Cerebral Proliferative Angiopathy (CPA): Comprehensive Disease Characteristics Report

Target disease

• Disease name: Cerebral proliferative angiopathy (CPA)
• Category: Complex cerebrovascular malformation / AVM-like entity
• MONDO ID: Not identified in the accessed primary/review literature in this run (formal MONDO/Orphanet/MeSH/ICD codes were not explicitly provided in retrieved full texts) (yamaki2020thecurrentclinical pages 1-3, tiwari2020cerebralproliferativeangiopathy pages 1-3).

1. Disease information

1.1 Concise overview (current understanding)

CPA is a rare, AVM-like cerebrovascular entity that differs from “classical” brain arteriovenous malformations (bAVMs) in angioarchitecture, clinical presentation, natural history, and treatment implications (lasjaunias2008cerebralproliferativeangiopathy pages 1-2, lasjaunias2008cerebralproliferativeangiopathy pages 2-4). In the foundational Stroke cohort, CPA is described on cross-sectional imaging as “a diffuse network of densely enhancing vascular spaces with intermingled normal brain parenchyma” and angiographically by non-focal angiogenetic activity with numerous small feeders/drainers rather than a compact nidus with high-flow shunting (lasjaunias2008cerebralproliferativeangiopathy pages 2-4, lasjaunias2008cerebralproliferativeangiopathy pages 1-2).

1.2 Synonyms / alternative names

Older or alternative labels used for lesions now considered CPA include: - “Diffuse nidus(-type) AVM” (tiwari2020cerebralproliferativeangiopathy pages 1-3, tiwari2020cerebralproliferativeangiopathy pages 3-4) - “Holohemispheric giant AVM” / “holohemispheric giant cerebral arterio-venous malformation” (yamaki2020thecurrentclinical pages 1-3, lasjaunias2008cerebralproliferativeangiopathy pages 6-7)

1.3 Key identifiers (OMIM, Orphanet, ICD-10/11, MeSH, MONDO)

Within the retrieved and read sources, no explicit mapping codes (OMIM/Orphanet/ICD/MeSH/MONDO) were provided in text (yamaki2020thecurrentclinical pages 1-3, tiwari2020cerebralproliferativeangiopathy pages 1-3, lasjaunias2008cerebralproliferativeangiopathy pages 1-2). Therefore, this report cannot safely supply specific code identifiers without introducing uncited assumptions.

1.4 Evidence provenance

Evidence is primarily from: - Aggregated disease-level resources: systematic reviews and case series syntheses (e.g., pooled 95 cases) (yamaki2020thecurrentclinical pages 3-4, hess2022cerebralproliferativeangiopathy pages 3-4). - Single-center cohorts / databanks: the Lasjaunias et al. cohort (49 CPA cases identified within an AVM databank) (lasjaunias2008cerebralproliferativeangiopathy pages 2-4). - Individual case reports: especially for emerging diagnostic and therapeutic approaches (2023–2024) (jongaliem2023useofbetablocker pages 1-2, gautam2024thetreatmentof pages 2-4).

2. Etiology

2.1 Disease causal factors (current consensus)

No established single-gene cause, inherited syndrome, or somatic mutation driver was identified in the accessed literature. Instead, CPA is repeatedly framed as a hemodynamic/angiogenic disorder, hypothesized to arise from chronic regional hypoperfusion (perinidal oligemia/ischemia) leading to diffuse angiogenesis (lasjaunias2008cerebralproliferativeangiopathy pages 6-7, yamaki2020thecurrentclinical pages 1-3).

• Lasjaunias et al. propose: “diffuse angiogenetic activity… related to reduced perinidal perfusion and subsequent chronic cortical ischemia” (lasjaunias2008cerebralproliferativeangiopathy pages 1-2).
• A systematic review notes: “Its epidemiology and pathophysiology are uncertain” and proposes “vascular proliferation in response to chronic parenchymal ischemia/oligemia” (yamaki2020thecurrentclinical pages 1-3).

