title: Neurovascular manifestations of connective-tissue diseases: A review.
abstract: 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.
authors: Mayo Clinic, Mayo Medical School, USA.
authors: Sarasa T
authors: ST
authors: Kim
authors: Mayo Clinic, Department of Radiology, USA brinjikji.waleed@mayo.edu.
authors: Waleed
authors: W
authors: Brinjikji
authors: Mayo Clinic, Department of Neurologic Surgery, USA.
authors: Giuseppe
authors: G
authors: Lanzino
authors: Mayo Clinic, Department of Radiology, USA.
authors: David F
authors: DF
authors: Kallmes
mesh_terms: D003240:Connective Tissue Diseases / Q000150:complications*; D004535:Ehlers-Danlos Syndrome / Q000150:complications; D006801:Humans; D002532:Intracranial Aneurysm / Q000209:etiology* / Q000628:therapy*; D055947:Loeys-Dietz Syndrome / Q000150:complications / Q000628:therapy; D008382:Marfan Syndrome / Q000150:complications; D009422:Nervous System Diseases / Q000209:etiology*; D014652:Vascular Diseases / Q000209:etiology*
keywords: Ehlers–Danlos syndrome; Loeys–Dietz syndrome; Marfan syndrome; dissection; intracranial aneurysm; neurofibromatosis type 1 (NF1)