Anterior Cerebral Artery Dissection in a Patient With Autosomal Dominant Polycystic Kidney Disease

Intracranial artery dissection secondary to autosomal dominant polycystic kidney disease is far less common than cerebral aneurysm. A 55-year-old man presented a sudden onset of headache and disturbed consciousness caused by ischemic stroke in the bilateral frontal lobes with minor subarachnoid hemorrhage. The bilateral anterior cerebral arteries were firstly occluded and re-perfused with irregular narrowing and dilation in 3 days after stroke onset, indicating dissection. He was diagnosed with autosomal dominant polycystic kidney disease by abdominal CT findings and by his family history though his renal function was almost normal. Dissection in the anterior cerebral artery has not been reported previously, while some cases with dissection in the vertebral and extracranial arteries were reported in autosomal dominant polycystic kidney disease. His family also had a history of aortic dissection and subarachnoid hemorrhage. Intracranial artery dissection may be a manifestation of systemic arteriopathy with familial clustering in autosomal dominant polycystic kidney disease. Strict antihypertensive treatment is needed in these cases.     

Intracranial hemorrhage in patients with polycystic kidney disease

To emphasize the important association of polycystic kidney disease and hypertensive cerebral hemorrhage, a registry of 900 consecutive cases of hemorrhagic stroke was reviewed. Eleven patients (1.2%) had intracranial hemorrhage (eight had hypertensive cerebral hemorrhage and the other three had aneurysmal subarachnoid hemorrhage) found to be associated with polycystic kidney disease. These 11 patients also accounted for 11% of the 98 cases of polycystic kidney disease during the 28-month study period. As verified by computed tomography, parenchymal hemorrhage occurred mainly in the putamen and the thalamus, the usual sites for hypertensive cerebral hemorrhage. One patient with cerebral hemorrhage was autopsied and one was studied angiographically, but in neither patient was an intracranial aneurysm identified. In the patients with polycystic kidney disease and intracranial hemorrhage, hypertension had been inadequately treated or even undetected; therefore, I emphasize early detection and more effective control of hypertension in patients with polycystic kidney disease for prophylaxis against hemorrhagic cerebrovascular events.     

Chronic subdural haematoma and arachnoid cyst in autosomal dominant polycystic kidney disease (ADPKD).

We present the unusual association between chronic subdural haematoma (CSDH), intracranial arachnoid cyst and autosomal dominant polycystic kidney disease (ADPKD) in a 27-year-old man. CSDH is a documented complication of intracranial arachnoid cyst, the incidence of which is increased in patients with ADPKD. Awareness of this association may lead to earlier diagnosis of ADPKD and treatment of its systemic complications, including renal insufficiency, systemic hypertension and previously unsuspected intracranial saccular aneurysm. Surgery for CSDH associated with intracranial arachnoid cyst may be complicated by over-drainage of cerebrospinal fluid due to communication between the cyst and the cisternal subarachnoid space, as illustrated in the present case, and the development of epidural haemorrhage.     

The association between polycystic kidney disease and cerebral aneurysms.

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.     

Intracranial aneurysm screening: indications and advice for practice

The advent of non-invasive methods of imaging intracranial blood vessels has facilitated screening for intracranial aneurysms in people who are at risk. A strong risk factor for intracranial aneurysm, which causes subarachnoid haemorrhage, is a positive family history, defined as two or more first-degree relatives with subarachnoid haemorrhages (relative risk [RR]=6.6). The greatest familial risk is associated with an affected sibling. Another strong risk factor is polycystic kidney disease (RR=4.4). People who have these risk factors are potential candidates for screening. Even if screening does not find abnormality, there is a high risk of new aneurysms 5 years later. Repeated screening might be done, although the optimum interval between screening assessments and the duration of repeated screening is unclear. Patients who have survived a subarachnoid haemorrhage are at increased risk of another from a newly developed aneurysm, but whether screening is beneficial in such patients is not clear. Most occurrences of subarachnoid haemorrhage in the general population are related to the more common risk factors of hypertension (RR=2.8) and smoking (RR=1.9). Therefore, screening of individuals who have a family history of subarachnoid haemorrhage or polycystic kidney disease will have little effect on the incidence of subarachnoid haemorrhage in the general population. When patients present for screening, the construction of a family tree and discussion of the history of relatives with stroke can be revealing. Before referring a patient for non-invasive imaging of the circle of Willis, the physician should discuss the risks and benefits of screening, the implications for holding a licence to drive or fly and for life insurance, and whether or not the patient wants to be informed about other incidental findings.     

