Intracranial aneurysms and autosomal dominant polycystic kidney disease

The implementation of a screening program for first degree relatives of patients with sporadic subarachnoid hemorrhage by means of magnetic resonance angiography does not seem to be warranted, since the resulting increase in life expectancy does not offset the risk of postoperative sequelae. Some exceptions, however, should be considered. We present a family suffering from autosomal dominant polycystic kidney disease. Two of the four siblings had a subarachnoid hemorrhage from an aneurysm of a cerebral artery. Although intracranial aneurysms are accepted as extrarenal manifestations of autosomal dominant polycystic kidney disease, the exact frequency of the association is unknown. Nevertheless, the maximum rate of coincidence dealt with in the literature is 41 percent. Because of this high association every patient suffering from a subarachnoid hemorrhage due to an intracranial aneurysm should be screened by an abdominal ultrasound. In case of an association first degree relatives should be examined for a polycystic kidney disease and in the case of a positive result a magnetic resonance angiography of the intracranial vessels should be performed.     

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.     

Early operation of ruptured basilar artery aneurysm associated with bilateral carotid occlusion (moyamoya disease)

A 34-year-old Caucasian man presented with subarachnoid hemorrhage. Angiography revealed bilateral carotid occlusion at the cavernous sinus and an aneurysm at the basilar artery bifurcation. The whole brain was supplied with blood from the basilar artery and posterior cerebral arteries through a large number of collateral vessels to the internal carotid artery bifurcation, middle cerebral and anterior cerebral arteries: the moyamoya phenomenon. The aneurysm was clipped within hours of the subarachnoid hemorrhage. The relation between moyamoya disease and basilar artery aneurysms is discussed and some surgical and management considerations are given.     

Catastrophic Secondary Infarctions with Onset Seizure Following Tissue Plasminogen Activator Therapy

Fatalities following intravenous recombinant tissue-type plasminogen activator therapy have been reported. Major fatal complications following intravenous recombinant tissue-type plasminogen activator therapy include intracranial hemorrhage, aortic dissection, and extracranial bleeding. However, the possibility that intravenous recombinant tissue-type plasminogen activator therapy itself paradoxically induces synchronized multiple cerebral novel infarctions has never been considered. We herein report the first case of bilateral internal carotid artery infarction with onset seizure following intravenous recombinant tissue-type plasminogen activator therapy for a vertebral-basilar artery infarction. A 75-year-old man was transferred to our hospital and diagnosed with acute ischemic stroke in the basilar artery. His National Institute of Health Stroke Scale score was 4. The intravenous recombinant tissue-type plasminogen activator therapy was initiated 234 minutes after stroke onset because no contraindications were present. Almost 2 hours after the intravenous recombinant tissue-type plasminogen activator therapy, the patient suddenly fell into a deep coma with generalized convulsions. A huge secondary infarction was found in the bilateral anterior circulation territories, and he died 7 days after stroke onset. This case alerts clinicians to the possibility of synchronized multiple cerebral infarctions following intravenous recombinant tissue-type plasminogen activator therapy as a dangerous complication in patients with multiple severe stenoses in the cerebral arteries.     

Vertebral artery dissection: warning symptoms, clinical features and prognosis in 26 patients