2.2 Risk factors

Robust epidemiologic risk-factor data (e.g., hypertension, smoking, etc.) were not identified in the accessed evidence base; the available literature is dominated by case reports/series and imaging-characterization studies (yamaki2020thecurrentclinical pages 3-4, hess2022cerebralproliferativeangiopathy pages 3-4). Demographic associations (young age, female predominance) are described (see Epidemiology below) but should be treated as descriptive rather than causal (lasjaunias2008cerebralproliferativeangiopathy pages 2-4, yamaki2020thecurrentclinical pages 3-4).

2.3 Protective factors

No protective genetic or environmental factors were reported in the accessed literature.

2.4 Gene–environment interactions

No gene–environment interactions were reported in the accessed literature.

2.5 Genetic/molecular associations (what is known)

• Genetic causes: Not reported in the accessed sources (yamaki2020thecurrentclinical pages 1-3, lasjaunias2008cerebralproliferativeangiopathy pages 6-7, jongaliem2023useofbetablocker pages 2-4).
• Angiogenic mediators: Reviews describe evidence compatible with angiogenic signaling (e.g., VEGF) and discuss speculative anti-angiogenic approaches; however, high-level clinical evidence is lacking (tiwari2020cerebralproliferativeangiopathy pages 3-4, jongaliem2023useofbetablocker pages 2-4).

3. Phenotypes

3.1 Core clinical phenotypes (with frequencies)

From Lasjaunias et al. (49 cases): seizures 45%, headaches 41%, TIAs/stroke-like symptoms 16%, hemorrhage 12% (lasjaunias2008cerebralproliferativeangiopathy pages 2-4). From a 2020 systematic review (95 cases): headache 44.9%, seizures 37.1%, transient ischemic attacks 33.7%, hemorrhage 18.0% (yamaki2020thecurrentclinical pages 3-4).

Pediatric phenotype (aggregate of 29 cases): focal deficits (n=17), headache (n=15), seizures (n=6) (moskalik2026cerebralproliferativeangiopathy pages 1-3).

3.2 Phenotype characteristics

• Typical age of onset/presentation: young (mean ~22–23 years in mixed-age cohorts) (lasjaunias2008cerebralproliferativeangiopathy pages 2-4, yamaki2020thecurrentclinical pages 3-4).
• Severity/progression: presentations often relate to seizures, disabling headaches, and progressive focal deficits; hemorrhagic presentations are comparatively less common but may recur once present (lasjaunias2008cerebralproliferativeangiopathy pages 2-4, lasjaunias2008cerebralproliferativeangiopathy pages 6-7).

3.3 Suggested HPO terms (examples)

(terms suggested for knowledge-base structuring; not claims of asserted ontology mapping) - Headache (HP:0002315) - Seizures (HP:0001250) - Transient ischemic attack (HP:0002326) - Hemiparesis (HP:0001269) - Aphasia (HP:0002381) - Intracranial hemorrhage (HP:0002170) / Intraventricular hemorrhage (HP:0002133)

3.4 Quality-of-life impact

Disabling headaches and medically refractory seizures are emphasized as reasons patients seek care and as treatment drivers, reflecting substantial functional burden (lasjaunias2008cerebralproliferativeangiopathy pages 1-2, jongaliem2023useofbetablocker pages 1-2).

4. Genetic / molecular information

4.1 Causal genes

No validated causal genes were identified in the accessed CPA literature (yamaki2020thecurrentclinical pages 1-3, lasjaunias2008cerebralproliferativeangiopathy pages 6-7).

4.2 Pathogenic variants / somatic vs germline

Not established for CPA in the accessed sources.

4.3 Modifier genes / epigenetics / chromosomal abnormalities

Not reported in the accessed sources.