De novo aneurysm after treatment of glioblastoma

A rare case of spontaneous subarachnoid hemorrhage from newly developed cerebral aneurysm in glioblastoma patient is presented. A 57-year-old man was presented with headache and memory impairment. On the magnetic resonance image and the magnetic resonance angiography, a large enhancing mass was found at right frontal subcortex and intracranial aneurysm was not found. The mass was removed subtotally and revealed as glioblastoma. He took concurrent PCV chemotherapy and radiation therapy, but the mass recurred one month later after radiotherapy. He was then treated with temozolomide for 7 cycles. Three months after the completion of temozolomide therapy, he suffered from a subarachnoid hemorrhage due to a rupture of a small de novo aneurysm at distal anterior cerebral artery. He underwent an aneurysm clipping and discharged without neurologic complication.     

Successful serial imaging of the mouse cerebral arteries using conventional 3-T magnetic resonance imaging

Serial imaging studies can be useful in characterizing the pathologic and physiologic remodeling of cerebral arteries in various mouse models. We tested the feasibility of using a readily available, conventional 3-T magnetic resonance imaging (MRI) to serially image cerebrovascular remodeling in mice. We utilized a mouse model of intracranial aneurysm as a mouse model of the dynamic, pathologic remodeling of cerebral arteries. Aneurysms were induced by hypertension and a single elastase injection into the cerebrospinal fluid. For the mouse cerebrovascular imaging, we used a conventional 3-T MRI system and a 40-mm saddle coil. We used non-enhanced magnetic resonance angiography (MRA) to detect intracranial aneurysm formation and T2-weighted imaging to detect aneurysmal subarachnoid hemorrhage. A serial MRI was conducted every 2 to 3 days. MRI detection of aneurysm formation and subarachnoid hemorrhage was compared against the postmortem inspection of the brain that was perfused with dye. The imaging times for the MRA and T2-weighted imaging were 3.7±0.5 minutes and 4.8±0.0 minutes, respectively. All aneurysms and subarachnoid hemorrhages were correctly identified by two masked observers on MRI. This MRI-based serial imaging technique was useful in detecting intracranial aneurysm formation and subarachnoid hemorrhage in mice.     

Diagnosis of cerebral arterial aneurysms in acute and subacute subarachnoid hemorrhage

The paper pools experience in using computed tomographic and magnetic resonance angiographies in patients with intracranial berry aneurysms in different periods following subarachnoid hemorrhage. The new low-invasive techniques for visualization of arterial vessels have been found to detect the aneurysm and to define its topographic and anatomic location in most cases (as high as 90%) by being on a par with direct cerebral angiography and to compare favourably with the latter. Based on the analysis of complex studies (angiography, CT- and MR-angiographies), the authors have developed algorithms for diagnosing intracranial aneurysms by the stage of subarachnoid hemorrhage.     

Delayed rupture of pre-existing cerebral aneurysm in a young patient with minor head trauma.

An intracranial saccular aneurysm is not commonly diagnosed in a patient with head injury. We present a patient with a history of minor head trauma and a CT scan of the brain revealing minimal subarachnoid hemorrhage 17 days prior to admission, complaining of severe headache, dysarthria and focal right limb seizures 3 hours prior to admission. A traumatic aneurysm was suspected based on clinical history and radiological findings including hematoma in the falx region on a CT scan of the brain and an aneurysm of the pericallosal artery on magnetic resonance angiography and four-vessel cerebral angiography. However, at craniotomy, an intracranial non-traumatic saccular aneurysm at the bifurcation of the pericallosal artery was found. The patient recovered fully after successful clipping the aneurysm.     

Aneurysms in relatives of patients with subarachnoid hemorrhage: frequency and risk factors. MARS Study Group. Magnetic Resonance Angiography in Relatives of patients with Subarachnoid hemorrhage.

OBJECTIVE: To evaluate the frequency and identify risk factors of unruptured aneurysms in first-degree relatives of patients with sporadic subarachnoid hemorrhage (SAH). BACKGROUND: First-degree relatives (parents, siblings, and children) of patients with SAH have a three to seven times increased risk of SAH. METHODS: Magnetic resonance angiography (MRA) was performed in 626 first-degree relatives of a consecutive series of 193 index patients with "sporadic" SAH (participation rate, 78% of eligible relatives). The authors recorded demographic and medical data, and performed blood pressure measurements and blood tests (cholesterol, high-density lipid cholesterol, triglycerides, apoprotein A1, lipoprotein a, glucose). RESULTS: Aneurysms were found in 25 of 626 relatives (4.0%; 95% CI, 2.6 to 5.8%), and 6 relatives had multiple aneurysms. Index patients with multiple aneurysms and a younger age had a higher risk of aneurysms in relatives. Siblings of index patients had a four times higher risk than children. In screened relatives female sex, increasing age, polycystic kidney disease, hypertension, and elevated levels of cholesterol and glucose tended to be associated with a higher risk of aneurysms. No increase in risk was conferred by smoking or alcohol use, or by a previous family history of SAH or of atherosclerosis. CONCLUSIONS: First-degree relatives of index patients with sporadic subarachnoid hemorrhage have a 4.0% chance of intracranial aneurysms. Being a sibling of the index patient is the most important risk factor. Risk factors for general atherosclerosis (hypertension, smoking, hypercholesterolemia, high levels of blood triglycerides, lipoprotein a, and apoprotein A1) and use of alcohol do not increase the risk of intracranial aneurysms significantly in these relatives.     

