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Cerebral Proliferative Angiopathy (CPA)

Summary

  • Rare vascular malformation characterised by diffuse network of abnormal vessels
  • Distinct from classical arteriovenous malformations (AVMs)
  • Presents with headaches, seizures, or focal neurological deficits

Pathophysiology

  • Diffuse vascular abnormality with interposed normal brain parenchyma
  • Angiogenesis likely plays a key role in pathogenesis
  • Lack of large arteriovenous shunts, unlike classical AVMs
  • Associated with chronic cerebral ischaemia and neoangiogenesis

Demographics

  • Rare condition, exact prevalence unknown
  • Typically affects younger patients (mean age 22 years)
  • Female predominance (2:1 female to male ratio)
  • No clear genetic or hereditary component identified

Diagnosis

  • Clinical presentation:
    • Headaches (most common)
    • Seizures
    • Focal neurological deficits
    • Rarely, intracranial haemorrhage
  • Differential diagnosis:
    • Classical AVM
    • Moyamoya disease
    • Sturge-Weber syndrome
  • Diagnosis confirmed by characteristic imaging findings

Imaging

  • CT:
    • Diffuse, enhancing vascular network
    • Absence of large nidus or dominant feeding arteries
  • MRI:
    • T1: Diffuse hypointense signal
    • T2: Flow voids and hyperintense interposed brain tissue
    • Susceptibility-weighted imaging (SWI): Prominent vascular network
  • Angiography:
    • "Puffy, cloudy" appearance of abnormal vessels
    • Absence of early venous drainage
    • Capillary blush in late arterial phase
  • Perfusion studies:
    • Decreased cerebral blood flow in affected regions
    • Increased mean transit time and time to peak

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  • 20 year old paitent presented with headaches.
  • Angiography showed an ill-defined nidus in the right cerebral hemisphere supplied by the anterior circulation.
  • Imaging modified from Shomura et al1.

Treatment

  • Conservative management preferred due to diffuse nature of lesion
  • Medical management:
    • Antiepileptic drugs for seizure control
    • Analgesics for headache management
  • Surgical intervention generally not recommended due to high risk
  • Targeted endovascular embolisation for specific symptoms:
    • Focal seizures
    • Progressive neurological deficits
  • Stereotactic radiosurgery:
    • Limited role due to diffuse nature of lesion
    • May be considered for small, focal areas of abnormality
  • Regular follow-up and monitoring:
    • Clinical assessment
    • Neuroimaging to evaluate progression

Differential diagnosis

Differential Diagnosis Distinguishing Feature
Arteriovenous Malformation (AVM) CPA lacks a dominant feeding artery or nidus; diffuse vascular network
Moyamoya Disease CPA does not show typical "puff of smoke" appearance; lacks progressive stenosis of internal carotid arteries
Sturge-Weber Syndrome CPA lacks facial port-wine stain and leptomeningeal angiomatosis
Hereditary Haemorrhagic Telangiectasia CPA does not show multiple small AVMs or telangiectasias in other organs
Cerebral Cavernous Malformation CPA shows flow voids on MRI, unlike the "popcorn" appearance of cavernomas
Dural Arteriovenous Fistula CPA lacks direct arteriovenous shunting and venous hypertension
Capillary Telangiectasia CPA has more extensive vascular network and larger affected area
Glioma with Vascular Proliferation CPA lacks solid tumour component and infiltrative growth pattern
Vein of Galen Malformation CPA does not involve the deep venous system or show aneurysmal dilatation of the vein of Galen
Sinus Pericranii CPA is intraparenchymal, while sinus pericranii is extracranial

  1. Shomura et al. Multiple Endovascular Treatments for Haemorrhagic Cerebral Proliferative Angiopathy: A Case Report. 2022. Case Reports in Neurology - Open in new tab. ↩