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Background: Vascular abnormalities, including dissections and aneurysms, can be found in patients with autosomal dominant kidney disease (ADPKD). While intracranial aneurysms have been reported in 10%-25% of ADPCKD, occurrences at other locations are exceedingly rare., Method: This is a first case report of a patient with ADPCKD who presented with a rupture of the left external carotid artery pseudoaneurysm., Conclusion: Rupture of a carotid artery aneurysm is rare with potentially high morbidity. An endovascular and surgical approach are effective strategies for successful management that depends on etiology, location, and surgeon experience., Competing Interests: Declaration of conflicting interestsThe author(s) declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.

Background: Autosomal dominant polycystic kidney disease (ADPKD) is associated with an increased risk for developing intracranial aneurysms (IAs). We aimed to evaluate the frequency of diagnosis of IAs in the cross-sectional, population-based, Genkyst cohort, to describe ADPKD-associated IAs and to analyse the risk factors associated with the occurrence of IAs in ADPKD patients., Methods: A cross-sectional study was performed in 26 nephrology centres from the western part of France. All patients underwent genetic testing for PKD1/PKD2 and other cystogenes., Results: Among the 2449 Genkyst participants, 114 (4.65%) had a previous diagnosis of ruptured or unruptured IAs at inclusion, and ∼47% of them had a positive familial history for IAs. Most aneurysms were small and saccular and located in the anterior circulation; 26.3% of the patients had multiple IAs. The cumulative probabilities of a previous diagnosis of IAs were 3.9%, 6.2% and 8.1% at 50, 60 and 70 years, respectively. While this risk appeared to be similar in male and female individuals <50 years, after that age, the risk continued to increase more markedly in female patients, reaching 10.8% versus 5.4% at 70 years. The diagnosis rate of IAs was >2-fold higher in PKD1 compared with PKD2, with no influence of PKD1 mutation type or location. In multivariate analysis, female sex, hypertension <35 years, smoking and PKD1 genotype were associated with an increased risk for diagnosis of IAs., Conclusions: This study presents epidemiological data reflecting real-life clinical practice. The increased risk for IAs in postmenopausal women suggests a possible protective role of oestrogen., (© The Author(s) 2022. Published by Oxford University Press on behalf of the ERA.)

Background: Professional societies provide conflicting guidelines on aneurysm screening in patients with polycystic kidney disease (PKD), and the rate of subarachnoid hemorrhage (SAH) is poorly understood., Objective: To evaluate screening, elective treatment, and the rate of SAH in patients with known PKD., Methods: We examined longitudinally linked claims data from a large private insurer, identifying screening, elective treatment, aneurysmal subarachnoid hemorrhage (aSAH) and secured aneurysmal SAH (saSAH) in 2004 to 2014 amongst patients with known PKD., Results: We identified 20 704 patients diagnosed with PKD. Among patients with an initial PKD diagnosis, 51/446 (15.9%) underwent angiographic screening within 2 yr. Forty aneurysms were treated electively in 48 868 yr at risk in PKD patients (82/100K patient yr, 95% confidence interval [CI] 60-112) vs 24 elective treatments in 349 861 yr at risk in age- and sex-matched controls (7/100K patient yr, 95% CI 5-10, P < .0001). Eleven admissions for aSAH were identified in PKD patients (23/100K patient yr, 95% CI 13-41) and 22 admissions for aSAH in controls (6/100K patient yr, 95% CI 4-10), giving an incidence rate ratio (IRR) of 3.6 (95% CI 1.7-7.4, P < .0001) and a comorbidity-adjusted IRR of 3.1 (95% CI 1.4-6.9). The incidence of saSAH was proportionally even higher in PKD patients than controls, 16 vs 2/100K patient years, IRR 9.5 (95% CI 3.3-27.5, P < .0001)., Conclusion: Screening in PKD is performed only selectively, though resulting rates of elective treatment were over 10× those of controls. Despite screening and treatment, the rate of SAH remains significantly elevated over that of controls., (Copyright © 2018 by the Congress of Neurological Surgeons.)

