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Polycystic kidney disease among 4,436 intracranial aneurysm patients from a defined population

Heidi J Nurmonen1, Terhi Huttunen1, Jukka Huttunen1

  • 1From Neurosurgery (H.J.N.), School of Medicine, Institute of Clinical Medicine, Faculty of Health Sciences, University of Eastern Finland, Kuopio; Neurosurgery of NeuroCenter (T.H., J.H., K.H., T.K., M.v.u.z.F., J.E.J. A.E.L.), Kuopio University Hospital, Finland; and Broad Institute (M.I.K.), Boston, MA.

Neurology
|October 6, 2017
PubMed

Insights

Autosomal dominant polycystic kidney disease (ADPKD) is linked to younger-onset subarachnoid hemorrhage from smaller aneurysms. Patients with ADPKD have a higher risk of developing new intracranial aneurysms, necessitating long-term monitoring.

Area of Science:

  • Neurology
  • Genetics
  • Nephrology

Background:

  • Autosomal dominant polycystic kidney disease (ADPKD) is a genetic disorder.
  • Intracranial aneurysms (IAs) and aneurysmal subarachnoid hemorrhage (aSAH) are serious cerebrovascular conditions.
  • The association between ADPKD and IA characteristics requires further definition.

Purpose of the Study:

  • To define the association of ADPKD with the characteristics of aSAH.
  • To compare IA disease in ADPKD patients versus the general IA population.
  • To identify risks for de novo IA formation in ADPKD patients.

Main Methods:

  • Data from the Kuopio Intracranial Aneurysm database and Finnish nationwide registries were fused.
  • A population-based series of 53 IA patients with ADPKD was analyzed.
  • Patient- and aneurysm-specific characteristics were compared between ADPKD and general IA populations.

Main Results:

  • Subarachnoid hemorrhage in ADPKD patients occurred at a younger median age (42.8 years) and from smaller ruptured IAs (median 6.00 mm) compared to the general population.
  • Multiple IAs were more frequent in ADPKD patients (45%) than in the general IA population (28%).
  • The cumulative risk of de novo IA formation was significantly elevated in ADPKD patients (1.3% per patient-year) with a hazard ratio of 7.7.

Conclusions:

  • Subarachnoid hemorrhage in ADPKD patients occurs at a younger age and from smaller intracranial aneurysms.
  • The risk for de novo intracranial aneurysm formation is significantly higher in patients with ADPKD.
  • ADPKD should be considered an indicator for long-term angiographic follow-up in patients with diagnosed intracranial aneurysms.
Abstract

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