Neurology Publish Ahead of PrintDOI: 10.1212/WNL.0000000000010425

Teaching NeuroImages: An oculo-cerebral metameric syndrome

Biswamohan Mishra 1, Vishnu Vishnu Y

2* , Anu Gupta

3, Ajay Garg

4, Rohit Saxena

5, Roopa

Rajan6, Rohit Bhatia

7, Mamta Bhushan Singh

8, MV Padma Srivastava

9

1Dr. Biswamohan Mishra, MD

Department of Neurology

All India Institute of Medical Sciences, New Delhi, India

2Dr. Venugopalan Y Vishnu, MD, DM Neurology

Department of Neurology

All India Institute of Medical Sciences, New Delhi, India

3Dr. Anu Gupta, MD, DM Neurology

Department of Neurology

All India Institute of Medical Sciences, New Delhi, India

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Published Ahead of Print on July 20, 2020 as 10.1212/WNL.0000000000010425

4Dr. Ajay Garg, MD Radiodiagnosis

Department of Neuroradiology,

All India Institute of Medical Sciences, New Delhi, India,

5Dr. Rohit Saxena, MD Ophthalmology

Department of Ophthalmology

All India Institute of Medical Sciences, New Delhi, India

6Dr. Roopa Rajan, MD, DM Neurology

Department of Neurology

All India Institute of Medical Sciences, New Delhi, India

7Dr.Rohit Bhatia, MD, DM neurology

Department of Neurology

All India Institute of Medical Sciences, New Delhi, India

8Dr.Mamta Bhushan Singh, MD, DM Neurology

Department of Neurology

All India Institute of Medical Sciences, New Delhi, India

9Dr.MV Padma Srivastava, MD, DM Neurology

Department of Neurology

All India Institute of Medical Sciences, New Delhi, India

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Word Count:

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Manuscript: 97, Figures :1, Tables:0, References: 4, Supplemental appendix : 1

Keywords:

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Cerebrofacial arteriovenous metameric syndrome, Arteriovenous malformation, Wyburn

Mason Syndrome, Bonnet-Dechaume-Blanc syndrome, Digital Subtraction Angiography,

intracranial haemorrhage *Corresponding Author: Dr Venugopalan Y Vishnu (vishnuvy16@yahoo.com)

Abbreviations:

CAMS - Cerebrofacial Arteriovenous Metameric Syndrome

AV- Arterio-venous

AVM - Arterio-venous Malformation

CT- Computed Tomography

MRI – Magnetic Resonance Imaging

DSA – Digital Subtraction Angiography

MCA- Middle Cerebral Artery

Study funding: No targeted funding reported

Disclosure: The authors report no disclosures relevant to the manuscript.

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A 55-year male with no comorbidities presented with sudden onset holocranial headache,

vomiting and transient loss of consciousness. Examination was normal except retinal arterio-

venous malformations in right eye. (figure 1A) MRI brain showed subacute hematoma in right

parieto- temporo-occipital region and frontal horn of right lateral ventricle. DSA showed AVMs

along the right ophthalmic artery and right posterior temporal region, suggestive of

Cerebrofacial arteriovenous metameric syndrome (CAMS) Type 2. (Figure 1B-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

Appendix 1: Authors

Names Location Contribution

Dr. Biswamohan Mishra, MD All India Institute of

Medical Sciences, New

Delhi, India

Drafted the manuscript,

Management of the patient

Dr. Venugopalan Y Vishnu,

MD, DM Neurology

All India Institute of

Medical Sciences, New

Delhi, India,

Concept, review and editing of

manuscript, Management of the

patient

Dr. Anu Gupta, MD, DM

Neurology

All India Institute of

Medical Sciences, New

Delhi, India,

Editing of manuscript

Dr. Ajay Garg, MD

Radiodiagnosis

All India Institute of

Medical Sciences, New

Delhi, India,

Neuroradiology expertise in

Diagnosis and Management

Dr. Rohit Saxena, MD

Ophthalmology,

All India Institute of

Medical Sciences, New

Delhi, India,

Ophthalmology expertise in

Diagnosis and Management

Dr. Roopa Rajan, MD, DM

Neurology

All India Institute of

Medical Sciences, New

Delhi, India,

Editing of manuscript

Dr. Rohit Bhatia, MD, DM

neurology,

All India Institute of

Medical Sciences, New

Delhi, India,

Management of the patient

Dr. Mamta Bhushan Singh,

MD,DM Neurology

All India Institute of

Medical Sciences, New

Delhi, India,

Management of the patient

Dr. MV Padma Srivastava,

MD, DM Neurology

All India Institute of

Medical Sciences, New

Delhi, India,

Management of the patient

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Teaching Slides-http://links.lww.com/WNL/B179

References

1. O’Loughlin L, Groves ML, Miller NR, Pearl MS. Cerebrofacial arteriovenous metameric

syndrome (CAMS): a spectrum disorder of craniofacial vascular malformations. Childs Nerv Syst.

2017 Mar;33(3):513–6.

2. Bhattacharya JJ, Luo CB, Suh DC, Alvarez H, Rodesch G, Lasjaunias P. Wyburn-Mason or

Bonnet-Dechaume-Blanc as Cerebrofacial Arteriovenous Metameric Syndromes (CAMS): A New

Concept and a New Classification. Interv Neuroradiol. 2001 Mar;7(1):5–17.

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Legend

Figure. Right fundus, MRI Brian images, and DSA imaging of the cerebral vessels

Right fundus photograph depicting retinal AV malformations in right eye (A). Axial T1 MRI

images depicting hematoma in the right temporo -parietal lobe and frontal horn of right lateral

ventricle and Subacute subdural hematoma along tentorium cerebelli on right side and along

the convexity of the right cerebral hemisphere(B). Right ophthalmic artery feeder AVMs (C).

Right posterior temporal artery feeder AVMs (D).

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DOI 10.1212/WNL.0000000000010425 published online July 20, 2020Neurology 

Biswamohan Mishra, Venugopalan Y. Vishnu, Anu Gupta, et al. Teaching NeuroImages: An oculo-cerebral metameric syndrome

This information is current as of July 20, 2020

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