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Progressive facial hemiatrophy (parry-romberg syndrome)

  • Ignacio Pascual-Castroviejo
  • Domenico A. Restivo
  • Pietro Milone

Abstract

Progressive facial hemiatrophy (PFH), also known as Parry-Romberg or Romberg syndrome, is a sporadic, but not so rare disease (OMIM # 141300) characterised by progressive and self-limited shrinking and deformation of one side of the face, which involves different tissues, scar-like cutaneous changes, subcutaneous connective and fatty tissue’ atrophy, circumscribed osteoporosis, bone deformation accompanied usually by contralateral Jacksonian epilepsy, trigeminal neuralgia (and/or peripheral nerve dysfunction), and changes in the eyes and hair. Evidence of mendelian basis is lacking. Larner and Bennison (1993) reported discordance in a pair of monozygotic twins and Anderson et al. (2005) reported two first cousins with PFH, whose fathers were dizygotic twins and whose mothers were sisters.

Keywords

Trigeminal Neuralgia Dizygotic Twin Central Nervous System Lesion Pediatr Ophthalmol Strabismus Excerpta Medica Foundation 
These keywords were added by machine and not by the authors. This process is experimental and the keywords may be updated as the learning algorithm improves.

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Copyright information

© Springer-Verlag/Wien 2008

Authors and Affiliations

  • Ignacio Pascual-Castroviejo
    • 1
  • Domenico A. Restivo
    • 2
  • Pietro Milone
    • 3
  1. 1.Paediatric Neurology ServiceUniversity Hospital La Paz, University of MadridMadridSpain
  2. 2.Neurology Unit, Garibaldi HospitalCataniaItaly
  3. 3.Institute of RadiologyUniversity of CataniaCataniaItaly

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