title: Neurovascular complications of marfan syndrome: a retrospective, hospital-based study.
abstract: OBJECTIVE
  Small case series have associated Marfan syndrome with cerebral and spinal ischemia or hemorrhage. However, there has been no investigation of the frequency and etiology of neurovascular disorders in a large series of Marfan patients.
  
  
  METHODS
  We conducted a retrospective, hospital-based study of all Marfan syndrome patients seen in an 8-year period. Records were reviewed in detail, and clinical characteristics of those with and without a neurovascular diagnosis compared.
  
  
  RESULTS
  Of 513 patients, 18 (3.5%) had a neurovascular diagnosis, as follows: transient ischemic attack (11), cerebral infarction (2), spinal cord infarction (2), subdural hematoma (2), and spinal subarachnoid hemorrhage (1). A cardioembolic source was identified in 12 of 13 patients with cerebral ischemia, as follows: prosthetic heart valves (9), mitral valve prolapse (2), and atrial fibrillation (1). Chronic anticoagulant therapy was a likely cause in 2 of 3 patients with hemorrhagic events. Compared with other Marfan syndrome patients, those with neurovascular events were older (39.6 versus 31.7 years, P=0.04) and more likely to be in atrial fibrillation (22.2% versus 3.2%, P=<0.01), to have prosthetic heart valves (61.1% versus 7.7%, P=0.001), and to be taking anticoagulant therapy (72.2% versus 16.1%, P<0.001). Aortic disease, a putative factor in the etiology of neurovascular complications, was present in equal measure in Marfan patients with and without neurovascular complications (78% versus 65%, P=NS).
  
  
  CONCLUSIONS
  Neurovascular complications of Marfan syndrome are rare during 8 years of follow-up, and generally are ischemic in nature. A high-risk cardiac source was identified in the majority. A significant association with vascular dissection was not established.
authors.affiliation: Department of Neurology, Johns Hopkins Hospital, Baltimore, Md, USA. rwityk@jhmi.edu
authors.forename: Robert J
authors.initials: RJ
authors.lastname: Wityk
authors.forename: Carla
authors.initials: C
authors.lastname: Zanferrari
authors.forename: Stephen
authors.initials: S
authors.lastname: Oppenheimer
mesh_terms: D000293:Adolescent; D000328:Adult; D017677:Age Distribution; D000368:Aged; D000369:Aged, 80 and over; D015984:Causality; D002561:Cerebrovascular Disorders / Q000175:diagnosis / Q000453:epidemiology*; D002648:Child; D002675:Child, Preschool; D015897:Comorbidity; D005260:Female; D005500:Follow-Up Studies; D006761:Hospitals / Q000706:statistics & numerical data*; D006801:Humans; D007223:Infant; D002546:Ischemic Attack, Transient / Q000175:diagnosis / Q000453:epidemiology; D008297:Male; D008382:Marfan Syndrome / Q000175:diagnosis / Q000453:epidemiology*; D008875:Middle Aged; D012189:Retrospective Studies; D012307:Risk Factors; D017678:Sex Distribution; D020758:Spinal Cord Vascular Diseases / Q000175:diagnosis / Q000453:epidemiology*