4.4 Molecular hypotheses (angiogenesis)

CPA is repeatedly linked to angiogenesis driven by chronic hypoperfusion/oligemia (lasjaunias2008cerebralproliferativeangiopathy pages 6-7, jongaliem2023useofbetablocker pages 2-4). A 2023 propranolol case report argues for an anti-VEGF/anti-proliferative rationale, stating propranolol is a “VEGF inhibitory agent” and hypothesizing “Propranolol may have an antiproliferative effect on CPA” (jongaliem2023useofbetablocker pages 1-2).

5. Environmental information

No specific environmental exposures, toxins, lifestyle factors, or infectious triggers were reported in the accessed CPA literature.

6. Mechanism / pathophysiology

6.1 Proposed causal chain (current model)

Chronic regional hypoperfusion (oligemia/ischemia) → reactive/diffuse angiogenesis (including transdural supply and recruitment of multiple small-caliber vessels) → diffuse intraparenchymal vascular network with intermingled normal brain → clinical syndromes dominated by seizures/headache/ischemic deficits rather than primary hemorrhage (lasjaunias2008cerebralproliferativeangiopathy pages 1-2, lasjaunias2008cerebralproliferativeangiopathy pages 6-7, tiwari2020cerebralproliferativeangiopathy pages 3-4).

Key supportive observations include perfusion MRI patterns: CPA shows increased blood volume with prolonged mean transit time and broader hemispheric hypoperfusion compared with classical AVMs (lasjaunias2008cerebralproliferativeangiopathy pages 6-7).

6.2 Pathology (human)

CPA differs from classic AVM histology by preservation of neural tissue within the lesion; pathology shows vascular wall abnormalities and “normal appearing neural tissue intermingled between these vascular channels” (lasjaunias2008cerebralproliferativeangiopathy pages 4-6).

6.3 Suggested GO biological process terms (examples)

• Angiogenesis (GO:0001525)
• Response to hypoxia (GO:0001666)
• Regulation of vascular permeability (GO:0043114)
• Endothelial cell proliferation (GO:0001935)

6.4 Suggested Cell Ontology (CL) terms (examples)

• Endothelial cell (CL:0000115)
• Vascular smooth muscle cell (CL:0000192)
• Pericyte (CL:0000669)

7. Anatomical structures affected

7.1 Organ/system level

• Primary: brain (central nervous system) (lasjaunias2008cerebralproliferativeangiopathy pages 2-4, singfer2023cerebralproliferativeangiopathy pages 1-4).

7.2 Localization patterns (imaging-derived)

Lesions are often large and may span lobes/hemisphere; in one pooled review, hemispheric extension was reported in 73.0% (yamaki2020thecurrentclinical pages 3-4). Watershed-zone predominance (70.6%) supports a hypoperfusion-linked distribution (yamaki2020thecurrentclinical pages 3-4).

7.3 Suggested UBERON terms (examples)

• Brain (UBERON:0000955)
• Cerebral cortex (UBERON:0000956)
• Cerebral hemisphere (UBERON:0002812)
• Basal ganglia (UBERON:0002532)
• Thalamus (UBERON:0001897)

8. Temporal development (onset and progression)

8.1 Typical onset pattern

CPA is usually recognized in young patients, often after neurologic symptoms (seizures, headache, TIAs) rather than hemorrhage (lasjaunias2008cerebralproliferativeangiopathy pages 2-4, yamaki2020thecurrentclinical pages 3-4).

8.2 Evidence for dynamic evolution

A 2023 pediatric case report described diagnosis of CPA five years after intraventricular hemorrhage and an initially negative catheter angiogram, interpreted as supportive of new-vessel formation/angiogenesis as part of disease evolution (singfer2023cerebralproliferativeangiopathy pages 1-4).

9. Inheritance and population

9.1 Epidemiology (descriptive)

CPA is a minority subset among lesions labeled AVM in major series: - Lasjaunias et al.: 49/1434 (3.4%) of AVM databank cases met CPA criteria (lasjaunias2008cerebralproliferativeangiopathy pages 2-4).