BACKGROUND AND OBJECTIVES: Internal carotid artery dissection has been extensively studied and well-described. Although there has been a recent increase in the number of reported cases of vertebral artery (VA) dissection, the clinical variety of presentation and the early warning symptoms have not been well-described before. Our objectives in this study include: (1) To determine the early symptoms and warning signs which may help the clinician in the early identification and treatment of patients with VA dissection. (2) To explore the variety of clinical presentation of VA dissection and its relation to prognosis. DESIGN AND SETTING: Retrospective analysis of hospital records in a tertiary academic centre for the period 1989-1999. RESULTS: Twenty-six patients were identified (13 men and 13 women). The mean age was 48. Possible precipitating factors were identified in 14 patients (53%). Sporting activity and chiropractic manipulations were the most common (15% and 11% respectively). Headache and/or neck pain was the prominent feature in 88% of patients and was a warning sign in 53%, preceding onset of stroke by up to 14 days. The most common clinical features included vertigo (57%), unilateral facial paresthesia (46%), cerebellar signs (33%), lateral medullary signs (26%) and visual field defects (15%). Bilateral VA dissection presented in six patients (24%). The most common region of dissection was the C1-C2 level (16 arteries, 51%). Intracranial VA dissection was found in eight arteries (25%). The majority of patients (83%) had favorable outcome. Poor prognosis was associated with (1) bilateral dissection; (2) intracranial VA dissection accompanied by subarachnoid hemorrhage. Only two patients reported stroke recurrence. CONCLUSIONS: Our findings show that VA dissection affects mainly middle age persons and involves both sexes equally. Headache and/or neck pain followed by vertigo or unilateral facial paresthesia is an important warning sign that may precede onset of stroke by several days. Although the majority of patients will have excellent prognosis, this was less likely in patients presenting with subarachnoid hemorrhage or bilateral VA dissection. Recurrence rate was low.     

Uncommon course for a vertebral artery dissection: rupture, occlusion and recanalization.

Intracranial arterial dissections of the vertebrobasilar system are recognized as a cause of stroke. Although the pathogenic mechanism underlying this phenomenon is unknown, in some cases the stroke originates from subarachnoid hemorrhage, while in others ischemia is the cause. In cases where hemorrhage occurs, occlusion of the lesion is effective in reducing the risk of re-bleeding. However, deciding on treatment is difficult in uncommon cases in which occlusion occurs immediately after hemorrhage. Intracranial arterial dissections of the vertebrobasilar system often present as subarachnoid hemorrhage, and the most appropriate surgical treatment remains controversial. In this report, we describe a rare case of vertebral artery dissection in which occlusion occurred immediately after subarachnoid hemorrhage. Serial angiography revealed spontaneous recanalization of the right vertebral artery dissection, as well as enlargement. The progressive angiographic changes of the ruptured vertebral artery dissection and the endovascular treatment of such arterial dissections are discussed.     

Moyamoya disease associated with polycystic kidney disease and eosinophilic granuloma

Moyamoya disease has been associated with renal artery stenosis, cerebral hemorrhage, and multiple cranial traumas. We report a unique case of moyamoya disease associated with polycystic kidney disease and eosinophilic granuloma. Although the etiology of moyamoya disease is unknown, a familial pattern of occurrence has been documented. Of particular importance is its presentation with polycystic kidney disease, an autosomal dominant disease, suggesting a hereditary component to the etiology of this unusual vasculitic disease.     

Pathology of spontaneous dissection of intracranial arteries

Spontaneous dissection of a major subarachnoid artery is an uncommon cause of stroke in young and apparently healthy individuals. Such dissection does not correlate well with systemic conditions such as emboli, thrombotic or hemorrhagic tendencies or hypertension, nor with disease of blood vessels such as atherosclerosis, fibromuscular dysplasia, cystic medial necrosis or Moya-Moya disease. Unusual exertion has been implicated as a factor in some cases. Gap defects were found in the internal elastic lamina near the site of dissection in three of four cases of spontaneous dissection reported here. It is suggested that such defects, because of their unusual size or number, may be responsible for initiating dissection in susceptible individuals. A detailed study of the major cerebral arteries in cases of dissection and control cases to document the size and frequency of such defects could shed light on the pathogenesis of spontaneous dissection. The subsequent course of events tends to differ in the internal carotid and vertebro-basilar systems. In most cases, the dissection in the internal carotid system is sub-intimal leading to thrombosis and cerebral infarction, while in the vertebrobasilar system dissection lies between the media and adventitia leading to subarachnoid hemorrhage. It is not known what structural differences of the two arterial systems may underlie this difference in the pattern of arterial dissection.     

