Journal Article

Intracranial aneurysms and dolichoectasia in autosomal dominant polycystic kidney disease

Stefan Graf, Alexander Schischma, Knut E. Eberhardt, Roland Istel, Birgit Stiasny and Bernd D. Schulze

in Nephrology Dialysis Transplantation

Published on behalf of European Renal Association - European Dialysis and Transplant Assoc

Volume 17, issue 5, pages 819-823
Published in print May 2002 | ISSN: 0931-0509
Published online May 2002 | e-ISSN: 1460-2385 | DOI: http://dx.doi.org/10.1093/ndt/17.5.819
Intracranial aneurysms and dolichoectasia in autosomal dominant polycystic kidney disease

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Background. Intracranial saccular aneurysms (ICA) are a known extrarenal manifestation of autosomal dominant polycystic kidney disease (ADPKD). In order to facilitate the definition of subgroups who are at risk for ICA and to determine the prevalence of ICA in these subgroups we studied ADPKD patients with a positive family history for a cerebral event, including cerebral stroke (group I) and intracranial bleeding or known ICA (group II).

Methods. Within an enrolment period of 21 months, 43 ADPKD patients from our outpatient clinic and hospital were examined with cerebral magnetic resonance angiography (MRA).

Results. ICA were detected in six patients (14%). Three out of 32 patients (9.4%) in group I and three out of 11 patients (27.3%) in group II had an ICA. A dolichoectasia of intracerebral vessels was found in two out of 43 patients (4.7%).

Conclusions. Using MRA a high prevalence of ICA was shown only in patients with a family history of cerebral bleeding or ICA. A family history for cerebral stroke does not imply an elevated risk for ICA. However, dolichoectasia, rare in the normal population, was detected in two patients. We recommend screening for ICA in patients with a positive family history for cerebral bleeding or ICA. Because of potential complications, examiners should direct their attention to dolichoectasia in ADPKD patients.

Keywords: ADPKD; aortic aneurysm; dolichoectasia; intracranial aneurysm; polycystic kidney disease

Journal Article.  3011 words. 

Subjects: Nephrology

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