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The authors report a case of a 45-year-old woman with pulsatile tinnitus who was found to have an unusual spontaneous fistula between the petrous internal carotid artery and internal jugular vein. The fistula resolved spontaneously, possibly related to daily manual compression of the ipsilateral common carotid artery. The patient also had a contralateral carotid artery dissection, multiple intracranial arachnoid cysts, and hemifacial atrophy. Her medical history was significant for easy bruisibility, abnormal scarring, and mitral valve prolapse. This association appears unique and may represent a previously underscribed generalized connective tissue disorder.

The familial occurrence of intracranial aneurysms has been well described. However, intracranial aneurysms are not rare and the great majority of reported families consist of only two affected members. Therefore, the familial aggregation of intracranial aneurysms could be fortuitous. The authors investigated the familial occurrence of aneurysmal subarachnoid hemorrhage (SAH) in their community to determine whether family members of patients with a ruptured aneurysm are at an increased risk of developing an SAH. All 81 patients from Rochester, Minnesota, who suffered an SAH between 1970 and 1989 from a proven aneurysmal rupture were identified, and they or their families were contacted and a family history was obtained. The number of expected SAHs among first-degree relatives was calculated using previously established age- and sex-specific incidence rates in the community of Rochester. Of the 81 index patients, 76 had complete follow up for family history. Fifteen (20%) of these 76 patients had a first- or second-degree relative with aneurysmal SAH. The number of observed first-degree relatives with aneurysmal SAH was 11, compared to an expected number of 2.66, giving a relative risk of 4.14 (95% confidence interval 2.06-7.40; p < 0.001). In the authors' community, aneurysmal SAH was familial in one of five patients, and this familial aggregation was not fortuitous. The increase in familial risk of aneurysmal SAH is approximately fourfold among first-degree relatives.

This investigation summarizes and evaluates the results of a clinical protocol that we designed to care for patients with acute liver failure (ALF). Adult patients with ALF were enrolled in the protocol. Grade II portal-systemic encephalopathy prompted admission to the intensive care unit (ICU). Patients who met the clinical criterion were activated for liver transplantation. Intracranial pressure (ICP) was monitored in patients with grade III encephalopathy. An increase in ICP was treated with hyperventilation, diuretics, barbiturates, or a combination thereof. Survival was considered to have occurred if the patient left the hospital alive. Our series included 25 patients. Orthotopic liver transplantation (OLT) was performed on 19 patients, 12 of whom survived. Only 2 of 6 patients who did not undergo transplantation survived. Ten of 11 patients who underwent transplantation before reaching grade IV encephalopathy survived. Only 2 of 8 patients who underwent transplantation after reaching grade IV survived (P = .006). The causes of death included cerebral edema (3 patients), disseminated aspergillosis (3 patients), and other (5 patients). ICP was monitored in 11 patients. Increased pressure was documented by seven of the monitors placed. There was one focal hemorrhage secondary to a subdural monitor. Outcome is improved if transplantation occurs before grade IV encephalopathy. ICP monitoring can be accomplished without significant risk of hemorrhage. In our series, infection with aspergillus occurred frequently and with fatal outcome.

The first 48 hours after aneurysmal subarachnoid hemorrhage are critical in determining final outcome. However, most patients who die during this initial period are not included in hospital-based studies. We investigated the occurrence of subarachnoid hemorrhage in a population-based study to evaluate possible predictors of poor outcome. All patients diagnosed with aneurysmal subarachnoid hemorrhage between 1955 and 1984 were selected for analysis of mortality in the first 30 days using the medical record-linkage system employed for epidemiological studies in Rochester, Minnesota. One hundred and thirty-six patients were identified. The mean age of these 99 women and 37 men was 55 years. Rates for survival to 48 hours were 32% for the 19 patients with posterior circulation aneurysms, 77% for the 87 patients with anterior circulation aneurysms, and 70% for the 30 patients with a presumed aneurysm (p < 0.0001). Rates for survival to 30 days were 11%, 57%, and 53%, respectively, in these three patient groups (p < 0.0001). Clinical grade on admission to the hospital, the main variable predictive of death within 48 hours, was significantly worse in patients with posterior circulation aneurysms than in others (p < 0.0001). The prognosis of ruptured posterior circulation aneurysms is poor. The high early mortality explains why posterior circulation aneurysms are uncommon in most clinical series of patients with subarachnoid hemorrhage. The management of incidentally discovered intact posterior circulation aneurysms may be influenced by these findings.

