medicine and Pathology, Division of Anatomic PathologyMayo ClinicRochesterUSA
  4. 4.Department of NeurologyMayo ClinicRochesterUSA
  5. 5.Department of DermatologyMayo ClinicRochesterUSA

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