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Images in Medicine
2019:32:3;189-189
doi: 10.4103/0970-258X.278698
PMID: 32129319

Brain vessel anomalies in autosomal dominant polycystic kidney disease

Maria Ejma1 , Pawel Urbanowski1 , Miroslaw Brodowski1 , Konstanty Guranski1 , Barbara Hendrich2 , Magdalena Krajewska3
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

Corresponding Author:
Magdalena Krajewska
Department and Clinic of Nephrology and Transplantation Medicine, Wroclaw Medical University, Borowska 213, 50-556 Wroclaw
Poland
kguransk@interia.pl
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
Copyright: (C)2019 The National Medical Journal of India

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


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