9.2 Demographics

• Lasjaunias et al.: mean age 22, female 67% (lasjaunias2008cerebralproliferativeangiopathy pages 2-4).
• Systematic review (95 cases): mean age 23, female 60% (yamaki2020thecurrentclinical pages 3-4).

9.3 Inheritance

No inheritance pattern has been established in the accessed evidence base (yamaki2020thecurrentclinical pages 1-3, lasjaunias2008cerebralproliferativeangiopathy pages 6-7).

10. Diagnostics

10.1 Core diagnostic modalities

Digital subtraction angiography (DSA) is emphasized as the diagnostic reference standard for angioarchitecture (tiwari2020cerebralproliferativeangiopathy pages 3-4, yamaki2020thecurrentclinical pages 1-3).

Key diagnostic imaging features include: - Diffuse vascular network with intermingled normal brain parenchyma (lasjaunias2008cerebralproliferativeangiopathy pages 2-4, singfer2023cerebralproliferativeangiopathy pages 1-4). - Discrepancy between large lesion size and small shunting volume (lasjaunias2008cerebralproliferativeangiopathy pages 1-2). - No dominant arterial feeder, many small feeders; small draining veins relative to lesion (lasjaunias2008cerebralproliferativeangiopathy pages 1-2, lasjaunias2008cerebralproliferativeangiopathy pages 4-6). - Transdural supply and proximal feeder stenoses (lasjaunias2008cerebralproliferativeangiopathy pages 2-4, yamaki2020thecurrentclinical pages 3-4).

Perfusion/functional hemodynamic testing may support a steal/hypoperfusion mechanism. - A 2024 case report used CT perfusion with acetazolamide challenge to attribute aphasia to steal physiology (gautam2024thetreatmentof pages 1-2, gautam2024thetreatmentof pages 2-4).

10.2 Differential diagnosis

Commonly discussed differentials include: - Classic brain AVM (compact nidus, higher hemorrhagic presentation proportion) (yamaki2020thecurrentclinical pages 3-4, lasjaunias2008cerebralproliferativeangiopathy pages 1-2). - Moyamoya disease / moyamoya-like vasculopathies (noted as differential in case literature) (jongaliem2023useofbetablocker pages 1-2).

10.3 Visual evidence (representative MRI + DSA)

A 2023 case report provides representative MRI and DSA figures showing a diffuse vascular network with persistent opacification into late arterial/early venous phases and no dominant feeders (singfer2023cerebralproliferativeangiopathy media 68b06269, singfer2023cerebralproliferativeangiopathy media 94c84d95).

11. Outcome / prognosis

11.1 Hemorrhage risk and rebleeding

CPA generally presents with hemorrhage less often than classical AVMs, but recurrence risk after hemorrhage may be high. - Lasjaunias cohort: hemorrhage at presentation 12% (6/49); among those with hemorrhage, recurrent bleeding reported in 67%, with one death (lasjaunias2008cerebralproliferativeangiopathy pages 2-4). - Systematic review: hemorrhage in 18% and reported rebleeding up to 67% in some series (yamaki2020thecurrentclinical pages 3-4).

11.2 Functional outcomes (systematic reviews)

• Systematic review (95 cases): improved 50.7%, stable 40.2%, worsened 9.0% at mean follow-up ~17 months (yamaki2020thecurrentclinical pages 3-4).
• Another systematic review (84 patients; 78 with outcomes): conservative management led to spontaneous improvement 39.7%, worsening 46.1%, no change 12.8% (hess2022cerebralproliferativeangiopathy pages 3-4).

12. Treatment

12.1 Overarching strategy (expert synthesis)

Because normal brain tissue is interspersed with abnormal vessels, aggressive curative therapies used for compact AVMs may cause unacceptable neurologic injury. Lasjaunias et al. explicitly caution that “normal brain is interspersed with the abnormal vascular channels increasing the risk of neurological deficit in aggressive treatments,” and given “low risk of hemorrhage,” aggressive treatment is often not indicated (lasjaunias2008cerebralproliferativeangiopathy pages 1-2).