Familial subarachnoid hemorrhage: Distinctive features and patterns of inheritance

To delineate the distinctive features of familial subarachnoid hemorrhage, we compared gender and age at the time of subarachnoid hemorrhage, as well as site and number of aneurysms, in patients with familial subarachnoid hemorrhage (at least 1 first-degree relative with subarachnoid hemorrhage) and patients with sporadic subarachnoid hemorrhage (no subarachnoid hemorrhage in first- or second-degree relatives), in a prospective, hospital-based series of patients. In addition we studied the pattern of inheritance in 17 families with familial subarachnoid hemorrhage. Mean age at the time of hemorrhage in patients with the familial form was 6.8 years lower than that in those with the sporadic form, and middle cerebral artery aneurysms occurred more often in patients with familial disease. Sex distribution and number of aneurysms were similar in the two groups. Inheritance was compatible with autosomal dominant transmission in some families, and with autosomal recessive or multifactorial transmission in others. In our 5 families as well as in all 18 previously reported families with two affected generations, the age at the time of subarachnoid hemorrhage was invariably lower in later generations, which is suggestive of anticipation. We conclude that familial subarachnoid hemorrhage is a separate entity with occurrence at a young age, predilection for aneurysms of the middle cerebral artery, and variable modes of inheritance, including autosomal dominant inheritance with possible anticipation.     

Ruptured dissecting aneurysms of both anterior cerebral arteries

A case of bilateral dissecting aneurysms of the anterior cerebral arteries presenting with subarachnoid haemorrhage is reported. Dissection involved the proximal A2 portions of both anterior cerebral arteries. Dissection of the left anterior cerebral artery was responsible for the subarachnoid haemorrhage. Both aneurysms were treated by coating with cotton and fibrin glue.     

脑动脉瘤手术中穿通支的损伤及其并发症

目的研究脑动脉瘤夹闭术中载瘤动脉及穿通支损伤对脑动脉瘤预后的影响,探讨减少并发症的方法。方法以施行开颅脑动脉瘤夹闭术治疗的52例病人为研究对象,分析术中载瘤动脉及其穿通支损伤与术后并发症的关系。结果发现脑动脉瘤57个。有载瘤动脉及穿通支损伤9例,其中术中破裂4例。大脑前动脉穿通支损伤4例,引起失语、偏瘫,出现严重的脑水肿、脑疝;大脑中动脉穿通支损伤3例,引起肢体活动不灵;前脉络膜动脉损伤2例,引起下肢为重的偏瘫、失语和意识障碍。结论脑动脉瘤开颅夹闭术中易引起载瘤动脉及其穿通支的损伤,应在显微镜下充分解剖蛛网膜下腔,避免在解剖关系不清的状态下盲目操作。     

A young adult case of spontaneous basilar artery dissection]

We reported a case of basilar artery dissection in a 20-year-old man suffering sudden onset of consciousness disturbance. Brain CT revealed a cerebral infarction of the whole territories of vertebro-basilar artery and his 3DCT showed the occlusion at the base of basilar artery. Autopsy revealed that the subintimal dissection was found at the base of basilar artery and the dissection was spreaded to the distal of bilateral posterior cerebral arteries. The characteristics of his vertebro-basilar artery were small in diameter, thin media and thickened intima. According to these findings, we supposed this rare case of basilar dissection occurred all at once based on a functional abnormality in his small vertebro-basilar arterial wall.     

