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Isolation and Cannulation of Cerebral Parenchymal Arterioles
Published on: May 23, 2016
Cerebral proliferative angiopathy accompanied by cerebral cavernous malformation: A case report
Eyüp Furkan Engin1, Burcu Polat2, Gülhan Ertan Akan3
1Medical Directory, School of Medicine, Istanbul Medipol University, Istanbul, Turkey.
Insights
Cerebral Proliferative Angiopathy (CPA) co-occurring with Cerebral Cavernous Malformation (CCM) in a patient with a family history of CCM suggests a potential shared cause for these rare vascular brain lesions.
Area of Science:
- Neurology
- Vascular Malformations
- Neuroimaging
Background:
- Cerebral Proliferative Angiopathy (CPA) is a rare vascular malformation distinct from arteriovenous malformations (AVMs).
- Understanding CPA pathophysiology is crucial due to its unique clinical progression and imaging characteristics.
Observation:
- A 37-year-old male presented with headache, right-sided weakness, and speech impairment.
- Neuroimaging revealed CPA on the left hemisphere and two Cerebral Cavernous Malformations (CCMs) on the right.
- The patient's family history included two brothers diagnosed with CCMs.
Findings:
- Digital subtraction angiography confirmed CPA and identified coexisting CCMs.
- The combined presence of CPA and CCM, along with a familial predisposition to CCM, was noted.
Implications:
- The co-occurrence of CPA and CCM may indicate a common underlying pathophysiological mechanism.
- Further research into shared genetic or molecular pathways is warranted to elucidate the etiology of these rare vascular lesions.
Abstract:
Cerebral Proliferative Angiopathy (CPA) is a rare vascular malformation that is distinguished from classical brain arteriovenous malformations (AVM) in its imaging findings and clinical progression but more importantly in its pathophysiology. Here we report the case of a 37-year-old male patient with CPA accompanied by Cerebral Cavernous Malformation (CCM) in hopes to expand the inquiry into the pathophysiology of this rare lesion. A patient with progressive headache, right-sided weakness, and impaired speech were evaluated at our medical center. Neuroimaging studies were performed, and the patient was diagnosed with CPA. The patient has been followed up with conservative management and periodic neuroradiological evaluation for 5 years. Digital subtraction angiography (DSA) showed a vascular malformation diffusely covering the left hemisphere that is consistent with CPA. In addition, 2 sequential CCMs were detected in the right hemisphere. Also, the patients' familial history included two brothers with CCMs. The coexistence of CPA with CCM and patients' familial history of CCM could suggest the possibility of a common pathophysiological element.