12.2 Real-world treatment utilization (systematic review data)

From Yamaki et al. (95 cases): conservative 54.4%, endovascular 34.2%, indirect revascularization 7.6%, radiosurgery 2.5%, decompression 1.3% (yamaki2020thecurrentclinical pages 5-7).

From Hess et al. (84 patients): conservative 59.5%, embolization 28.6% (hess2022cerebralproliferativeangiopathy pages 3-4).

12.3 Endovascular therapy (targeted/partial embolization)

Targeted embolization is discussed as a selective strategy (e.g., fragile angioarchitecture or hemorrhagic foci) rather than curative obliteration, due to risk to intermingled parenchyma (yamaki2020thecurrentclinical pages 3-4, lasjaunias2008cerebralproliferativeangiopathy pages 1-2).

12.4 Surgical / revascularization approaches

A systematic review focused on revascularization summarizes the rationale: revascularization is proposed to “disrupt regional hypoperfusion and interrupt the angiogenesis that defines CPA,” with early small-series results favorable but limited (hess2022cerebralproliferativeangiopathy pages 3-4).

Pediatric aggregation emphasizes indirect revascularization (e.g., pial synangiosis, burr-hole dural inversion) for symptomatic hypoperfusion, with reported durable collateralization and functional gains in illustrative follow-up (moskalik2026cerebralproliferativeangiopathy pages 1-3).

12.5 Emerging medical therapies (prioritize 2023–2024)

(a) Propranolol (beta-blocker) – 2023 case report A 2023 report describes long-term propranolol use for disabling headaches with reported angiographic “shrinkage of the vascular network” over 7 years, hypothesized via VEGF inhibition/antiproliferative effects; this remains anecdotal evidence (jongaliem2023useofbetablocker pages 2-4).

(b) Cilostazol (vasodilating agent) – 2024 case report A 2024 case report (Diffuse Proliferative Cerebral Angiopathy) reported rapid symptomatic improvement of aphasia after cilostazol in a steal-phenomenon phenotype confirmed by perfusion/acetazolamide challenge. The abstract states: “Within three days of treatment with cilostazol, the patient showed significant improvement in his aphasia.” (gautam2024thetreatmentof pages 1-2). Quantitative perfusion metrics (Tmax>4s and rCBF<38% volumes) improved after acetazolamide and over follow-up (gautam2024thetreatmentof pages 2-4).

12.6 MAXO term suggestions (examples)

• Endovascular embolization procedure (MAXO:0000508; suggest)
• Cerebral revascularization (MAXO:0000463; suggest)
• Antiepileptic drug therapy (MAXO:0000635; suggest)
• Beta-adrenergic blocker therapy (MAXO:0000955; suggest)
• Antiplatelet therapy (MAXO:0000645; suggest)

12.7 Clinical trials

A ClinicalTrials.gov search using CPA terms did not identify CPA-specific interventional trials in the retrieved results; the returned trials were unrelated (diabetes microangiopathy) (tool result; no CPA trial context IDs available for citation).

13. Prevention

No primary-prevention interventions (vaccines, exposure avoidance) are described for CPA in the accessed literature. Prevention is best framed as: - Secondary prevention: avoiding misclassification as classic AVM and avoiding harmful aggressive AVM-directed eradication attempts (lasjaunias2008cerebralproliferativeangiopathy pages 1-2, singfer2023cerebralproliferativeangiopathy pages 1-4). - Tertiary prevention: seizure control, headache management, and individualized management of hypoperfusion/steal phenomena (jongaliem2023useofbetablocker pages 1-2, gautam2024thetreatmentof pages 2-4).

14. Other species / natural disease

No natural disease analogs in non-human species were identified in the accessed literature.

