(Stroke. 2000;31:2734.)
© 2000 American Heart Association, Inc.
Abstracts of Literature |
| Cerebral Aneurysms |
|---|
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 149 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 3.34.0). Conclusions: On the
basis of the currently available data, an association between
symptomatic IAs and PXE is unlikely. However, the
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