Some patients with aneurysmal subarachnoid hemorrhage (SAH) never reach the hospital alive ("sudden death") and, although their numbers are significant, they are not included in most studies of SAH. To clarify the clinical profile of sudden death from aneurysmal SAH, we reviewed the epidemiology and clinicopathologic features of patients with aneurysmal SAH who never reached medical attention. Using the medical record linkage system employed for epidemiologic studies for the population of Rochester, Minnesota, we identified all patients who were diagnosed with aneurysmal SAH between 1960 and 1989. There were 80 women and 33 men with a mean age of 55 years. Of these 113 patients, 13 (12%) died without reaching medical attention. The proportion of those with sudden death remained stable during the study period. In comparing patients with sudden death with those who reached medical attention, the only significant variable was the frequency of posterior circulation aneurysms that was found in 38%, compared with 14% in those who received medical attention (p = 0.042). At autopsy, intraventricular hemorrhage was present in 12 patients (92%) with sudden death, and intracerebral hemorrhage was present in two (15%). Twelve patients (92%) had acute pulmonary edema. In our community, the frequency of sudden death from aneurysmal SAH has not changed during the last three decades in spite of the advances in medical care. The typical clinical profile of sudden death in SAH includes intraventricular hemorrhage, pulmonary edema, and a ruptured posterior circulation aneurysm. Intracerebral hemorrhage is uncommonly associated with sudden death from aneurysmal SAH.

An elevated serum carcinoembryonic antigen (CEA) without evidence of neoplasia was noted in an 84-year-old woman. She subsequently developed a lytic skull lesion, which at surgery proved to be an atypical meningioma. Immunohistochemical analysis demonstrated that this tumor was producing CEA. This aggressive meningioma required two further resections and adjuvant radiotherapy in the following 18 months. Serum elevation of CEA has been reported only twice in association with meningiomas; both of those tumors were of the secretory subtype.

Background: A primary arteriopathy is suspected in most patients with spontaneous dissections of the carotid artery, although the nature of this arteriopathy usually remains elusive. Angiographic changes of fibromuscular dysplasia (FMD), however, are found in 10% to 20% of patients with carotid dissections., Case Description: A 26-year-old woman with Marfan's syndrome presented with bilateral amaurosis fugax after surgical repair of an aortic dissection. Angiography revealed a dissection extending from the ascending aorta into the right internal carotid artery and, separate from the aortic dissection, a dissection of the left internal carotid artery. After surgery, microscopic examination of the right carotid artery revealed a medial dissection but no evidence of an underlying arteriopathy, while the left internal carotid artery displayed the typical features of FMD. Eighteen months later the patient died after resection of a large thoracoabdominal aortic dissecting aneurysm. Microscopic examination of the aorta revealed moderately extensive cystic medial necrosis., Conclusions: Carotid dissections associated with Marfan's syndrome may be the result of an extension of an aortic dissection or occur isolated from the aorta, even in the same patient. The occurrence of FMD in a patient with Marfan's syndrome in conjunction with previous reports of FMD in a variety of connective tissue disorders suggests that FMD, like cystic medial necrosis, may be a nonspecific disease entity.

Objective: It has been known for some time that paragangliomas of the head and neck may be familial. Recent evidence suggests genomic imprinting is an important factor in the clinical development of these tumors. In genomic imprinting the imprintable gene is transmitted in a mendelian manner, but expression of the gene is determined by the sex of the transmitting parent. In the case of paragangliomas, the gene does not result in the development of tumors when maternally inherited. The purpose of this study was to determine if this hypothesis correctly predicted the inheritance pattern of familial paraganglioma in a large series of patients., Setting: A retrospective review of case records of the Mayo Clinic, Rochester, Minn, a tertiary referral center, was performed for the years 1950 to 1992 to identify patients with familial paragangliomas of the head and neck., Patients: A total of 38 patients with familial paragangliomas of the head and neck were identified in nine kindreds., Results: Carotid body tumors were the most common paragangliomas of the head and neck (34 cases). Glomus jugulare or glomus vagale occurred in eight cases. In 16 (42%) of the patients there were multiple tumors. Surgical complications, primarily cranial nerve palsies, were more frequent for tumors larger than 5 cm in diameter., Conclusions: An autosomal dominant inheritance pattern could be shown for paragangliomas of the head and neck that was consistent with genomic imprinting. It is recommended that all individuals in kindreds with paragangliomas be screened after the age of 16 years to detect early asymptomatic tumors.

Aneurysms of the extracranial internal carotid artery (ICA) are uncommon. A significant proportion of such aneurysms are now recognized to be caused by arterial dissection. In some patients, surgical treatment may become necessary. The surgical treatment of 22 patients with spontaneous or traumatic dissecting aneurysms arising from the extracranial ICAs is reviewed. The mean age of the 7 women and 15 men was 39 years. The aneurysm arose from the proximal third of the extracranial ICA in 1 patient, from the middle third in 1 patient, and from the distal third in 20 patients. Five patients underwent cervical carotid ligation; in 13 patients, the aneurysms were resected, and the ICAs were reconstructed, and 4 patients underwent cervical-to-intracranial ICA bypasses. There were 2 postoperative strokes (9%). Facial and lower cranial nerve palsies were commonly seen after high cervical exposure, but these cranial nerve palsies were transient. There were no long-term neurological sequelae during a mean follow-up of 6.2 years. In our relatively limited experience, extracranial ICA dissecting aneurysms can be treated with acceptable morbidity using a variety of techniques. However, the indications for surgical intervention in these aneurysms remain limited.