15. Model organisms

No CPA-specific animal models, cellular models, or iPSC/organoid models were identified in the accessed literature.

Summary artifact

The following table consolidates key quantitative and qualitative disease facts from the strongest available evidence (foundational cohort + systematic reviews + recent case reports).

Table: This table summarizes the most clinically relevant facts about cerebral proliferative angiopathy, including how it is defined, how it presents, how it is diagnosed on imaging, and how it is managed. It is useful as a concise evidence-backed reference for distinguishing CPA from classical brain AVM.

Key references (with publication dates and URLs)

• Lasjaunias PL et al. 2008-03. Stroke. “Cerebral proliferative angiopathy…” https://doi.org/10.1161/STROKEAHA.107.493080 (lasjaunias2008cerebralproliferativeangiopathy pages 2-4)
• Yamaki VN et al. 2020-03. Acta Neurochirurgica. “The current clinical picture…” https://doi.org/10.1007/s00701-020-04289-7 (yamaki2020thecurrentclinical pages 3-4)
• Hess RM et al. 2022-08. World Neurosurgery. “CPA presenting as subdural hematoma…” https://doi.org/10.1016/j.wneu.2022.05.096 (hess2022cerebralproliferativeangiopathy pages 3-4)
• Singfer U et al. 2023-08. J Belgian Soc Radiol. “CPA in a child…” https://doi.org/10.5334/jbsr.3247 (singfer2023cerebralproliferativeangiopathy pages 1-4)
• Jong-A-Liem GS et al. 2023-03. Interdisciplinary Neurosurgery. “Use of beta-blocker…” https://doi.org/10.1016/j.inat.2022.101663 (jongaliem2023useofbetablocker pages 2-4)
• Gautam D et al. 2024-06. Cureus. “Treatment… with cilostazol” https://doi.org/10.7759/cureus.63387 (gautam2024thetreatmentof pages 1-2)

References

1. (yamaki2020thecurrentclinical pages 1-3): Vitor Nagai Yamaki, Davi Jorge Fontoura Solla, João Paulo Mota Telles, Glaucia Lexy Jong Liem, Saul Almeida da Silva, José Guilherme Mendes Pereira Caldas, Manoel Jacobsen Teixeira, Eric Homero Albuquerque Paschoal, and Eberval Gadelha Figueiredo. The current clinical picture of cerebral proliferative angiopathy: systematic review. Acta Neurochirurgica, 162:1727-1733, Mar 2020. URL: https://doi.org/10.1007/s00701-020-04289-7, doi:10.1007/s00701-020-04289-7. This article has 25 citations and is from a peer-reviewed journal.

2. (tiwari2020cerebralproliferativeangiopathy pages 1-3): Sarbesh Tiwari, Pawan K. Garg, Pushpinder S. Khera, Santhosh Babu, Binit Sureka, and Taruna Yadav. Cerebral proliferative angiopathy: an uncommon and misdiagnosed entity. Journal of Clinical Interventional Radiology ISVIR, 4:107-110, Feb 2020. URL: https://doi.org/10.1055/s-0039-3401329, doi:10.1055/s-0039-3401329. This article has 7 citations.

3. (lasjaunias2008cerebralproliferativeangiopathy pages 1-2): Pierre L. Lasjaunias, Pierre Landrieu, Georges Rodesch, Hortensia Alvarez, Augustin Ozanne, Staffan Holmin, Wen-Yuan Zhao, Sasikhan Geibprasert, Dennis Ducreux, and Timo Krings. Cerebral proliferative angiopathy: clinical and angiographic description of an entity different from cerebral avms. Stroke, 39:878-885, Mar 2008. URL: https://doi.org/10.1161/strokeaha.107.493080, doi:10.1161/strokeaha.107.493080. This article has 174 citations and is from a highest quality peer-reviewed journal.

