Cerebral proliferative angiopathy

BACKGROUND: Cerebral proliferative angiopathy (CPA) is a rare subtype of arteriovenous malformation. It is extremely rare in pediatric patients and has serious implications for developing children. However, reports of these disorders worldwide are limited, and no uniform reference for diagnosis and treatment options exists. We report the case of a 6-year-old with CPA having predominantly neurological dysfunction and review the literature on pediatric CPA. CASE SUMMARY: We report the case of a pediatric patient with CPA analyzed using digital subtraction angiography (DSA) who presented initially with a neurological disorder as the main manifestation...

Cerebral proliferative angiopathy (CPA) is an entity distinct from that of classical arteriovenous malformations. As such, few reports have considered the long-term follow-up of patients with hemorrhage in CPA. Accordingly, herein the authors present a case of recurrent hemorrhage in CPA with 32 years of follow-up and in so doing summarize the literature of hemorrhagic cases in CPA. A 19-year-old presented with focal awareness seizures and diagnostic work-up revealed a left hemispheric vascular lesion...

Hepatopulmonary syndrome (HPS) is a condition characterized by chronic liver disease, intrapulmonary arteriovenous shunting, and increased alveolar-arterial oxygen gradient. This case report presents a 54-year-old male patient with a history of stroke, liver cirrhosis, portal vein thrombosis, hypertension, diabetes, and bladder cancer, who presented with worsening headaches and confusion over the course of five years. Digital subtraction angiogram (DSA) revealed multiple bilateral arteriovenous shunts, suggesting a shunting mechanism similar to that observed in HPS...

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UNLABELLED: Cerebral proliferative angiopathy (CPA) is a rare cerebrovascular disease comprised of a diffuse network of blood vessels, often spread over several lobes. Due to the presence of normal brain tissue within the malformation, complete resection is not desirable. Several imaging characteristics help to distinguish CPA from brain arteriovenous malformations. We report the case of a patient diagnosed with CPA five years after a spontaneous intraventricular hemorrhage and an initial negative angiogram...

An 18-year-old man presented to the neurosurgery outpatient department with recurrent episodes of epistaxis for the past 8 years and altered behavior for the past month. Epistaxis was scanty in amount, intermittent, spontaneous, and not associated with any trauma or nasal obstruction or breathing difficulties. Bleeding used to stop spontaneously after some time. There was no history of associated headache, seizures, vomiting, fever, or loss of consciousness. On physical examination, the patient was afebrile, with normal vital signs and normal Glasgow Coma Scale score (15 of 15) at the time of presentation...

BACKGROUND: Cerebral proliferative angiopathy (CPA) is a rare vascular proliferative disease; however, long-term follow-up reports are scarce. The authors report a rare case and document a patient's medical history over 20 years. OBSERVATIONS: A 5-year-old girl developed left frontal lobe hemorrhage, presenting with headache. At 8 years of age, angiography showed diffuse capillary ectasia without an arteriovenous shunt. Single-photon emission computed tomography (SPECT) showed normal cerebral blood flow (CBF)...

OBJECTIVE: Genetic alterations are increasingly recognized as etiologic factors linked to the pathogenesis and development of cerebrovascular anomalies. Their identification allows for advanced screening and targeted therapeutic approaches. The authors aimed to describe the role of a collaborative approach to care and genetic testing in pediatric patients with neurovascular anomalies, with the objectives of identifying what genetic testing recommendations were made, the yield of genetic testing, and the implications for familial screening and management at present and in the future...

OBJECTIVE: Cerebral proliferative angiopathy (CPA) is a rare disease, characterized by a large vascular nidus, diffuse angiogenesis, and intermingled normal brain tissue. Conservative treatment, endovascular intervention, indirect revascularization, and radiotherapy have been applied to treat this disease. However, some cases deteriorate even after non-conservative treatment, and there has been no standard of treatment for this disease so far. In order to build a better treatment strategy, we review literature, present our case and propose an algorithm for managing CPA...

