Severely impaired cerebrovascular reserve in patients with cerebral proliferative angiopathy

J Neurosurg Pediatr. 2011 Sep;8(3):310-5. doi: 10.3171/2011.6.PEDS1170.


Object: Cerebral proliferative angiopathy (CPA) has been morphologically distinguished from classically appearing brain arteriovenous malformations (AVMs) by exhibition of functional brain parenchyma that is intermingled with abnormal vascular channels. The presence of oligemia in this intralesional brain tissue may suggest ischemia, which is not detected in classic brain AVMs. The authors hypothesized that patients with CPA would exhibit a greater impairment of cerebrovascular reserve in neuronal tissue surrounding the true nidus compared with those with brain AVMs.

Methods: Four patients with CPA, 10 patients with brain AVMs and seizures, and 12 young healthy individuals were studied. The 4 patients with CPA underwent blood oxygen level-dependent MR imaging examinations while applying normoxic step changes in end-tidal CO(2) to obtain quantitative cerebrovascular reactivity measurements.

Results: Patients with a CPA lesion exhibited severely impaired perilesional cerebrovascular reserve in comparison with patients with brain AVMs and seizures (0.10 ± 0.03 vs 0.16 ± 0.03, respectively; p < 0.05), and young healthy individuals (0.10 ± 0.03 vs 0.21 ± 0.06, respectively; p < 0.01).

Conclusions: This study demonstrated severely impaired cerebrovascular reserve in the perilesional brain tissue surrounding the abnormal vessels of patients with CPA. This finding may provide an additional means to distinguish CPA from classic brain AVMs.

MeSH terms

  • Brain / blood supply
  • Brain / pathology
  • Brain Ischemia / diagnosis
  • Brain Ischemia / etiology
  • Carbon Dioxide / metabolism
  • Cerebrovascular Circulation / physiology*
  • Child
  • Diagnosis, Differential
  • Epilepsy / diagnosis*
  • Epilepsy / etiology
  • Female
  • Humans
  • Intracranial Arteriovenous Malformations / classification*
  • Intracranial Arteriovenous Malformations / complications
  • Intracranial Arteriovenous Malformations / diagnosis*
  • Magnetic Resonance Angiography*
  • Magnetic Resonance Imaging
  • Male
  • Severity of Illness Index
  • Young Adult


  • Carbon Dioxide