Spontaneous disappearance of an intracranial aneurysm after subarachnoid hemorrhage.

Spontaneous disappearance of an intracranial aneurysm after subarachnoid hemorrhage is an uncommon event and usually associated with severe cerebral vasospasm, giant aneurysms or the use of antifibrinolytics. We present a young woman who suffered a grade 5 subarachnoid hemorrhage with severe vasospasm caused by a small anterior communicating artery aneurysm. The patient underwent a slow recovery and two years later requested surgery. Angiography demonstrated complete disappearance of the aneurysm. The neurosurgeon should be aware that spontaneous thrombosis of cerebral aneurysms can occur and ensure that angiography is repeated when surgery is significantly delayed.     

Transient oculomotor nerve paresis in congenital distal basilar artery aneurysm.

The clinical and pathologic findings of a 10-month-old girl with congenital heart disease who died after rupture of a congenital distal basilar artery aneurysm are reported. The patient developed transient minimal oculomotor nerve paresis 7 days prior to suffering a massive subarachnoid hemorrhage. The finding of transient third nerve dysfunction, particularly in the context of recurrent syncope, should prompt investigation for an intracranial arterial aneurysm.     

Long-term aneurysm recurrence and de novo aneurysm formation after surgical treatment of unruptured intracranial aneurysms: a cohort study and systematic review

ABSTRACT

Objective: There is a relative lack of literature on long-term aneurysm recurrence and de novo aneurysm formation following surgical treatment of unruptured intracranial aneurysms. This retrospective single-center cohort study, therefore, analyzes the incidence of aneurysm recurrence, and the incidence of de novo aneurysms formation in patients with at least 10yrs of radiological follow-up. The data are put into the context of a systematic review of the literature.

Methods: Patients that underwent surgical treatment of an unruptured intracranial aneurysm at the Basel University Hospital were retrospectively identified. The rate of recurrent or de novo aneurysm formation was assessed for all patients with imaging follow-up ≥10yrs. A systematic review including studies with a mean follow-up period of ≥10yrs was then performed.

Results: A total of 95 patients had undergone surgical treatment of an unruptured intracranial aneurysm between 1994 and 2008. Twenty-one patients (22.1%) had available imaging follow-up ≥10yrs (mean: 13.1yrs). In these patients, aneurysm recurrence and de novo aneurysm formation were equally found in 23.8% (n = 5; 1.8%/yr). There was no case of aneurysm rupture from a recurrent or a de novo aneurysm. The systematic literature review covered a combined cohort of 1778 patients over a mean follow-up period of 14.0yrs. In this cohort, the aneurysm recurrence rate was 16.4% (0.7%/yr), and the rate of de novo aneurysm formation was 6.2% (0.4%/yr).

Discussion: Despite some discrepancy regarding the incidence, both cohorts show a non-negligible long-term risk of aneurysm recurrence and de novo aneurysm formation, which warrants life-long imaging follow-up.

Abbreviations: SD: standard deviation; DSA: digital subtraction angiography; CTA: computed tomography angiography; MRA: magnetic resonance angiography; MCA: middle cerebral artery; ACA: anterior cerebral artery; ACommA: anterior communicating artery; ICA: internal carotid artery; ADPKD: autosomal dominant polycystic kidney disease; MeSH: Medical Subject Headings     

Multiple intracranial aneurysms associated with primary hyperaldosteronism

Multiple intracranial aneurysms have been reported in association with polycystic disease of the kidney, brain tumor, pituitary adenoma and coarctation of the aorta. We report the association of multiple aneurysms with primary hyperaldosteronism due to bilateral adrenal hyperplasia in an 18 year old left-handed man who presented with subarachnoid hemorrhage and arterial hypertension. We report the excellent outcome of this patient in spite of a difficult and surgical management. Ligation of all three intracranial aneurysms was performed after an extra-intracranial arterial bypass was done as a protective measure.     

