Intracranial aneurysms and dolichoectasia in autosomal dominant polycystic kidney disease

Abstract

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.