Spontaneous intracranial internal carotid artery dissection: report of 10 patients

BACKGROUND: Spontaneous intracranial internal carotid artery (ICA) dissection is an uncommon cause of cerebral infarction, particularly when compared with the dissection of the ICA's cervical portion. Most reports describe extensive strokes with very high mortality rates. OBJECTIVE: To report the clinical and radiological findings of 10 patients with spontaneous intracranial ICA dissection. METHODS: Ten patients (5 women) were included with ages ranging from 15 to 59 years (mean age, 28 years). RESULTS: Nine patients had a stroke (1 had an associated subarachnoid hemorrhage), whereas 1 patient had only transient ischemic attacks. Severe retro-orbital or temporal headache followed by contralateral hemiparesis was the most common initial clinical symptom. No patient had vascular risk factors or a history of neck or head trauma. Stenosis of the supraclinoid portion of the ICA occurred in 8 patients, with extension to the middle cerebral artery or anterior cerebral artery in 2 patients each. Aneurysm formation in the ipsilateral anterior cerebral artery was seen in 1 patient. Two patients had a total occlusion of the supraclinoid portion of the ICA. All patients did well, with no (n = 3), mild (n = 4), or moderate (n = 3) disability on the Modified Rankin Scale during a 3-month follow-up period. CONCLUSIONS: Spontaneous intracranial ICA dissection can cause ischemic stroke with or without subarachnoid hemorrhage and should be considered in the differential diagnosis of intracranial ICA stenosis or occlusion, especially in young patients. Some patients survive with few or moderate deficits.     

Pathological study of Willis's circle in autopsy cases of polycystic kidney disease

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.     

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.     

A case of agenesis of the corpus callosum accompanied by a ruptured azygos anterior cerebral artery aneurysm and lipoma--a case report

A 53-year-old woman suffered from sudden onset of severe headache on February 28 in 1982. She was admitted to our hospital soon after onset. On admission, she had a severe headache and nausea, and her consciousness was drowsy. CT scan showed a marked subarachnoid hemorrhage with intracerebral and intraventricular hematoma, a separation of the lateral ventricles with enlargement of posterior horns, and deformity of anterior horn. By these findings, the patient was diagnosed as an agenesis of the corpus callosum with subarachnoid hemorrhage. Left carotid arteriogram revealed an azygos anterior cerebral artery and an aneurysm at the terminal portion of this artery. Surgery was performed on the 24 th day after subarachnoid hemorrhage. Operative finding revealed a little finger's head-sized tumor was situated over the right frontobasal artery. An azygos anterior cerebral artery aneurysm was clipped and tumor was removed. Pathological diagnosis of the tumor was a lipoma. Namely, she had an agenesis of the corpus callosum, an azygos anterior cerebral artery, an aneurysm and a lipoma. There are some reports in which an agenesis of the corpus callosum is accompanied with a lipoma and an azygos anterior cerebral artery is accompanied with an aneurysm. But a case of agenesis of the corpus callosum with an azygos anterior cerebral artery was rarely reported. So this rare case with these anomalies was reported and pathogenesis about the development of 4 anomalies was also discussed.     

Intracranial dissecting aneurysms of the posterior circulation: report of 3 cases and review of the literature]

Dissecting aneurysms of cerebral arteries are unusual causes of stroke. The carotid system is the commonest site of this pathology, the vertebral arteries are less involved and dissection of the basilar artery is rare. The authors report three cases of arterial dissection of the vertebrobasilar system, two of the vertebral arteries and one of the basilar artery. An extensive review of the literature is presented. The clinical picture of dissection of vertebrobasilar system was inespecific but pain was a prominent symptom, though had not occurred in the site of the arteries involved. The pain was suggestive of subarachnoid hemorrhage. Associated or risk factors were mild trauma, migraine and high blood pressure. The angiographic findings were suggestive, however just the "double lumen" has been considered pathognomonic. The prognosis is variable. It was benign in case 3, left sequela in case 2, and case 1 rebleed fatally.     

Endovascular treatment of a ruptured accessory middle cerebral artery aneurysm

A 51-year-old female presented with bilateral accessory middle cerebral arteries (MCA) with associated ruptured aneurysm manifesting as a subarachnoid hemorrhage. Angiography demonstrated the bilateral accessory MCA and associated ruptured aneurysm at the junction of the left anterior cerebral artery and left accessory MCA. This was successfully treated by coil embolization. Magnetic resonance angiography obtained 6 months later revealed no evidence of residual or recurring aneurysm.     