Spontaneous Intracranial Arterial Dissection and Subarachnoid Hemorrhage

Spontaneous intracranial arterial dissection may cause subarachnoid or rarely intraventricular hemorrhage. The clinical presentation may resemble aneurysmal rupture. Experience using magnetic resonance imaging (MRI) and magnetic resonance angiography (MRA) in establishing the diagnosis of intracranial arterial dissection has been limited. We report three patients with intracranial arterial dissection and describe the correlation between findings on conventional angiography and MRI or MRA.     

CT在诊断动脉瘤性蛛网膜下腔出血中的临床应用研究(附140例报道)

目的 探讨CT在诊断动脉瘤性蛛网膜下腔出血(ASAH)中的应用价值。方法 回顾性分析了140例CT。表现为SAH并拟诊为颅内动脉瘤(AN)的临床病例资料，从中找出AN破裂在CT。上的出血规律。结果 经CT诊断为ASAH140例，其中133例经脑血管造影(DSA)、核磁共振血管造影(MRA)和直接手术证实为AN，7例没有发现AN，CT诊断AN的阳性率为95％。发现不同部位的AN破裂所引起的SAH在CT上的表现不同，其出血有一定规律。结 论通过CT检查，可以初步对AN进行定位和定性诊断，对AN的进一步检查和治疗有重要的临床意义。     

Actively bleeding intracranial aneurysm demonstrated by CT angiography

CT angiography (CTA) is increasingly being utilized in the initial diagnosis of subarachnoid hemorrhage. While active bleeding from an intracranial aneurysm has been demonstrated on conventional angiography, CT angiogram findings of active aneurysmal hemorrhage are not well described. We present a case of an actively bleeding anterior communicating artery aneurysm demonstrated by CT angiography. The initial CTA demonstrated the extravasation of contrast from the anterior communicating artery. A second CT scan less than 1h later confirmed the ongoing hemorrhage, with extension of hemorrhage into the subarachnoid cisterns and the ventricular system. Recognition of active aneurismal hemorrhage by CTA may modify plan of treatment and follow-up imaging.     

Moyamoya病患者颅内出血原因探讨——75例Moyamoya病分析

目的 探讨Moyamoya病(MMD)发生颅内出血的原因.方法 收集作者近期收治的21例有颅内出血的MMD患者的临床资料,并结合文献中报道的54例MMD患者进行回顾性分析.结果 75例患者中,CT显示MMD患者脑内出血位于脑实质内47例,脑室内出血22例,蛛网膜下腔出血6例,表现为蛛网膜下腔出血的MMD患者均经DSA证实为伴发颅内动脉瘤.结论 MMD出血主要位于脑实质内或脑室内,有蛛网膜下腔出血时,应考虑并存颅内Willis环附近主要动脉动脉瘤可能.     

颅内动脉瘤破裂合并颅脑损伤5例分析

目的探讨颅内动脉瘤破裂合并颅脑损伤的临床特征,以此鉴别创伤性蛛网膜下腔出血。方法对我院近三年来收治的5例颅内动脉瘤破裂合并颅脑损伤患者的临床资料进行回顾性研究,总结其临床特征。结果 4例患者入院后急诊经CTA检查证实为颅内动脉瘤破裂出血,其中前交通动脉瘤2例,大脑中动脉瘤1例,颈内动脉-后交通动脉瘤l例;1例患者为动脉瘤再次破裂后行CTA检查示颈内动脉-后交通动脉瘤。急诊开颅血肿清除及动脉瘤夹闭术2例,动脉瘤夹闭术1例,血管内介入栓塞治疗术1例,药物保守治疗1例。恢复良好3例,重残1例,死亡l例。结论对伴有颅脑外伤史的蛛网膜下腔出血应注意考虑颅内动脉瘤破裂的可能,以便采取积极合理的治疗方案。     

Neuroimaging of hemorrhage and vascular malformations

Nontraumatic spontaneous intracranial hemorrhage occurs most commonly into the subarachnoid space and brain parenchyma, in contrast to subdural and epidural hematomas that are usually traumatic. The differential diagnosis of nontraumatic subarachnoid hemorrhage includes intracranial aneurysm rupture and vascular malformations, both of which may be investigated noninvasively with computed tomography and magnetic resonance imaging. An isolated intraparenchymal hematoma may be caused by hypertensive vasculopathy, amyloid angiopathy, vascular malformations, or by primary or secondary neoplasms. Knowledge of the appearance of intracerebral vascular malformations will help clinicians request appropriate further imaging and direct treatment.