Cerebrofacial arteriovenous metameric syndrome (CAMS): clinical features

Written and reviewed by Dr Ibrahim Imam, Consultant Neurologist. Last reviewed .

Cerebrofacial arteriovenous metameric syndrome (CAMS): clinical features. This is an arterial or venous malformation that affects the brain and the face.

Definition

  • This is an arterial or venous malformation that affects the brain and the face
  • It results from the abnormal development of the neural crest or adjacent mesoderm
  • It manifests with a transverse (metameric) cerebrofacial pattern

Ophthalmic features

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Bleeding features

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Neurological features

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Differential diagnosis: Sturge Weber syndrome (SWS)

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Differential diagnosis: others

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Synonyms

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References

  1. Agid R, Terbrugge KG. Cerebrofacial venous metameric syndrome 2 plus 3: facial and cerebral manifestations. Interv Neuroradiol 2007; 13:55-58.
  2. Ng JC, Appuhamy C, Lee W. Cerebrofacial arteriovenous metameric syndrome with hypopituitarism: a rare association. BMJ Case Rep 2018; pii: bcr-2017-222708.
  3. Jiarakongmun P, Alvarez A, Rodesch G, Lasjaunias P. Clinical course and angioarchitecture of cerebrofacial arteriovenous metameric syndromes. Three demonstrative cases and literature review. Interv Neuroradiol 2002; 8:251-264.
  4. Khodeiry MM, Khodeiry MM, Mendoza-Santiesteban CE. Retinal and intracranial arteriovenous malformations in Wyburn-Mason syndrome. Can J Ophthalmol 2023; 58:e162-e163.
  5. Vucic D, Kalezic T, Kostic A, Stojkovic M, Risimic D, Stankovic B. Duane type I retraction syndrome associated with Wyburn-Mason syndrome. Ophthalmic Genet 2013; 34:61-64. 
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