Proteus syndrome: Craniofacial and cerebral MRI

D. R. Delone, W. D. Brown, L. R. Gentry

Research output: Contribution to journalArticle

42 Scopus citations

Abstract

The Proteus syndrome is a rare hamartoneoplastic syndrome that may affect the brain, skull, and extracranial head and neck. We present a case with severe, characteristic findings. Brain abnormalities are not common in Proteus syndrome; when present, hemimegalencephaly and migrational disorders are typically seen, commonly with an associated seizure disorder. Maxillary and mandibular dysmorphism may occur, including unilateral condylar hyperplasia. Subcutaneous fatty, fibrous, lymphangiomatous masses commonly seen in this syndrome may involve the neck and face, leading to disfigurement and potential airway compromise.

Original languageEnglish (US)
Pages (from-to)840-843
Number of pages4
JournalNeuroradiology
Volume41
Issue number11
DOIs
StatePublished - Nov 1 1999

Keywords

  • Magnetic resonance imaging
  • Proteus syndrome

ASJC Scopus subject areas

  • Radiology Nuclear Medicine and imaging
  • Clinical Neurology
  • Cardiology and Cardiovascular Medicine

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    Delone, D. R., Brown, W. D., & Gentry, L. R. (1999). Proteus syndrome: Craniofacial and cerebral MRI. Neuroradiology, 41(11), 840-843. https://doi.org/10.1007/s002340050853