Autosomal-dominant polycystic kidney disease (ADPKD) is a relatively frequent genetic disorder that is associated with increased prevalence of intracranial aneurysms (IAs). However, evidence on the natural history of IAs in ADPKD is suboptimal. That leads to difficulties in development of recommendations on surveillance on patients with IAs in their medical history, or the need for repeat imaging for IAs in those with a negative result of the initial screening. The aim of the article is to present our experience on the natural history of IAs in ADPKD patients., Material and Methods: Thirty-four ADPKD patients, managed at our outpatient department, with imaging for intracranial aneurysms performed at least twice, were included into present retrospective analysis., Results: Among 8 patients with an IA in their medical history, no new IA was observed during 93 patient-years of follow-up. In 6 patients with untreated, unruptured IAs, IA growth was observed in 2 cases during 32 patient-years of follow-up. Finally, among 20 patients with a negative result of initial screening, 2 new IAs were noticed during 115 patient-years of follow-up, including 1 patient with a positive family history for an IA, and 1 patient without a family history., Conclusions: Our observations support repeat imaging for IAs in patients with ADPKD, positive family history of IA, and negative result of initial screening. Additionally, efforts should be made to develop clinical and/or laboratory risk factors for IAs development in ADPKD patients without family history of IA, which enable to identify patients who should undergo repeat imaging for IAs., (Copyright © 2017. Published by Elsevier Urban & Partner Sp. z o.o.)

Objective: To define the association of autosomal dominant polycystic kidney disease (ADPKD) with the characteristics of aneurysmal subarachnoid hemorrhage (aSAH) and unruptured intracranial aneurysm (IA) disease., Methods: We fused data from the Kuopio Intracranial Aneurysm database (n = 4,436 IA patients) and Finnish nationwide registries into a population-based series of 53 IA patients with ADPKD to compare the aneurysm- and patient-specific characteristics of IA disease in ADPKD and in the general IA population, and to identify risks for de novo IA formation., Results: In total, there were 33 patients with ADPKD with aSAH and 20 patients with ADPKD with unruptured IAs. The median size of ruptured IAs in ADPKD was significantly smaller than in the general population (6.00 vs 8.00 mm) and the proportion of small ruptured IAs was significantly higher (31% vs 18%). Median age at aSAH was 42.8 years, 10 years younger than in the general IA population. Multiple IAs were present in 45% of patients with ADPKD compared to 28% in the general IA population. Cumulative risk of de novo IA formation was 1.3% per patient-year (vs 0.2% in the general IA population). Hazard for de novo aneurysm formation was significantly elevated in patients with ADPKD (Cox regression hazard ratio 7.7, 95% confidence interval 2.8-20; p < 0.0005)., Conclusions: Subarachnoid hemorrhage occurs at younger age and from smaller IAs in patients with ADPKD and risk for de novo IAs is higher than in the general Eastern Finnish population. ADPKD should be considered as an indicator for long-term angiographic follow-up in patients with diagnosed IAs., (© 2017 American Academy of Neurology.)

Background: Iodine-based contrast medium used in diagnostic and therapeutic cerebrovascular imaging may cause renal toxicity, especially in patients with underlying renal impairment. Contrast dilution may impede efforts of the neurointerventionalist to treat intracranial vascular pathology., Methods: A 36-year-old man with renal impairment presented with an unruptured A1 segment anterior cerebral artery aneurysm. Previously obtained magnetic resonance angiography was fused with intraoperative noncontrast computed tomography and live 2-dimensional fluoroscopic images. The aneurysm was successfully treated with stent-assisted coil embolization without the use of contrast., Results: Neurointervention without contrast was feasible, and although the presented case is one example, the imaging fusion techniques used in this case can substantially decrease the exposure to contrast and subsequent risk of renal injury during intracranial procedures., Conclusions: Further development of and experience with this technique is needed to improve its safety and efficacy., (Copyright © 2014 National Stroke Association. Published by Elsevier Inc. All rights reserved.)

