Cerebral Proliferative Angiopathy in a Child

Puerta, Patricia; Guillén, Antonio; Muchart, Jordi; González, Verónica; Ferrer, Enrique

doi:10.1159/000459629

Cited by 13 publications

(8 citation statements)

References 7 publications

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“…All studies utilized indirect forms of revascularization with 3 patients receiving calvarial burr holes, 18,26 and the remaining 4 receiving various forms of encephaloduroarteriosynangiosis (EDAS). 4,15,19,25 No studies utilized direct revascularization.…”

Section: Revascularizationmentioning

confidence: 99%

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Cerebral revascularization in cerebral proliferative angiopathy: a systematic review

Somji¹,

McEachern

Silvaggio³

2019

Neurosurgical Focus

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OBJECTIVECerebral proliferative angiopathy (CPA) is considered a discrete vascular malformation of the brain separate from classical brain arteriovenous malformations (AVMs). It has unique angiographic characteristics and has been hypothesized to result from chronic cortical ischemia and perinidal oligemia. Treatment with cerebral revascularization has been proposed in an attempt to disrupt regional hypoperfusion and interrupt the angiogenesis that defines CPA. A systematic review of the literature pertaining to the role of cerebral revascularization may highlight a treatment paradigm for this rare disease.METHODSA systematic review was performed in accordance with Preferred Reporting Items for Systematic Reviews and Meta-Analysis (PRISMA) guidelines. MEDLINE and Embase were searched from inception for papers relating to CPA. Included articles were categorized according to methodology (case series or imaging study) and treatment modality (conservative, radiation, endovascular, or revascularization). A synthesis was compiled summarizing the current evidence regarding cerebral revascularization in CPA.RESULTSThe initial search revealed 43 articles, of which 28 studies met the inclusion criteria. Nine studies were identified that described imaging findings, which suggested hemodynamic dysregulation and perinidal impairments in the cerebrovascular reserve could be identified compared to unaffected hemispheres and classical brain AVMs. Six studies including 7 patients undergoing indirect forms of cerebral revascularization were identified. Clinical and radiological outcomes following revascularization were favorable in all but one study.CONCLUSIONSA small body of radiological and clinical studies has emerged, suggesting that CPA is a response to perinidal oligemia. While the long-term clinical efficacy of revascularization remains unclear, early results suggest that this may be a novel treatment paradigm for patients with CPA.

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Section: Revascularizationmentioning

confidence: 99%

“…26 One study noted a neurological deficit developing 2 years after cerebral revascularization. 25 Radiological outcomes reported in 3 studies demonstrated diffuse neovascularization following cerebral revascularization. 4,26,15 Ellis et al demonstrated robust revascularization of the perinidal region 8 months following EDAS.…”

Section: Revascularizationmentioning

confidence: 99%

Cerebral revascularization in cerebral proliferative angiopathy: a systematic review

Somji¹,

McEachern

Silvaggio³

2019

Neurosurgical Focus

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“…In some hemorrhagic patients, calvarial burr hole treatment may be preferred to relieve arterial ischaemic zone because one of the major pathomechanism of this disease is ischaemia, any therapy that enhances cortical blood supply can be indicated. [21][22][23][24][25] This strategy differs from that of classical brain AVMs, in which management is directed mainly towards preventing haemorrhage. Yamaki et al 26 performed a systematic review of CPA, which include 33 studies, rendering a total of 95 cases and concluded that both conservative and endovascular treatments are valid choices.…”

Section: Treatmentmentioning

confidence: 99%

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

Srivastava

Gandhi

Kumar³

2022

Neuroradiol J

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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.

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“…Perfusion images may play a role in the diagnosis and follow-up of these patients, to assess the extension of ischemic compromise and the efficacy of treatments (3,20) . Several methods were used across case reports and series, such as perfusion-weighted MRI (PW-MRI) (3,6,15,(20)(21)(22) , 123I-IMP-SPECT (21) , 99mTc-ECD-SPECT (18) , Positron Emission Tomography (PET) (23) , and 11C-METH PET (24) .…”

Section: Diagnosismentioning

confidence: 99%

“…There is widespread consensus that cortical blood supply enhancement is a rational approach in a subset of patients presenting with poor transdural supply, evident ischemic abnormalities and persisting symptoms during follow-up. Approaches such as skull burr-hole (2) , pial synangiosis (22) and encephalomyosynangiosis, and encephalo-duroarterio-synangiosis (EDAS) (18) have been reported, all with good outcomes.…”

Section: Treatmentmentioning

confidence: 99%

Cerebral proliferative angiopathy: a review / Angiopatia proliferativa cerebral: uma revisão

Nunes

Santos

Aguiar

et al. 2019

Arq Med Hosp Fac Cienc Med Santa Casa São Paulo

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Introduction: Cerebral Arteriovenous Malformations (AVMs) are vascular lesions defined by arteriovenous shunting flowing through a nidus of coiled and tortuous vascular connections. Cerebral Proliferative Angiopathy (CPA) is a rare type of AVM with pathological, clinical, diagnostic features and treatment differences from a typical AVM. We aimed to summarize important features of CPA already described and identify important gaps of evidence. Methods: Data was selected by a search of PUBMED with the term "cerebral proliferative angiopathy". The articles considered relevant were included in this review. We also screened article references and included them as needed. Discussion: CPA shows a diffuse nidus appearance, presence of transdural supply and stenosis of feeding arteries, without dominant feeders or flow-related aneurysms. There is intermingled brain between the vascular spaces. The usual clinical presentation is seizures, headaches, and progressive neurological deficits. Sound diagnosis can be made based on CT, MRI, Angiography and perfusion techniques, which are essential to treatment choices. Invasive treatment is seldom indicated, in order to preserve the normal brain parenchyma, and clinical control of symptoms, associated with a thorough follow-up, is the usual approach in most reported cases. Conclusion: Still much remains to be learned about CPA. Establishing more precise roles of various radiological assessing methods, how they affect the follow-up of patients, and safety and efficacy profiles of different treatment approaches will provide us the tools to give patients a more solid intervention and clearer follow-up.

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Cerebral Proliferative Angiopathy in a Child

Cited by 13 publications

References 7 publications

Cerebral revascularization in cerebral proliferative angiopathy: a systematic review

Cerebral revascularization in cerebral proliferative angiopathy: a systematic review

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

Cerebral proliferative angiopathy: a review / Angiopatia proliferativa cerebral: uma revisão

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