Images in… Alex Rebello ‍ ‍,1 Chirag Kamal Ahuja ‍ ‍,2 Sarath Aleti ‍ ‍,1 Rajveer Singh ‍ ‍1 1 Department of Neurology, Post Graduate Institute of Medical Education and Research, Chandigarh, India 2 Department of Radiodiagnosis and Imaging, Post Graduate Institute of Medical Education and Research, Chandigarh, India Correspondence to Dr Rajveer Singh; ​rajveersaharan@​gmail.​com Accepted 28 January 2021 DESCRIPTION A 12-­year-­old boy presented with one episode of generalised tonic-­ clonic seizure and sudden onset left hemiplegia to the emergency department. He had two episodes of febrile seizures in childhood with no other significant history. His non-­contrast CT of the head showed right frontal lobar haemorrhage with mass effect and an old infarct in the right anterior frontal lobe (figure 1A). He underwent decompressive hemicraniectomy. His digital subtraction angiography in the arterial phase (figure 1B) showed multiple areas of abnormal leash of small vessels in the right frontal and parietal regions fed by the cortical branches of the right Middle Cerebral Artery with no early draining vein appreciated in the venous phase (figure 1C) suggesting cerebral proliferative angiopathy. He was given physiotherapy and he improved with a modified Rankin Scale (mRS) score of 1 after 2 months on follow-­up. DISCUSSION © BMJ Publishing Group Limited 2021. No commercial re-­use. See rights and permissions. Published by BMJ. To cite: Rebello A, Ahuja CK, Aleti S, et al. BMJ Case Rep 2021;14:e240468. doi:10.1136/bcr-2020240468 Cerebral proliferative angiopathy is a rare and distinct entity, which differs from classical cerebral arteriovenous malformation in epidemiological, clinical and angiomorphological features. It is more commonly seen in adolescent and middle-­aged females. The common presentations are seizures, headache and progressive neurological deficits. Haemorrhages are less common in cerebral proliferative angiopathy than in classical arteriovenous malformation. The lesions are large with normal brain parenchyma between the vascular spaces.1 2 The angiography in cerebral proliferative angiopathy shows multiple arterial feeders, which are not enlarged or moderately enlarged without any dominant feeder. The draining vessels are scanty compared with the size of the nidus unlike classical arteriovenous malformation.1 The treatment is often challenging as surgery and embolisation may cause permanent neurological deficit, due to damage of interspersed normal neural tissue in cerebral proliferative angiopathy.3 Twitter Alex Rebello @drAlexRebello Contributors AR drafted the manuscript and had role in organisation and execution of the project. CKA had role in critical review of the manuscript and assessment of the radiological Figure 1 Cranial non-­contrast CT (A) reveals right frontal lobe haematoma with mass effect. Catheter digital subtraction angiography of right Internal Cerebral Artery (RICA) in the arterial phase (B) demonstrates abnormal leash of vessels (white arrows) in the right frontal lobe fed by cortical branches of Middle Cerebral Artery (MCA) with no early draining vein appreciated in the venous phase (C). Learning points ►► Cerebral proliferative angiopathy is a distinct entity, which differs from classical cerebral arteriovenous malformation. ►► It is a rare cause of stroke and seizures in young and its treatment is challenging. findings. SA had role in organisation and execution of the project. RS had role in conception, organisation and execution of the report and critically reviewed the manuscript. Funding The authors have not declared a specific grant for this research from any funding agency in the public, commercial or not-­for-­profit sectors. Competing interests None declared. Patient consent for publication Parental/guardian consent obtained. Provenance and peer review Not commissioned; externally peer reviewed. ORCID iDs Alex Rebello http://​orcid.​org/​0000-​0002-​0954-​5475 Chirag Kamal Ahuja http://​orcid.​org/​0000-​0003-​0734-​3252 Sarath Aleti http://​orcid.​org/​0000-​0003-​2847-​629X Rajveer Singh http://​orcid.​org/​0000-​0001-​7173-​8035 REFERENCES 1 Lasjaunias PL, Landrieu P, Rodesch G, et al. Cerebral proliferative angiopathy: clinical and angiographic description of an entity different from cerebral AVMs. Stroke 2008;39:878–85. 2 Liu P, Lv X, Lv M, et al. Cerebral proliferative angiopathy: clinical, angiographic features and literature review. Interv Neuroradiol 2016;22:101–7. 3 Chin LS, Raffel C, Gonzalez-­Gomez I, et al. Diffuse arteriovenous malformations: a clinical, radiological, and pathological description. Neurosurgery 1992;31:863–8. discussion 8-9. Rebello A, et al. BMJ Case Rep 2021;14:e240468. doi:10.1136/bcr-2020-240468 1 BMJ Case Rep: first published as 10.1136/bcr-2020-240468 on 11 February 2021. Downloaded from http://casereports.bmj.com/ on April 18, 2024 by guest. Protected by copyright. Cerebral proliferative angiopathy: a rare cause of stroke and seizures in young Images in… Become a Fellow of BMJ Case Reports today and you can: ►► Submit as many cases as you like ►► Enjoy fast sympathetic peer review and rapid publication of accepted articles ►► Access all the published articles ►► Re-use any of the published material for personal use and teaching without further permission Customer Service If you have any further queries about your subscription, please contact our customer services team on +44 (0) 207111 1105 or via email at support@bmj.com. Visit casereports.bmj.com for more articles like this and to become a Fellow 2 Rebello A, et al. BMJ Case Rep 2021;14:e240468. doi:10.1136/bcr-2020-240468 BMJ Case Rep: first published as 10.1136/bcr-2020-240468 on 11 February 2021. Downloaded from http://casereports.bmj.com/ on April 18, 2024 by guest. Protected by copyright. Copyright 2021 BMJ Publishing Group. All rights reserved. For permission to reuse any of this content visit https://www.bmj.com/company/products-services/rights-and-licensing/permissions/ BMJ Case Report Fellows may re-use this article for personal use and teaching without any further permission.