The authors report on 3 rare cases of ruptured lenticulostriate artery (LSA) aneurysms that were heralded by deep cerebral hematomas. The hematomas were unilateral in 2 cases and bilateral in 1; in the bilateral case, only a single LSA aneurysm could be identified on the right side of the brain. Because of their small size (≤ 2 mm), fusiform aspect, and deep location within the brain, all of the aneurysms were treated conservatively. There was no hemorrhage recurrence, and follow-up angiography demonstrated spontaneous thrombosis in 2 of the 3 cases. The clinical course was favorable in 2 of the 3 patients. The course in the patient with the bilateral hematoma was marked by an ischemic event after the initial episode, resulting in an aggravation of deficits. The cause of this second event was uncertain. Because our knowledge about the natural history of LSA aneurysms is incomplete, there is no consensus concerning a therapeutic strategy. The authors' experience in 3 reported cases leads them to think that a conservative approach involving close angiographic monitoring may be proposed as first-line treatment. If the monitored aneurysm then persists or grows in size, its occlusion should be considered. Nonetheless, other studies are needed to further strengthen the legitimacy of this strategy.

We report an unusual case of simultaneous hemorrhage in intracranial aneurysms and in renal cyst in a case of polycystic kidney disease. A 27-year-old gentleman presented with progressive headache and intermittent vomiting of one month duration. Initial computerized tomography (CT) scan and magnetic resonance imaging/angiography revealed a large mass lesion in the right temporal fossa. Over the previous 15 days, he developed progressive weakness in his left upper and lower limbs, and the headache worsened in severity. A repeat of CT scan showed an evidence of aneurysmal bleed and a large temporal lobe hematoma. The patient underwent urgent evacuation of the intracerebral of hematoma and excision of the redundant aneurysmal sac. The patient made excellent recovery in the post-operative period; however, for him, the pain abdomen was persisting. Detailed work-up with contrast-enhanced abdominal CT scan revealed bilateral multiple cysts in the kidneys with evidence of intracystic hemorrhage on the left side. An extensive search of the literature revealed that this kind of presentation has not been reported previously.

We report a rare case with polycystic kidney disease (PKD) having an intracranial internal carotid artery aneurysm associated with extracranial occlusion of the ipsilateral internal carotid artery. A 55-year-old man with chronic renal failure due to PKD presented with headache. CT scan and MRI showed no abnormal findings. MRA showed cervical occlusion of the right internal carotid artery and an ipsilateral intracranial carotid aneurysm. At surgery, the saccular aneurysm protruded anterolaterally at the C2 portion of the right internal carotid and was clipped. Hemodynamic stress of the blood flow through the posterior communicating artery and the fragility of arteries because of PKD were considered to be two main causes of aneurysmal formation in this case.

Background and Purpose: Subarachnoid haemorrhage is a common cause of death in patients with autosomal dominant polycystic kidney disease (ADPKD), but little is known about specific characteristics of subarachnoid haemorrhage and intracranial aneurysms in this group of patients. We performed a systematic review on site, size and number of aneurysms, age at time of rupture, gender, and family history in patients with ADPKD and intracranial aneurysms. We also studied the frequency of ADPKD in patients with subarachnoid haemorrhage treated in our hospital., Methods: We performed a MEDLINE search and checked the reference lists of all relevant publications to identify all articles published from 1980 to 2000 on intracranial aneurysms or subarachnoid haemorrhage in ADPKD. We studied our database of patients with subarachnoid haemorrhage treated between 1978 and 1999 for the presence of ADPKD., Results: We included 53 articles on 369 ADPKD patients (139 [54 %] women) with 462 intracranial aneurysms. Of the 273 aneurysms with specified locations 105 (38 %) were located on the middle cerebral artery in and on the anterior communicating artery in 83 patients (30 %). In 253 patients with data about relatives, the family history was positive for intracranial aneurysms or subarachnoid haemorrhage in 102 (40 %). The average age at which subarachnoid haemorrhage had occurred in 258 was 41 years; of 158 in whom the gender was given; 96 (52 %) were women. Of the 160 patients with data on outcome, 69 (43 %) had died as the result of the subarachnoid haemorrhage. Of the 1147 patients treated for aneurysmal subarachnoid haemorrhage in our institution (mean age 53 years; 65.5 % women), 5 (0.44 %) had ADPKD., Conclusions: Compared with data on patients without ADPKD, subarachnoid haemorrhage in patients with ADPKD occurs not only often in a familial setting of subarachnoid haemorrhage, but also at an earlier age and more often in men. In patients with ADPKD, the most frequent site of aneurysms is the middle cerebral artery. The proportion of patients with ADPKD among all patients with subarachnoid haemorrhage is very small.

