Clinico-radiological features of cerebral proliferative angiopathy: A large case series from India

Background: Based on angiographic features, a rare subgroup, different from classical brain AVMs, called cerebral proliferative angiopathy (CPA) was suggested by Lasjaunias et al. in 1989. There are only few reports of CPA described from Asia. Liu et al. has described 5 cases of CPA from China. The objective of study was to evaluate the clinico-radiological features of patients with CPA in our cohort and to find out the differences between our study and the study by Lasjaunias and Liu et al. Methods: We retrospectively analysed cases of CPA from March 2012 to April 2020. All radiological images of the patients with AVMs were studied and findings suggestive of CPA were identified. We found 10 patients with CPA out of 272 cases of AVM (10/272) and described the various demographic and clinico-radiological features. Results: The age of the patients with CPA in our series varied from 14 to 53 years and the mean age was 28.6 years. There was male preponderance (60%) with male to female ratio of 3:2. The most common presentation was seizure (70%). Intracranial haemorrhage was present in five patients. One patient had cerebral infarction. Conclusions: There were substantial differences in clinico-radiological profile of patients with CPA in our cohort as compared to the study by Lasjaunias et al. and Liu et al. in form of male dominance, higher rate of ICH and seizure. In our knowledge, this is the second largest series of CPA worldwide and the first largest series in Asia.