Cerebral Proliferative Angiopathy - Literature Review and Single Institute Experience
Fiza Laheji1, Wilmot Bonnet2, Michael Dowling1
1University of Texas Southwestern, 2University of Texas SW Medical School, Child Neurology
Objective:
We aim to share a case series of 3 patients with Cerebral Proliferative Angiopathy(CPA) and different management methods used for these refractory cases.
Background:
Cerebral proliferative angiopathy is a rare phenomenon. There is limited data in the literature on the management and natural history of this condition.Formerly known as the “diffuse nidus” or “holohemispheric giant cerebral arteriovenous malformation (AVM),” it is morphologically considered to be a congenital vascular anomaly. According to the Stroke paper published in 2008, CPA was defined as an atypical entity that appears to differ significantly from normal brain AVMs. Until 2016 per review published in "Interventional Neuroradiology", only a total number of 74 cases had been reported thus far in the literature.
Design/Methods:
We conducted a retrospective chart review at our institution and reviewed patients with a diagnosis of cerebral proliferative angiopathy, confirmed on neuroimaging.
We reviewed different parameters such as : age of diagnosis, history of infarct or bleed, treatments used, repeat imaging to evaluate for progression of disease, development of seizures, latest PSOM score.
Results:
All 3 of the patients in our cohort were Male and were noted to have refractory progressive disease resulting in infarcts and intracerebral hemorrhages. they were treated with gama knife , Vascular emobilization and also the novel use of rapamycin and sirolimus in one of the cases. All 3 of the patients were on AEDs for seizure management.
Conclusions:
CPA is a rare disease with limited data in the literature on treatment guidelines and the progression of disease. By highlighting our single institute experience of 3 cases of pediatric CPA, and the novel use of sirolimus, rapamycin we aim to shed light on this condition and help develop treatment guidelines in the future.