Teaching NeuroImages: An oculocerebral metameric syndrome
Citation Manager Formats
Make Comment
See Comments

A 55-year-old man with no comorbidities presented with sudden-onset holocranial headache, vomiting, and transient loss of consciousness. Examination was normal except for retinal arterio-venous malformations in the right eye (figure, A). MRI brain showed subacute hematoma in the right parieto-temporo-occipital region and frontal horn of the right lateral ventricle. Digital subtraction angiography showed arterio-venous malformations along the right ophthalmic artery and right posterior temporal region, suggestive of cerebrofacial arteriovenous metameric syndrome (CAMS) type 2 (figure, B–D). He was managed conservatively and is asymptomatic at 2 months follow-up. Fundus examination can be a diagnostic clincher for CAMS in adults with intracranial haemorrhages.1,2
(A) Right fundus photograph depicting retinal arteriovenous malformations (AVMs) in the right eye. (B) Axial T1 MRI depicting hematoma in the right temporo-parietal lobe and frontal horn of right lateral ventricle and subacute subdural hematoma along tentorium cerebelli on the right side and along the convexity of the right cerebral hemisphere. (C) Right ophthalmic artery feeder AVMs. (D) Right posterior temporal artery feeder AVMs.
Study funding
No targeted funding reported.
Disclosure
The authors report no disclosures relevant to the manuscript. Go to Neurology.org/N for full disclosures.
Appendix Authors

Footnotes
Go to Neurology.org/N for full disclosures. Funding information and disclosures deemed relevant by the authors, if any, are provided at the end of the article.
Teaching slides links.lww.com/WNL/B179
- © 2020 American Academy of Neurology
References
Disputes & Debates: Rapid online correspondence
NOTE: All authors' disclosures must be entered and current in our database before comments can be posted. Enter and update disclosures at http://submit.neurology.org. Exception: replies to comments concerning an article you originally authored do not require updated disclosures.
- Stay timely. Submit only on articles published within the last 8 weeks.
- Do not be redundant. Read any comments already posted on the article prior to submission.
- 200 words maximum.
- 5 references maximum. Reference 1 must be the article on which you are commenting.
- 5 authors maximum. Exception: replies can include all original authors of the article.
- Submitted comments are subject to editing and editor review prior to posting.