Adult polycystic kidney disease and intracranial aneurysms.

Adult polycystic kidney disease and intracranial aneurysmsWe describe for the first time a family whose three members all had adult polycystic kidney disease and intracranial aneurysms. Case reportsCase I-A 34 year old man presented in 1981 with accelerated hypertension.Renal ultrasonography confirmed that he had polycystic kidneys.In 1985 he was admitted with a subarachnoid haemorrhage.Neurosurgery showed a right cerebral haematoma, probably from an intracranial aneurysm.This was evacuated, but he died soon afterwards.Case 2-A 39 year old woman, the sister of the patient in case 1, presented in 1978 with a subarachnoid haemorrhage.Cerebral arteriography showed bilateral intracranial aneurysms, but she was managed conservatively.In 1983 intravenous pyelography showed that she had polycystic kidneys.In 1986 she had a prolonged grand mal fit, and subsequently her renal function deteriorated.She was referred for continuous ambulatory peritoneal dialysis and remained well.Case 3-A 36 year old man, the brother of the patients in cases 1 and 2, presented in 1978 with haematuria.Intravenous urography showed that he had polycystic kidneys.In 1986 his family history became known and he underwent elective cerebral digital subtraction angiography, which showed an aneurysm 6 mm in diameter of the right middle cerebral artery (figure).This was clipped, and he remained well.Cerebral digital subtraction angiography showing aneurysm at trifurcation ofright middle cerebral artery.