Neurovascular manifestations of connective-tissue diseases: A review

Sarasa T. Kim, Waleed Brinjikji, Giuseppe Lanzino, David F. Kallmes

Research output: Contribution to journalArticlepeer-review

29 Scopus citations


Patients with connective tissue diseases are thought to be at a higher risk for a number of cerebrovascular diseases such as intracranial aneurysms, dissections, and acute ischemic strokes. In this report, we aim to understand the prevalence and occurrences of such neurovascular manifestations in four heritable connective tissue disorders: Marfan syndrome, Ehlers-Danlos syndrome, Neurofibromatosis Type 1, and Loeys-Dietz syndrome. We discuss the fact that although there are various case studies reporting neurovascular findings in these connective tissue diseases, there is a general lack of case-control and prospective studies investigating the true prevalence of these findings in these patient populations. Furthermore, the differences observed in the manifestations and histology of such disease pathologies encourages future multi-center registries and studies in better characterizing the pathophysiology, prevalence, and ideal treatment options of neurovascular lesions in patents with connective tissue diseases.

Original languageEnglish (US)
Pages (from-to)624-637
Number of pages14
JournalInterventional Neuroradiology
Issue number6
StatePublished - Dec 1 2016


  • Ehlers-Danlos syndrome
  • Loeys-Dietz syndrome
  • Marfan syndrome
  • dissection
  • intracranial aneurysm
  • neurofibromatosis type 1 (NF1)

ASJC Scopus subject areas

  • Radiology Nuclear Medicine and imaging
  • Clinical Neurology
  • Cardiology and Cardiovascular Medicine


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