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Cerebral Amyloid Angiopathy (CAA)/Amyloidoma

Last Updated: October 1, 2018

Open Table of Contents: Cerebral Amyloid Angiopathy (CAA)/Amyloidoma

Figure 1: These images demonstrate T1 hypointense (top row left), FLAIR hyperintense (top row right) signal within the left parietal white matter radiating medially to the ventricular wall with irregular, somewhat serpiginous enhancement (middle row left). There was no reduction in diffusivity on DWI/ADC images (middle row right and bottom row).

Description

  • Cerebrovascular disorder characterized by the deposition of amyloid protein in the media and adventitia of small and medium-sized vessels of the cerebral cortex, subcortical white matter and leptomeninges

Pathology

  • Unclear
  • Thought to be related to a benign clonal proliferation of plasma cells
  • Three varieties
    • Cerebral amyloid angiopathy, amyloidoma, rare inflammatory subtype

Clinical Features

  • Symptoms
    • Seizures, headache, focal motor signs, and cognitive decline
  • Age

    • Angiopathy fairly common in elderly
    • Amyloidoma found in younger patients with mean age of 48
  • Gender

    • Slightly more common in women (8:5)
  • Associations

    • 40% of patients with CAA have Alzheimer’s disease

Imaging

  • General
    • Focal nonhemorrhagic masses
    • Mass effect generally minimal/mild
    • May show moderate/striking enhancement
    • Often extends medially to lateral ventricular wall with fine radial enhancing margins
    • Radiating pattern from the ventricular wall may help in the diagnosis
  • Modality Specific

    • CT
      • Hyperdense, enhances
    • MR

      • T1
        • Variable appearance
      • T2

        • Heterogeneous, though more commonly hyperintense
      • Contrast

        • Variable
  • Mimic

    • Classic amyloid angiopathy can mimic treated or hemorrhagic metastases. The less common amyloidoma can mimic a neoplasm, with or without hemorrhage. The radiating pattern from the ventricular wall when present can help in differentiating, though this is a fairly nonspecific finding.

For more information, please see the corresponding chapter in Radiopaedia.

Contributor: Sean Dodson, MD

DOI: https://doi.org/10.18791/nsatlas.v1.03.02.08

References

Chao CP, et al. Cerebral Amyloid Angiopathy: CT and MR Imaging Findings. Radiographics. 2006; 26(5):1517-31.

Galluci M, et al. Neuroradiological findings in two cases of isolated amyloidoma of the central nervous system. Neuroradiology 2002; 44(4):333-337.

Gandhi D, et al. CT and MR Imaging of Intracerebral Amyloidoma: Case Report and Review of the Literature. Am J Neuroradiol. 2003; 24:519-522.

Haacke EM, et al. Imaging Cerebral Amyloid Angiopathy with Susceptibility-Weighted Imaging. AJNR. 2007; 28:316-17.

Landau D, et al. Cerebral amyloidoma mimicking intracranial tumor: a case report. Journal of Medical Case Reports. 2010; 20;4:308.

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