Background: We set out to determine the prevalence of incidental intracranial aneurysms in first-degree relatives aged 30 years or more of people with intracranial aneurysms, and to see if polycystic kidney disease contributes to the aggregation of familial intracranial aneurysms., Methods: 91 families with two or more affected members had previously been identified from a 14 year series of 1150 intracranial aneurysm patients treated at the University Hospital of Kuopio, Finland. Magnetic resonance angiography was used as a preliminary screening method, followed by conventional four-vessel angiography to verify suspected aneurysms. Participants were also screened for polycystic kidneys by ultrasonography., Findings: Incidental aneurysms were detected in 40 individuals: 38 of 438 individuals from 85 families without polycystic kidney disease or other diagnosed heritable disorders, and two of 22 individuals from six families known to have polycystic kidney disease. The crude and age-adjusted prevalence of incidental intracranial aneurysms among screened first-degree relatives was 8.7 (SE 1.3)% (95% CI 6.2-11.7) and 9.1 (1.4)% (6.2-11.7), respectively, for the familial group and the crude prevalence for the polycystic kidney group was 9.1 (6.1)% (1.1-29.2)., Interpretation: Our results demonstrate a high prevalence of incidental intracranial aneurysms among first-degree relatives aged 30 years or older of patients with the condition and indicate that the risk of having an aneurysm is about four times higher for a close relative than for someone from the general population. Also, polycystic kidney disease families are a small fraction of the familial intracranial aneurysm families.

We have encountered a 66-year-old woman with a polycystic kidney (PCK). This was combined with the fact that the new growth of an aneurysm at the left anterior cerebral artery (A2-A3 junction) had ruptured 3 years after an operation for a right IC-PC aneurysm in 1985. The cause of the neogenesis and enlargement of an intracranial aneurysm is important because of the changes in the arterial wall due to congenital factors, hypertension, arteriosclerosis, etc, or hemodynamic stress caused by hypertension. We suspected that intracranial aneurysm formation was caused by hypertension due to PCK and the intracranial aneurysm is explained as being due to maldevelopment. In patients with PCK, it should be kept in mind that intracranial aneurysms may be present, and it is advisable to use cerebral angiography and MRA to see whether this is so or not. It should be also stressed that the control of hypertension is very important for the prognosis of patients with PCK. Not only new growth of cerebral aneurysms but also intracerebral hemorrhage is to be prevented.

Purpose: To estimate the prevalence, location, and type of cerebral aneurysms in patients with adult polycystic kidney disease (PCKD) and identify associated risk factors., Materials and Methods: Ninety-three patients with PCKD were screened for aneurysms with spin-echo parenchymal magnetic resonance (MR) imaging and three-dimensional time-of-flight MR angiography. Prevalence was estimated with the calculated proportions of patients who had aneurysms, adjusted for reported sensitivity and specificity of MR angiography., Results: Thirteen aneurysms, found in 10 patients, were 7 mm or smaller in largest dimension; 11 of these aneurysms were saccular. Intra- and interobserver variabilities were 96%-98%. Conventional arteriograms, obtained in six patients, helped confirm the MR angiographic findings. The best estimates of prevalence of aneurysms were 11.7% in the study group (n = 93) and 25.8% in patients with a family history of aneurysms (n = 6)., Conclusion: The prevalence of aneurysms in the study group was greater than in the general population. MR angiography is useful in screening for cerebral aneurysms.

