Ruptured Cerebral Aneurysm and Acute Bilateral Carotid Artery Dissection in a Patient with Polycystic Kidney Disease and Polycystic Liver Disease

Abstract

weight was normal but that the size of her abdomen was massively enlarged. A CT scan showed a small hyperintensity in the area of the right Sylvian fissure. Subarachnoidal hemorrhage was suggested and digital subtraction angiography showed three aneurysms of the middle cerebral artery on the right side. Moreover, a dissection of the right internal carotid artery was suspected due to a wedgeshaped high-grade stenosis at the base of the skull. After interdisciplinary discussion, a decision was made to clip the aneurysm. Intraoperative blood clots were found perianeurysmal. Postoperatively, the patient again showed a mild hemiparesis on the left side. Over the following days she developed an organic psychosis. Five days after admission, MRI presented a middle cerebral artery infarction on the right side. Furthermore, fat-suppressed axial T1-weighted images showed a semilunar-shaped hematoma in the arterial wall of both internal carotid arteries with a high-grade stenosis on the right side and only a low-grade stenosis on the left side ( fig. A CT scan of the abdomen revealed a massively enlarged polycystic liver reaching down to the pelvis minor, but with only a few cysts of the kidneys ( fig. Family history yielded several relatives with polycystic kidney and/or liver disease on both the paternal and maternal sides. Our patient was the only one to have suffered from cerebral vascular disease. Five weeks after admission, the patient could be transferred to a rehabilitation clinic with only a mild cognitive impairment. Liver transplant was performed 20 months after the initial event. Molecular analysis of the patient revealed both a homozygote mutation in the PKD1 gene (ADPKD) as well as in the ADPLD gene (PCLD); we have described this previously