Neurovascular complications of marfan syndrome: a retrospective, hospital-based study.
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.
Wityk
Robert J
RJ
Department of Neurology, Johns Hopkins Hospital, Baltimore, Md, USA. rwityk@jhmi.edu
Zanferrari
Carla
C
Oppenheimer
Stephen
S
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*