Background and Methods: Intracranial aneurysms are a feature of autosomal dominant polycystic kidney disease, but their prevalence is uncertain. We studied 92 subjects with autosomal dominant polycystic kidney disease who had no symptoms or signs of any neurologic disorder. To determine the prevalence of intracranial aneurysms, we performed high-resolution computed tomography (CT) in 60 subjects, four-vessel cerebral angiography in 21, and both procedures in 11., Results: Four of the 88 subjects in whom the radiologic studies were successfully completed had intracranial aneurysms (4 percent; 95 percent confidence interval, 0.1 to 9 percent), as compared with the prevalence of 1 percent reported for an angiographic study of the general population. Three of the four subjects had multiple aneurysms. Seven subjects for whom the results of CT studies were suspicious underwent cerebral angiography: two had aneurysms, and five had normal vascular structures that accounted for the suspicious results of tomography. Four subjects who had normal CT imaging studies also had normal angiographic examinations. Eight of the 32 subjects who underwent angiography (25 percent) had transient complications, as compared with 22 of 220 control subjects (10 percent) who did not have polycystic kidney disease (P less than 0.05). We could not identify any risk factor in these subjects that was related to the occurrence of aneurysm., Conclusions: Asymptomatic intracranial aneurysms appear to be more frequent in people with polycystic kidney disease than in the general population, although our 95 percent confidence interval includes the possibility of no difference. Because cerebral angiography is associated with increased morbidity in people with polycystic kidney disease, we recommend high-resolution CT as a screening test.

A patient who had bilateral distal anterior cerebral artery aneurysms and a right middle cerebral artery aneurysm in association with polycystic kidney and liver disease is reported. A 57-year-old woman was referred to our center with headache and disturbance of consciousness. On admission, her level of consciousness as evaluated by the Japan Coma Scale was 10. CT revealed subarachnoid hemorrhage, especially in the interhemispheric fissures. Right carotid angiography demonstrated bilateral distal anterior cerebral artery aneurysms and a right middle cerebral artery aneurysm. All three aneurysms were clipped in a one-stage procedure. The patient was discharged without any neurological deficits two weeks after the operation. Bilateral distal anterior cerebral artery aneurysms are extremely rare. This is the first report of such aneurysms and a right middle cerebral artery aneurysm in association with polycystic kidney and liver disease. The etiology of these aneurysms is discussed.

The literature on the association of intracranial aneurysms in autosomal dominant polycystic kidney disease (ADPKD) consists mainly of case reports and small series of patients. To provide a more-detailed description of this association and its frequency, the records of all ADPKD patients with saccular intracranial aneurysms, all ADPKD autopsy cases including brain examination, and sex- and age-matched autopsy cases without ADPKD seen at the Mayo Clinic between 1950 and 1989 and of all Rochester residents with a diagnosis of subarachnoid hemorrhage or ADPKD between 1945 and 1984 were reviewed. The presentation of the 41 patients (22 men and 19 women; mean age, 46.4 yr) with this association was subarachnoid hemorrhage in 33, transient ischemic attacks in 2, incidental angiographic or autopsy finding in 5, and discovery during angiographic screening in 1. Thirty-one, seven, and three patients harbored one, two, and three aneurysms, respectively, arising from the middle cerebral artery (N = 23), anterior communicating artery (N = 16), internal carotid artery (N = 11), and vertebral or basilar artery (N = 4). A family history of intracranial aneurysm, subarachnoid hemorrhage, or intracranial hemorrhage at an early age was present in 22% of the patients. Small aneurysms (less than 5 mm) were less likely to have ruptured or caused symptoms (P less than 0.04). There was a trend for hypertension to be associated with the severity of the subarachnoid hemorrhage. Aneurysmal rupture occurred before age 50 in 64% of patients. Of the 89 ADPKD autopsy cases with brain examination, 22.5% had intracranial aneurysms.(ABSTRACT TRUNCATED AT 250 WORDS)

The pathogenic basis of the association between adult polycystic kidney disease (APKD) and cerebral aneurysms is unknown. We have compared cerebral aneurysms in 79 patients with APKD gleaned from the literature to the sporadic aneurysm cases reported by the Cooperative Study to determine if there are significant biological differences between these two groups. Sixty-eight patients had a single aneurysm and 11 (14%) had multiple aneurysms. In APKD patients with subarachnoid hemorrhage from a single aneurysm there was a significant over-representation of males (72%, p less than 0.01); and the APKD group had more aneurysms of the middle cerebral artery (37%, p less than 0.05). The peak decennial incidence and mean age of rupture of APKD-associated aneurysms was younger (mean age 39.7 years, p less than 0.01) and over 77% of APKD-associated aneurysms had ruptured by age 50 versus 42% for sporadic aneurysms (p less than 0.001). Cerebral aneurysms co-existed with APKD in the absence of hypertension in 25% of 45 cases where the presence or absence of hypertension was recorded. These biological differences and the occurrence of aneurysms in normotensive APKD patients suggests an etiology which may be independent of hypertension and that APKD-associated aneurysms may be genetically determined. It is hypothesized that cases of inherited, familial cerebral aneurysms could be linked to a genetic defect resembling that which occurs on chromosome 16 in APKD.

