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October 27, 2020; 95 (17) Resident & Fellow Section

Teaching NeuroImages: An oculocerebral metameric syndrome

Biswamohan Mishra, Venugopalan Y. Vishnu, Anu Gupta, Ajay Garg, Rohit Saxena, Roopa Rajan, Rohit Bhatia, Mamta Bhushan Singh, M.V. Padma Srivastava
First published July 20, 2020, DOI: https://doi.org/10.1212/WNL.0000000000010425
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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

Figure
Figure Right fundus, MRI brain, and digital subtraction angiography imaging of the cerebral vessels

(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

Table1

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

References

  1. 1.↵
    1. O'Loughlin L,
    2. Groves ML,
    3. Miller NR,
    4. Pearl MS
    . Cerebrofacial arteriovenous metameric syndrome (CAMS): a spectrum disorder of craniofacial vascular malformations. Childs Nerv Syst 2017;33:513–516.
    OpenUrl
  2. 2.↵
    1. Bhattacharya JJ,
    2. Luo CB,
    3. Suh DC,
    4. Alvarez H,
    5. Rodesch G,
    6. Lasjaunias P
    . Wyburn-Mason or Bonnet-Dechaume-Blanc as cerebrofacial arteriovenous metameric syndromes (CAMS): a new concept and a new classification. Interv Neuroradiol 2001;7:5–17.
    OpenUrlCrossRefPubMed
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