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Intracranial arterial dolichoectasia in autosomal dominant polycystic kidney disease
W I Schievink1, V E Torres, D O Wiebers
1Department of Neurologic Surgery, Mayo Clinic, Rochester, Minnesota 55905, USA.
Insights
Autosomal dominant polycystic kidney disease (ADPKD) patients have a higher risk of developing intracranial arterial dolichoectasia and dissections. This condition can lead to stroke and indicates a more widespread arteriopathy in ADPKD.
Area of Science:
- Nephrology
- Neurology
- Vascular Medicine
Background:
- Autosomal dominant polycystic kidney disease (ADPKD) is a genetic disorder.
- ADPKD is associated with various cardiovascular complications.
- Intracranial arterial dolichoectasia is a rare condition involving the widening and elongation of cerebral arteries.
Purpose of the Study:
- To investigate the prevalence of intracranial arterial dolichoectasia in patients with ADPKD.
- To determine if ADPKD is associated with an increased risk of intracranial arterial dolichoectasia and dissections.
- To understand the clinical significance and potential complications of this association.
Main Methods:
- Screening of 178 ADPKD patients using magnetic resonance angiography (MRA).
- Conventional angiography in 40 ADPKD patients.
- Brain autopsy in 98 ADPKD patients.
- Comparison with 360 non-ADPKD patients undergoing similar imaging or autopsy.
Main Results:
- Asymptomatic intracranial arterial dolichoectasia was found in 2.0-2.5% of ADPKD patients.
- No intracranial arterial dolichoectasia was observed in the control group.
- Nine ADPKD patients had intracranial arterial dolichoectasia, with two experiencing ischemic stroke symptoms.
- Six of these nine patients had other vascular manifestations of ADPKD.
- Arterial dissection was implicated in some cases.
Conclusions:
- Patients with ADPKD have an increased risk of developing intracranial arterial dolichoectasia and dissections.
- This association may be a cause of stroke in ADPKD patients.
- The findings suggest a more generalized arteriopathy in ADPKD than previously recognized.
Abstract:
Autosomal dominant polycystic kidney disease (ADPKD) is a systemic disorder with a variety of cardiovascular manifestations. This study presents a group of patients with ADPKD who had intracranial arterial dolichoectasia. One hundred seventy-eight ADPKD patients were screened with magnetic resonance angiography, 40 ADPKD patients had conventional angiography, and 98 ADPKD patients underwent a brain autopsy. For comparison, 360 patients without ADPKD who had magnetic resonance angiography and conventional angiography or brain autopsy were also studied. The prevalence of asymptomatic intracranial arterial dolichoectasia was 2.2% (4 of 178), 2.5% (1 of 40), and 2.0% (2 of 98) in the three ADPKD groups, respectively. None of the patients without ADPKD had intracranial arterial dolichoectasia. In addition to the seven patients with asymptomatic disease, two ADPKD patients with vertebrobasilar dolichoectasia had posterior circulation ischemic symptoms. The mean age of the nine patients (five men and four women) was 56.6 yr (range, 41 to 67 yr). The posterior circulation was involved in five patients, the anterior circulation was involved in two patients, and both were involved in two patients. Arterial dissection was believed to have caused middle cerebral artery dolichoectasia in one patient, and intracranial arterial dissections were strongly suspected in two other patients. Six of the nine patients with intracranial arterial dolichoectasia had additional vascular manifestations of ADPKD. Some patients with ADPKD are at an increased risk of developing intracranial arterial dolichoectasia and dissections. Recognizing this association is important because (1) it may be a cause of stroke; (2) it may mimic a saccular aneurysm on radiographic studies; and (3) it suggests that the arteriopathy of ADPKD may be more generalized than previously believed.