Proteus syndrome: Craniofacial and cerebral MRI

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

Research output: Contribution to journalArticle

  • 38 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.

LanguageEnglish (US)
Pages840-843
Number of pages4
JournalNeuroradiology
Volume41
Issue number11
DOIs
StatePublished - Nov 1999
Externally publishedYes

Fingerprint

Proteus Syndrome
Neck
Brain
Skull
Hyperplasia
Epilepsy
Head

Keywords

  • Magnetic resonance imaging
  • Proteus syndrome

ASJC Scopus subject areas

  • Clinical Neurology
  • Radiology Nuclear Medicine and imaging
  • Radiological and Ultrasound Technology

Cite this

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

Proteus syndrome : Craniofacial and cerebral MRI. / Delone, D. R.; Brown, W. D.; Gentry, L. R.

In: Neuroradiology, Vol. 41, No. 11, 11.1999, p. 840-843.

Research output: Contribution to journalArticle

Delone, DR, Brown, WD & Gentry, LR 1999, 'Proteus syndrome: Craniofacial and cerebral MRI' Neuroradiology, vol. 41, no. 11, pp. 840-843. https://doi.org/10.1007/s002340050853
Delone, D. R. ; Brown, W. D. ; Gentry, L. R. / Proteus syndrome : Craniofacial and cerebral MRI. In: Neuroradiology. 1999 ; Vol. 41, No. 11. pp. 840-843.
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