Cerebral proliferative angiopathy (CPA) is a rare cerebrovascular pathology that presents with unique clinical features due to distinct histologic, angiographic, and pathophysiologic characteristics that separate it from classical arteriovenous malformation. The disorder is characterized by uncontrolled angiogenesis in which functional brain parenchyma is interspersed with abnormal vascular channels without a distinct nidus. Common presenting symptoms include headache, seizures, and stroke-like symptoms. Hemorrhagic presentations are rare in contrast to the typical presentations of classical arteriovenous malformation...

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...

Cerebral proliferative angiopathy is a rare cerebrovascular disorder characterized by diffuse abnormal vessels with intermingled brain parenchyma fed by many arteries and draining into many veins without high-flow arteriovenous shunts, which is usually confirmed by conventional digital subtraction angiography. However, dilution of the contrast medium due to the markedly increased blood flow and volume in cerebral proliferative angiopathy leads to low-contrast angiography. We report a 53-year-old man with cerebral proliferative angiopathy who underwent CT, MR imaging, MR angiography, digital subtraction angiography and 4D-CTA...

Diffuse proliferative cerebral angiopathy (DPCA) is an uncommon type of cerebral vascular malformation, mostly diagnosed in young females. It is characteristically different from other cerebral arteriovenous malformations and can be differentiated by its peculiar imaging findings. A nidus of normal brain parenchyma is present between the abnormal vascular channels. Therefore, it is crucial to diagnose it as a separate entity because unnecessary treatment of DPCA increases the risk of damage to the normal parenchyma leading to neurological deficits...

Purpose of the Article Cerebral proliferative angiopathy (CPA) is a rare and recently characterized vascular malformation that is often mistaken for a large, diffuse arteriovenous malformation (AVM). However, distinguishing the two entities is critical, as while the diseases may appear similar on imaging, they are completely different entities. The most distinguishing features of CPA compared to AVM are the presence of normal functioning brain within the 'nidus' of the abnormality and the proliferative nature of the nidus...

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...

We are describing a case of a 14-year-old girl who developed acute cerebral infarction which was documented on diffusion-weighted magnetic resonance imaging (DWI). On detailed evaluation, diagnosis of cerebral proliferative angiopathy (CPA) was made. Incidentally, she had capillary malformation on the forehead contralateral to cerebral vascular malformation. To our knowledge, this is the first case of CPA in the literature where DWI abnormality was due to acute cerebral infarction.

BACKGROUND: Cerebral proliferative angiopathy (CPA) is a rare vascular disease characterized by the presence of diffuse vascular proliferation, progressive vascular hyperflow and vasodilation of multiple vessels in the normal brain parenchyma. Unlike cerebral arteriovenous malformations, CPA has a mixed appearance between that of lesions with cell proliferation and endothelial proliferation. To date, the pathogenesis of CPA is unclear, in which changes induced by cortical ischemia in the elastic layer of the blood supply artery and smooth muscle cells may be involved...

Cerebral proliferative angiopathy (CPA) is an infrequent vascular malformation. It is composed of a nidus, arterial feeders, and venous drainage. Some special features differentiate it from the common arteriovenous malformations (AVM). The nidus has normal cerebral tissue intermingled and occupies a large portion of the brain. There is a frequent transdural arterial supply. Arterial feeders and draining veins are of small caliber concerning the nidus size. Ischemic strokes are the most common clinical event...

No abstract text is available yet for this article.

BACKGROUND: Cerebral proliferative angiopathy is a vascular malformation associated with compromised blood-brain barrier and with migraine-like headache. Treating blood-brain barrier-compromised patients with erenumab, an anti-calcitonin gene-related peptide receptor monoclonal antibody, may be risky. CASE: We describe a case of a 22-year-old chronic migraine patient with cerebral proliferative angiopathy who presented to our hospital in status epilepticus 2 d after his first dose of erenumab...