Post on 06-Apr-2022
transcript
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
Neurology® Published Ahead of Print articles have been peer reviewed and accepted for
publication. This manuscript will be published in its final form after copyediting, page
composition, and review of proofs. Errors that could affect the content may be corrected during
these processes.
ACCEPTED
Copyright © 2020 American Academy of Neurology. Unauthorized reproduction of this article is prohibited
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
ACCEPTED
Copyright © 2020 American Academy of Neurology. Unauthorized reproduction of this article is prohibited
Word Count:
----------------
Manuscript: 97, Figures :1, Tables:0, References: 4, Supplemental appendix : 1
Keywords:
--------------
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.
ACCEPTED
Copyright © 2020 American Academy of Neurology. Unauthorized reproduction of this article is prohibited
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
ACCEPTED
Copyright © 2020 American Academy of Neurology. Unauthorized reproduction of this article is prohibited
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.
ACCEPTED
Copyright © 2020 American Academy of Neurology. Unauthorized reproduction of this article is prohibited
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).
ACCEPTED
Copyright © 2020 American Academy of Neurology. Unauthorized reproduction of this article is prohibited
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
ServicesUpdated Information &
425.citation.fullhttp://n.neurology.org/content/early/2020/07/20/WNL.0000000000010including high resolution figures, can be found at:
Subspecialty Collections
http://n.neurology.org/cgi/collection/mriMRI
http://n.neurology.org/cgi/collection/intracerebral_hemorrhageIntracerebral hemorrhage
http://n.neurology.org/cgi/collection/clinical_neurology_examinationClinical neurology examination
http://n.neurology.org/cgi/collection/arteriovenous_malformationArteriovenous malformation
http://n.neurology.org/cgi/collection/all_neuroophthalmologyAll Neuro-ophthalmologyfollowing collection(s): This article, along with others on similar topics, appears in the
Permissions & Licensing
http://www.neurology.org/about/about_the_journal#permissionsits entirety can be found online at:Information about reproducing this article in parts (figures,tables) or in
Reprints
http://n.neurology.org/subscribers/advertiseInformation about ordering reprints can be found online:
rights reserved. Print ISSN: 0028-3878. Online ISSN: 1526-632X.1951, it is now a weekly with 48 issues per year. Copyright © 2020 American Academy of Neurology. All
® is the official journal of the American Academy of Neurology. Published continuously sinceNeurology