Rupture of cerebral aneurysms is an important cause of morbidity and mortality in patients with adult polycystic kidney disease (PKD). In the present paper, we review the literature regarding the association of cerebral aneurysms and PKD and emphasize, by means of a case report, the importance of familial clustering of aneurysms in patients with PKD. We conclude that the prevalence of intracranial aneurysms in patients with PKD approaches 40% and is likely significantly higher in patients with PKD and a positive family history for cerebral aneurysm. We recommend that screening cerebral angiography be strongly considered in all patients with PKD and a family history of cerebral aneurysm or subarachnoid hemorrhage.

High resolution computed tomography or magnetic resonance imaging of the head with 3-mm sections through the circle of Willis was obtained in 96 patients with autosomal dominant polycystic kidney disease. These scans are well suited for the noninvasive, presymptomatic diagnosis of intracranial aneurysms at high risk of rupture, since the risk of rupture of unruptured aneurysms is heavily dependent on their size, and aneurysms equal to or larger than 6 mm in diameter are not likely to escape detection by these techniques. No definite aneurysm was observed in any of the 96 patients. Small areas (2-4 mm) of contrast enhancement or signal void were detected in 11 patients. These areas are possibly due to vascular tortuosity, but the studies were considered to be indeterminate because a small aneurysm could not be ruled out. Cerebral angiography in two of these 11 patients was normal. Arachnoid cysts were observed more frequently in the patients with polycystic kidney disease than in a control group of patients matched by gender, age, and type and date of examination.

From August, 1981, to August, 1982, the authors performed four-vessel angiography in 17 patients with polycystic kidney disease (PKD) who had no neurological deficit and no history of subarachnoid hemorrhage. Seven cases of unruptured aneurysms were found among these 17 patients (an incidence of 41.2%). Five of the unruptured aneurysms were operated on prophylactically, with no mortality or morbidity. Nine of the 17 patients had hypertension and, of these, two (22.2%) had aneurysms. Of the eight patients without hypertension, five (62.5%) had aneurysms. This study suggests that the coexistence of PKD and intracranial aneurysms might not be due to the hypertension that occurs concomitant with PKD, but instead may be attributable to congenital factors. The authors stress the necessity of early diagnosis and early operation for unruptured aneurysms in patients with PKD.

A 48 year-old man is presented who died of rupture of intracranial aneurysm. Autopsy findings revealed a ruptured anterior communicating artery aneurysm associated with polycystic kidneys and polycystic liver. Epidermiological review is performed using "Annual of the Pathological Autopsy Case in Japan" (Vol. 17-20). In 92854 autopsy cases, there are 243 cases of polycystic kidney, 1542 cases of intracranial aneurysm and 10 cases of association with both of them. Several points come clear from comparing our data with foreign ones, as follows; 1) The frequency of polycystic kidney (0.25%) is almost the same between Japan and foreign country. 2) The frequency of intracranial aneurysm (1.6%) is higher in Japan. 3) The frequency of association with both (0.01%) is lower in Japan. 4) The frequency of intracranial aneurysm appears to be higher in cases suffered from polycystic kidney than in others. We suspected that cerebral aneurysm formation is caused by hypertension due to polycystic kidney, although in many reports, the association of polycystic kidney and cerebral aneurysm is explained to be maldevelopment. The operation of cerebral aneurysm with polycystic kidney is the same as that without polycystic kidney. But the control of hypertension and renal function is necessary during and after operation.

Five cases of intracranial aneurysms with polycystic kidneys are reported: --15% of polycystic kidneys have an intracranial aneurysm, and about 4% - 5% of intracranial aneurysms are joined with polycystic kidneys; --the surgical treatment of intracranial aneurysm with this association doesn't present more risk than an intracranial aneurysm, and the surgical prognosis isn't bad; --the pathogeny isn't clear. This association is perhaps a congenital elastic system disease.