4. (lasjaunias2008cerebralproliferativeangiopathy pages 2-4): Pierre L. Lasjaunias, Pierre Landrieu, Georges Rodesch, Hortensia Alvarez, Augustin Ozanne, Staffan Holmin, Wen-Yuan Zhao, Sasikhan Geibprasert, Dennis Ducreux, and Timo Krings. Cerebral proliferative angiopathy: clinical and angiographic description of an entity different from cerebral avms. Stroke, 39:878-885, Mar 2008. URL: https://doi.org/10.1161/strokeaha.107.493080, doi:10.1161/strokeaha.107.493080. This article has 174 citations and is from a highest quality peer-reviewed journal.

5. (tiwari2020cerebralproliferativeangiopathy pages 3-4): Sarbesh Tiwari, Pawan K. Garg, Pushpinder S. Khera, Santhosh Babu, Binit Sureka, and Taruna Yadav. Cerebral proliferative angiopathy: an uncommon and misdiagnosed entity. Journal of Clinical Interventional Radiology ISVIR, 4:107-110, Feb 2020. URL: https://doi.org/10.1055/s-0039-3401329, doi:10.1055/s-0039-3401329. This article has 7 citations.

6. (lasjaunias2008cerebralproliferativeangiopathy pages 6-7): Pierre L. Lasjaunias, Pierre Landrieu, Georges Rodesch, Hortensia Alvarez, Augustin Ozanne, Staffan Holmin, Wen-Yuan Zhao, Sasikhan Geibprasert, Dennis Ducreux, and Timo Krings. Cerebral proliferative angiopathy: clinical and angiographic description of an entity different from cerebral avms. Stroke, 39:878-885, Mar 2008. URL: https://doi.org/10.1161/strokeaha.107.493080, doi:10.1161/strokeaha.107.493080. This article has 174 citations and is from a highest quality peer-reviewed journal.

7. (yamaki2020thecurrentclinical pages 3-4): Vitor Nagai Yamaki, Davi Jorge Fontoura Solla, João Paulo Mota Telles, Glaucia Lexy Jong Liem, Saul Almeida da Silva, José Guilherme Mendes Pereira Caldas, Manoel Jacobsen Teixeira, Eric Homero Albuquerque Paschoal, and Eberval Gadelha Figueiredo. The current clinical picture of cerebral proliferative angiopathy: systematic review. Acta Neurochirurgica, 162:1727-1733, Mar 2020. URL: https://doi.org/10.1007/s00701-020-04289-7, doi:10.1007/s00701-020-04289-7. This article has 25 citations and is from a peer-reviewed journal.

8. (hess2022cerebralproliferativeangiopathy pages 3-4): Ryan M. Hess, Jeff F. Zhang, Justin M. Cappuzzo, Amade Bregy, and Elad I. Levy. Cerebral proliferative angiopathy presenting as subdural hematoma: a case report and systematic literature review. World Neurosurgery, 164:281-289, Aug 2022. URL: https://doi.org/10.1016/j.wneu.2022.05.096, doi:10.1016/j.wneu.2022.05.096. This article has 5 citations and is from a peer-reviewed journal.

9. (jongaliem2023useofbetablocker pages 1-2): Glaucia Suzanna Jong-A-Liem, Lillian dos Santos Carneiro, Fernando Mendes Paschoal Junior, Feres Eduardo Aparecido Chaddad Neto, Eberval Figueiredo Gadelha, E. Bor-Seng-Shu, and Eric Homero Albuquerque Paschoal. Use of beta-blocker in cerebral proliferative angiopathy: a case report. Interdisciplinary Neurosurgery, 31:101663, Mar 2023. URL: https://doi.org/10.1016/j.inat.2022.101663, doi:10.1016/j.inat.2022.101663. This article has 1 citations and is from a peer-reviewed journal.

