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Original Investigation
May 8, 2017

Population-Based Prevalence of Cerebral Cavernous Malformations in Older AdultsMayo Clinic Study of Aging

Author Affiliations
  • 1Department of Neurology, Mayo Clinic, Rochester, Minnesota
  • 2Department of Health Sciences Research, Mayo Clinic, Rochester, Minnesota
  • 3Department of Radiology, Mayo Clinic, Rochester, Minnesota
  • 4Department of Neurosurgery, Mayo Clinic, Rochester, Minnesota
JAMA Neurol. Published online May 8, 2017. doi:10.1001/jamaneurol.2017.0439
Key Points

Question  What is the prevalence of symptomatic and asymptomatic cerebral cavernous malformations in older adults?

Findings  In this population-based study of 2715 participants aged 50 to 89 years who underwent brain magnetic resonance imaging for nonclinical purposes, the overall prevalence of cerebral cavernous malformation was 0.46%, and the observed frequency of symptomatic cerebral cavernous malformation was 0.037%.

Meaning  Because symptomatic cerebral cavernous malformations are rare, multicenter involvement will be necessary for future clinical trials.


Importance  The prevalence of cerebral cavernous malformation (CCM) is unknown. Case ascertainment in most previous studies was based on autopsy data or clinical convenience samples, often without detailed clinical or radiologic information.

Objective  To determine the prevalence of CCM in a population-based sample of older adults.

Design, Setting, and Participants  This prospective imaging study included 4721 participants aged 50 to 89 years who were enrolled between January 1, 2004, and December 15, 2015, in the Mayo Clinic Study of Aging, a longitudinal, population-based study of residents of Olmsted County, Minnesota. An age- and sex-stratified sampling strategy was used to randomly select participants from Olmsted County using the medical records linkage system of the Rochester Epidemiology Project. Participants were invited to undergo brain magnetic resonance imaging (MRI). Of the 4721 participants, 2715 had an evaluable MRI. All images were reviewed by a board-certified neuroradiologist, and MRI reports were searched for the terms cavernous malformation, cavernous angioma, and cavernoma. Two vascular neurologists reviewed MRIs, and potential CCMs were classified using Zabramski classification. Medical records of the identified individuals with CCM were reviewed along with their demographic information, medical history, and any symptoms referable to the identified CCM lesion.

Main Outcomes and Measures  Prevalence of CCM and clinical and radiologic characteristics of study participants with CCM.

Results  Of the 2715 participants who underwent MRI scans, 12 (0.44%) had CCM. With the use of inverse probability weights to adjust for participation bias, the overall prevalence was 0.46% (95% CI, 0.05-0.86). The age-adjusted prevalence was found to be 0.61% (95% CI, 0-1.47) for the 50- to 59-year age group, 0.17% (95% CI, 0-0.50) for the 60- to 69-year age group, 0.45% (95% CI, 0.09-0.81) for the 70- to 79-year age group, and 0.58% (95% CI, 0-1.29) for the 80- to 89-year age group. The sex-adjusted prevalence was 0.41% (95% CI, 0-1.00) for women and 0.51% (95% CI, 0-1.07) for men. Observed frequencies were similar in men and women, with a slight male predominance. Of the 12 participants with CCM, 9 (75%) had a single Zabramski type 2 lesion in a supratentorial location. Only 1 participant (0.037%) was symptomatic from the CCM during the study period.

Conclusions and Relevance  The findings and data from this study are important for determining the potential number of patients available for cohort studies and anticipated clinical trials in older patients with CCM.