A young man with polycystic kidney disease was seen initially with an brain-stem infarction. Postmortem examination disclosed a dissecting aneurysm of the basilar artery and a saccular aneurysm of the right vertebral artery. Dissecting intracranial aneurysms rarely are associated with saccular aneurysms and, to our knowledge, have not been reported in association with polycystic kidney disease.

The clinical course of a case of polycystic disease of the liver and kidney associated with the rupture of a cerebral aneurysm and fatal fulminant hepatitis is outlined, and the clinical significance is discussed.

To detect unruptured intracranial aneurysms, we performed cerebral angiography in five patients with polycystic kidney disease (PKD) who had no neurological deficits and no history of subarachnoid haemorrhage. Three of the five patients had unruptured intracranial aneurysms and two underwent surgery with no mortality or morbidity. Our review of the literature revealed that the surgical risk of unruptured intracranial aneurysms is smaller than the risk of bleeding in conservatively treated patients. We discuss the importance of an early diagnosis, and early operation for unruptured aneurysms in patients with polycystic kidney disease and stress the need for intensive care for their renal dysfunction and hypertension during and after the operation.

Two cases of cerebral aneurysm combined with polycystic kidneys (PCKs) were presented. Case 1, a 24-year-old hypertensive male, was referred to our clinic owing to sudden onset of severe headache at August 20, 1982. Neurological findings on admission were stuporous, right vitreous hemorrhage (so-called Terson's syndrome), and hypertension. CT scans showed subarachnoid hemorrhage, and right MCA bifurcation aneurysm with marked vasospasms by cerebral angiography was revealed. Intentional delayed operation with V-P shunt was performed. He discharged with mild left upper limb paresis, and visual impairment on the right. Bilateral PCKs were confirmed by postoperative DIP and CT scan. Case 2, a 51-year-old female, who suddenly complained of severe headache, was referred to our department 3 days after subarachnoid hemorrhage. One year previously, she had been pointed out PCKs. Neurological findings on admission at February 29, 1980, were drowsy, left third cranial nerve palsy, and hypertension. Cerebral angiography showed multiple aneurysms (bilateral IC-PC & A-com). Neck clipping (1-IC-PC & A-com) and coating (r-IC-PC) were performed at the next day of admission, and V-P shunt operation was followed about 8 weeks after first operation. About 2 weeks after discharge, she suddenly became loss of consciousness and expired. Autopsy revealed intracerebral hemorrhage in left basal ganglia and thalamus. Both kidneys were PCKs of Potter type 3 and cysts of the liver were also noted. In young hypertensive patients with cerebral aneurysms, it should be in mind whether PCKs may be combined or not, and cerebral angiography in PCKs were reasonable to find out harbored cerebral aneurysm.(ABSTRACT TRUNCATED AT 250 WORDS)

Polycystic kidney is a familial disorder known to be complicated often by liver cyst, pancreas cyst and cerebral aneurysm. While numerous reports have been made on the concurrence polycystic kidney and cerebral aneurysm in foreign countries, there are only several autopsy cases documented so far in Japan. This paper describes a surgically treated case of proven non-ruptured aneurysms of bilateral middle cerebral arteries associated with polycystic kidney, and discusses the epidermology of cerebral aneurysm accompanying polycystic kidney in Japan. According to the Annual Report of Pathological Autopsy Cases in Japan, 1981, 8.4% of the cases of polycystic kidney were associated with cerebral aneurysms. The patients with polycystic kidney should be investigated for cerebral aneurysms.

Two autopsy cases of polycystic kidney disease with intracranial aneurysms were reported. We made a pathological study of their vascular system with special reference to Willis's circle in order to prove the inherent abnormality of vascular system in patients with polycystic kidney disease. A 57-year-old man was revealed to have polycystic kidney disease with left middle cerebral artery aneurysm and aortic stenosis. The other 58-year-old man was revealed to have polycystic kidney disease with cerebral aneurysms both in anterior communicating artery and basilar artery, aortic dissecting aneurysm, cerebral infarction and myocardial infarction. Histologically, both abrupt interruption and partial disappearance of internal elastic lamina are found frequently in the wall of Willis's circle. This abnormality of internal elastic lamina may be attributable to congenital factors and rise the frequency of occurrence of intracranial aneurysms in polycystic kidney disease.