10. (gautam2024thetreatmentof pages 2-4): Diwas Gautam, Daryl E Morrison, Michael T. Bounajem, Lubdha M. Shah, and Ramesh Grandhi. The treatment of symptomatic diffuse proliferative cerebral angiopathy with cilostazol: a case report. Cureus, Jun 2024. URL: https://doi.org/10.7759/cureus.63387, doi:10.7759/cureus.63387. This article has 2 citations.

11. (jongaliem2023useofbetablocker pages 2-4): Glaucia Suzanna Jong-A-Liem, Lillian dos Santos Carneiro, Fernando Mendes Paschoal Junior, Feres Eduardo Aparecido Chaddad Neto, Eberval Figueiredo Gadelha, E. Bor-Seng-Shu, and Eric Homero Albuquerque Paschoal. Use of beta-blocker in cerebral proliferative angiopathy: a case report. Interdisciplinary Neurosurgery, 31:101663, Mar 2023. URL: https://doi.org/10.1016/j.inat.2022.101663, doi:10.1016/j.inat.2022.101663. This article has 1 citations and is from a peer-reviewed journal.

12. (moskalik2026cerebralproliferativeangiopathy pages 1-3): Anzhela D. Moskalik, Jonathan Mo, Monifa Sawyerr, Marike Zwienenberg, Branden Cord, and Julia D. Sharma. Cerebral proliferative angiopathy in pediatric patients: case-based review with an illustrative case. Child's Nervous System, Feb 2026. URL: https://doi.org/10.1007/s00381-026-07129-8, doi:10.1007/s00381-026-07129-8. This article has 0 citations.

13. (lasjaunias2008cerebralproliferativeangiopathy pages 4-6): Pierre L. Lasjaunias, Pierre Landrieu, Georges Rodesch, Hortensia Alvarez, Augustin Ozanne, Staffan Holmin, Wen-Yuan Zhao, Sasikhan Geibprasert, Dennis Ducreux, and Timo Krings. Cerebral proliferative angiopathy: clinical and angiographic description of an entity different from cerebral avms. Stroke, 39:878-885, Mar 2008. URL: https://doi.org/10.1161/strokeaha.107.493080, doi:10.1161/strokeaha.107.493080. This article has 174 citations and is from a highest quality peer-reviewed journal.

14. (singfer2023cerebralproliferativeangiopathy pages 1-4): Uri Singfer, Edward Baert, and Luc Defreyne. Cerebral proliferative angiopathy in a child, five years after an intraventricular hemorrhage and negative catheter angiography. Journal of the Belgian Society of Radiology, Aug 2023. URL: https://doi.org/10.5334/jbsr.3247, doi:10.5334/jbsr.3247. This article has 5 citations.

15. (gautam2024thetreatmentof pages 1-2): Diwas Gautam, Daryl E Morrison, Michael T. Bounajem, Lubdha M. Shah, and Ramesh Grandhi. The treatment of symptomatic diffuse proliferative cerebral angiopathy with cilostazol: a case report. Cureus, Jun 2024. URL: https://doi.org/10.7759/cureus.63387, doi:10.7759/cureus.63387. This article has 2 citations.

16. (singfer2023cerebralproliferativeangiopathy media 68b06269): Uri Singfer, Edward Baert, and Luc Defreyne. Cerebral proliferative angiopathy in a child, five years after an intraventricular hemorrhage and negative catheter angiography. Journal of the Belgian Society of Radiology, Aug 2023. URL: https://doi.org/10.5334/jbsr.3247, doi:10.5334/jbsr.3247. This article has 5 citations.

17. (singfer2023cerebralproliferativeangiopathy media 94c84d95): Uri Singfer, Edward Baert, and Luc Defreyne. Cerebral proliferative angiopathy in a child, five years after an intraventricular hemorrhage and negative catheter angiography. Journal of the Belgian Society of Radiology, Aug 2023. URL: https://doi.org/10.5334/jbsr.3247, doi:10.5334/jbsr.3247. This article has 5 citations.

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