Central nervous system involvement in progressive facial hemiatrophy: further evidence of chronic localised meningoencephalitis

  • K. Terstegge
  • W. Küster
  • S. Felber
  • H. Henkes
  • R. Felix
Conference paper


Progressive facial haemiatrophy is a sporadic disease that affects children and adolescents and leads to atrophy and derformity of one side of the face. It was recently suggested that a characteristic form of chronic localised meningoencephalitis may be the histological correlate of occasional central nervous system (CNS) involvement. We performed an analysed MRI CT and cerebrospinal fluid (CSF) examinations and ophthalmoscopy in 15 patients with CNS involvement, and present our preliminary data. The triad of corticomeningeal dysmorphia, white matter lesion and homolateral hemiatrophy represented a typical (but not always complete) morphological pattern. The cortical lesions were of variable size. Five patients had small areas of cortical calcification. In 2 patients MRI revealed regional parenchymal and meningeal contrast enhancement. Contrast enhancement, CSF findings and ophthalmoscopic findings support the hypothesis of an inflammatory disease of the CNS.

Key words

Progressive facial hemiatrophy Central nervous system Meningoencephalitis Magnetic resonance imaging 


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Copyright information

© Springer-Verlag Berlin Heidelberg 1995

Authors and Affiliations

  • K. Terstegge
    • 1
  • W. Küster
    • 2
  • S. Felber
    • 3
  • H. Henkes
    • 4
  • R. Felix
    • 1
  1. 1.Department of RadiologyUniversitätsklinikum Rudolf VirchowBerlinGermany
  2. 2.Department of DermatologyKlinikum der Phillips-UniversitätMarburgGermany
  3. 3.Institute of Magnetic ResonanceUniversitätsklinikumInnsbruckAustria
  4. 4.Institute of NeuroradiologyUniversitätsklinikenHomburg/SaarGermany

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