Cerebrofacial arteriovenous metameric syndrome (CAMS): clinical features

Evidence-based neurology checklist on cerebrofacial arteriovenous metameric syndrome (cams): clinical features: 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…

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

Bleeding features

Neurological features

Differential diagnosis: Sturge Weber syndrome (SWS)

Differential diagnosis: others

Synonyms

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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