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Stroke. 2000;31:2734-2741

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(Stroke. 2000;31:2734.)
© 2000 American Heart Association, Inc.


Abstracts of Literature

Abstracts of Literature

Askiel Bruno Engin Y. Yilmaz

*    Cerebral Aneurysms
 
AB-14809-00
Prevalence of Symptomatic Intracranial Aneurysm and Ischemic Stroke in Pseudoxanthoma Elasticum— van den Berg JSP (Dept of Neurology, Univ Hospital Nijmegen, PO Box 9101 NL-6500 HB Nijmegen, Netherlands), Hennekam RCM, Cruysberg JRM, Steijlen PM, Swart J, Tijmes N, Limburg M—Cerebrovasc Dis. 2000;10:315–319. Copyright © 2000 S. Karger AG, Basel.

Background: Pseudoxanthoma elasticum (PXE) is an heritable connective tissue disorder with clinical manifestations of the ocular, dermal, and cardiovascular system. The purpose of this study was to investigate the prevalence of symptomatic intracranial aneurysms (IAs) and ischaemic stroke (IS) in PXE. Methods: The records of 100 patients with PXE were retrieved. All patients were contacted and data on complications were collected. The literature was reviewed regarding PXE, ISs, and IAs. Results: No patient with PXE had a symptomatic IA as presenting symptom. One patient presented with an IS. During follow-up of 94 of the 100 patients (mean follow-up 17.1 years, range 1–49 years), none presented a symptomatic IA (3,168 retrospective patient observation years and 1,602 prospective patient observation years). Upper gastrointestinal haemorrhage during follow-up occurred in 17 patients, in 1 patient during aspirin use. One patient has IS as presenting symptom and a recurrence during follow-up, and 7 patients had IS during follow-up. All were caused by small-vessel disease. The relative risk of IS in PXE under 65 years compared with the general population was 3.6 (95% confidence interval 3.3–4.0). Conclusions: On the basis of the currently available data, an association between symptomatic IAs and PXE is unlikely. However, the . . . [Full Text of this Article]