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Case of the Week

Section Editors: Sapna Rawal,1 Juan Pablo Cruz2
1University of Toronto, Toronto, Ontario, Canada
2Pontificia Universidad Catolica de Chile, Santiago, Chile

Submit a Case Previous Cases ASPNR Pediatric Cases

August 22, 2019
  • Description
  • Legends
  • Diagnosis
  • Brain Teaser
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Parry Romberg Syndrome (PRS)

  • Background
    • Parry Romberg syndrome (PRS) is a rare condition characterized by insidious, but progressive, varying degrees of facial hemiatrophy that subsequently culminates in a “burned out” phase without further progression.
    • The underlying pathogenesis remains poorly understood. The clinical response to immunosuppressive therapy supports an immunologic-mediated process as the most accepted etiology. 
  • Clinical Presentation
    • More commonly affects females in the first 2 decades of life. Progressive facial atrophy (predilection for left-sided involvement) is typical. Neurologic symptoms occur in 15%–20%, with ipsilateral headaches, seizures, and facial pain being most common.
    • Ophthalmologic symptoms due to enophthalmos, uveitis and retinal or optic nerve abnormalities account for symptoms in 10%–30% patients.
  • Key Diagnostic Features
    • Progressive hemifacial atrophy is the key diagnostic feature of PRS.
    • Atrophy of the scalp and calvarium, cerebral white matter FLAIR hyperintensities and microbleeds are useful diagnostic features of PRS.
    • The unilateral distribution of these abnormalities is an important clue to the diagnosis.
  • Differential Diagnosis
    • En coup de sabre (variant of linear scleroderma): Shows overlapping clinico-radiologic findings with PRS and is therefore considered a spectrum by many authors.
    • Rasmussen encephalitis: Presence of epilepsia partialis continua and absence of involvement of cutaneous structures help differentiate from PRS.
    • Hemifacial microsomia and Goldenhar Syndrome: Typically congenital and nonprogressive.​
  • Treatment 
    • Anticonvulsive therapy
    • Immunosuppressive therapy (corticosteroids, immunomodulators, and plasmapheresis)
    • Autologous fat grafting with adipose-derived stem cells (favorable cosmetic results)

Suggested Reading

  1. Wong M, Phillips CD, Hagiwara M, et al. Parry romberg syndrome: 7 cases and literature review. AJNR Am J Neuroradiol 2015;36:1355 –61, 10.3174/ajnr.A4297.
  2. Blitstein MK, Glenn, AT. MRI of cerebral microhemorrhages. AJR Am J Roentgenol 2007;189:720–25, 10.2214/AJR.07.2249.

Current Issue

American Journal of Neuroradiology: 40 (11)
American Journal of Neuroradiology
Vol. 40, Issue 11
1 Nov 2019
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