Spontaneous bilateral carotid and vertebral artery dissections associated with multiple disparate intracranial aneurysms, subarachnoid hemorrhage and spontaneous resolution. Case report and literature review

Spontaneous bilateral carotid and vertebral artery dissections (CADs and VADs) are rare. A 29-year-old female presented with a collapse, 4 weeks after a sudden onset of severe neck and shoulder pain. CT scan revealed diffuse subarachnoid hemorrhage (SAH) and early hydrocephalus. Angiography revealed bilateral CADs and VADs, along with multiple fusiform and saccular aneurysms. Systemic vessels - including the renal arteries - were normal, and no risk factors or underlying vasculopathy were apparent. The presumed source of SAH (a posterior cerebral artery aneurysm) was successfully clipped. Each dissection, by contrast, was managed conservatively with heparin prophylaxis; and spontaneous CAD and VAD resolution occurred within 6 months. We present a unique case of four-vessel dissection associated with multiple disparate saccular and fusiform aneurysms. We suspect that underlying vasculopathy - perhaps novel - may become apparent with time.     

Spontaneous intracranial internal carotid artery dissection: 6 case reports and a review of 39 cases in the literature]

Angiographic, CT and MR findings, precipitating factors, and clinical features of spontaneous intracranial internal carotid artery dissection, in particular the terminal segment of the ICA, are described. Patients who had a possible posttraumatic dissection were excluded from the study. Six patients, 3 men and 3 women, mean age 25.8 years, were studied. Spontaneous ICA dissection accounted for 0.63 percent of 797 patients with ischemic stroke and 14 percent of 36 patients with ischemic stroke who were 45 years of age or younger. Headache was absent in two but clearly preceded the onset of cerebral stroke in four patients, the interval ranging from less than a few minutes to 6 hours. Focal cerebral ischemic symptoms were present in all six patients. Four presented with loss of consciousness. Only one patient had a subarachnoid hemorrhage (SAH). Dissection occurred at the level of the carotid knee portion just beyond the origin of the ophthalmic artery in all the patients. Moreover, there was additional involvement of the ipsilateral MCA in four and ipsilateral ACA in three. Angiographic features of the six ICAs that involved dissection were a smooth tapered luminal narrowing or occlusion in two patients, irregular narrowing in three, a pearl and string sign in one, and retention of the contrast media in three. Follow up angiography or magnetic resonance (MR) angiography was performed on all the patients. The interval from the initial to follow-up angiography was one to six months. Stenosis persisted in one artery but was resolved in the other five arteries. MR angiography showed segmental narrowing of the right supraclinoid internal carotid artery in one patient. MR angiographic source images showed a narrow lumen in the right intracavernous portion of the ICA and a crescent-shaped filling defect. Follow-up MR imaging and MR angiography, 6 months after the initial study, showed amelioration of the luminal narrowing and no filling defect. The filling defect is considered to be due to a thrombosed pseudolumen. Treatment consisted of anticoagulation therapy for two patients, hyperbaric therapy for two, and supportive measures only for two. Follow-up ranged from ten months to three years. Of these six patients with completed stroke, four had full recovery, and two some residual neurological deficits. Trivial precipitating events were reported by five of the patients (playing tennis, running, bicycling, and drinking alcohol). Migraine headaches were cited by one patient, use of oral contraceptives by another, and use of tobacco by a third. All the patients were normotensive. In none of them was family history contributory, nor was any underlying arteriopathy identified. Of the six personal cases and the reviewed 25 cases during the last 15 years, represented on the Glasgow Outcome Scale, good recovery was achieved in 61%. The outcomes for these patients was better than previously reported. Spontaneous intracranial ICA dissection may cause ischemic stroke, with or without subarachnoid hemorrhage, especially in young patients.