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IMAGES IN MEDICINE
Year : 2019  |  Volume : 32  |  Issue : 3  |  Page : 189

Brain vessel anomalies in autosomal dominant polycystic kidney disease


1 Department of Neurology, Wroclaw Medical University, Borowska 213, 50-556 Wroclaw, Poland
2 Department of Radiology, Wroclaw Medical University, Borowska 213, 50-556 Wroclaw, Poland
3 Department and Clinic of Nephrology and Transplantation Medicine, Wroclaw Medical University, Borowska 213, 50-556 Wroclaw, Poland

Date of Web Publication18-Feb-2020

Correspondence Address:
Magdalena Krajewska
Department and Clinic of Nephrology and Transplantation Medicine, Wroclaw Medical University, Borowska 213, 50-556 Wroclaw
Poland
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0970-258X.278698


How to cite this article:
Ejma M, Urbanowski P, Brodowski M, Guranski K, Hendrich B, Krajewska M. Brain vessel anomalies in autosomal dominant polycystic kidney disease. Natl Med J India 2019;32:189

How to cite this URL:
Ejma M, Urbanowski P, Brodowski M, Guranski K, Hendrich B, Krajewska M. Brain vessel anomalies in autosomal dominant polycystic kidney disease. Natl Med J India [serial online] 2019 [cited 2020 May 30];32:189. Available from: http://www.nmji.in/text.asp?2019/32/3/189/278698



Autosomal dominant polycystic kidney disease (ADPKD) is frequently associated with intracranial aneurysms. We describe two sisters with ADPKD and unusual cerebral vessel anomalies. Moyamoya syndrome was found in the younger sister, who had an ischaemic stroke at the age of 5 years [Figure 1]. In the older sister, with no neurological deficit, a monstrous venous anomaly was diagnosed [Figure 2]. The patients’ father, also suffering from ADPKD, had a history of cerebellar stroke at the age of 34 years. He had no malformations of the vessels. These cases describe the rare reports of patients with ADPKD who have cerebral vasculopathies other than aneurysms.
Figure 1: Magnetic resonance angiography of the younger sister. Severe stenosis of both internal carotid arteries ICAs (⇒ ) Compensatory collateral circulation by small, abnormally dilated vessels (‘puff of cigarette smoke’) (⇓)

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Figure 2: Computed tomography angiography of older sister. A monstrous venous anomaly draining into a cavernous sinus (←)

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    Figures

  [Figure 1], [Figure 2]



 

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