Background and Purpose: The familial occurrence of intracranial aneurysms suggests the presence of a genetically determined underlying arteriopathy. The pattern of inheritance in these families usually is not known., Methods: A family with seven members with intracranial aneurysms is described and, from the literature before 1994, a total of 238 families with 560 affected members (56% female and 44% male) with intracranial aneurysms not associated with a known heritable disease are reviewed. A segregation analysis was performed on 73 of these families., Results: Two members were affected in the great majority of families (79%); five or more members were reported in only eight families (3%). The most common affected kinship was among siblings. Angiographic screening in 12 families detected an intracranial aneurysm in 29% of 51 asymptomatic relatives. Segregation analysis revealed several patterns of inheritance that were consistent with the compiled pedigrees, but no single mendelian model was the overall best fitting, suggesting that genetic heterogeneity may be important. Twenty-two percent of siblings of male probands had an intracranial aneurysm compared with 9% of siblings of female probands (P = .003)., Conclusions: Genetic heterogeneity may be important in the genetics of intracranial aneurysms. In families with intracranial aneurysms, siblings of an affected male proband may be at a higher risk of developing an aneurysm than siblings of an affected female proband. Screening for intracranial aneurysms in asymptomatic relatives should be considered in families with two or more affected members. In most families, the nature of the underlying arteriopathy remains obscure.

Among 263 consecutive patients with spontaneous cervicocephalic arterial dissections evaluated at the Mayo Clinic, 18 (6.8%) were 18 years of age or younger (mean age, 12 years). The dissection involved the cervical arteries in 11 patients and the intracranial arteries in seven. Extracranially, the internal carotid artery was involved in eight patients, the vertebral artery in two, and both arteries in one. Intracranially, only the anterior circulation was affected. All the patients had cerebral or retinal ischemic symptoms, usually preceded by headache. Death occurred in a 13-year-old boy with intracranial arterial dissection and coarctation of the aorta. For the 17 remaining patients, the follow-up period ranged from 1 to 21 years. A complete or good clinical recovery occurred in 10 of the 11 patients with cervical arterial dissection but in only four of the seven with dissection of intracranial arteries. Recurrent arterial dissection occurred in two patients with cervical arterial dissections and in one patients with intracranial arterial dissection.

Fifty-four patients with primary neoplasms of the anterior skull base were treated by craniofacial resection with curative intent. The most common tumor was esthesioneuroblastoma (24), followed by squamous cell carcinoma (10). The overall 2-year and 5-year survivals were 75% and 49%, respectively. High-grade tumors, grades 3 and 4, had a poorer prognosis. Tumor size, dural involvement, sphenoid sinus involvement, age, and sex had no significant influence on survival when examined by multivariate survival analysis. Sixteen complications were noted in the postoperative period. Cerebrospinal fluid leakage occurred in 2 patients and loss of frontal bone occurred in 4. There were no operative or perioperative deaths. Craniofacial resection permits surgical resection of the majority of anterior skull base tumors with acceptable morbidity.

Background: Nonaneurysmal perimesencephalic hemorrhage, a distinct form of subarachnoid hemorrhage, is a recently described variant of intracranial hemorrhage. We describe two patients who presented with unusual features of this type of subarachnoid hemorrhage and also two patients who had a perimesencephalic pattern of hemorrhage due to a ruptured posterior circulation aneurysm., Case Descriptions: The first patient, a 41-year-old woman with perimesencephalic hemorrhage, underwent an exploratory craniotomy because angiography had suggested an anomaly of the basilar tip. No source of hemorrhage could be identified at the time of surgery. The second patient was a 3-year-old boy who presented with opisthotonos and who was found to have a perimesencephalic hemorrhage. Angiography revealed no source for the hemorrhage. The third patient, a 54-year-old man, had a perimesencephalic pattern of subarachnoid hemorrhage from a vertebrobasilar junction aneurysm associated with a fenestration that was missed on the initial angiographic study. The fourth patient, a 43-year-old man, suffered a perimesencephalic pattern of subarachnoid hemorrhage from a small posterior cerebral artery aneurysm, which had not been recognized on two angiograms., Conclusions: These patients elaborate on the clinical spectrum of subarachnoid hemorrhage with a perimesencephalic pattern. First, a negative exploratory craniotomy suggests that the source of nonaneurysmal perimesencephalic hemorrhage may not be arterial. Second, nonaneurysmal perimesencephalic hemorrhage may also occur in children. Finally, the index of suspicion for a posterior circulation aneurysm should remain high in patients who present with a perimesencephalic pattern of subarachnoid hemorrhage, and these aneurysms may rise from unusual locations.

An aneurysmal dissection of a right middle cerebral artery (MCA) branch is described in a 56-year-old woman. The abnormality was an incidental finding on computerized tomography and subsequently appeared on magnetic resonance imaging performed to evaluate the patient for subjective pulsatile tinnitus. The intracranial aneurysm was documented to have enlarged on serial angiography over a 6-week interval. Treatment was believed to be necessary because of the unknown etiology of the aneurysm, with the differential diagnosis including mycotic or neoplastic aneurysm with a risk of hemorrhage. The lesion was excised and flow to the distal MCA branch was preserved with an anastomosis of the superficial temporal artery to the MCA. The aneurysm, which developed at the level of the sylvian fissure, proved on pathological study to be related to a focal dissection of the MCA branch. The radiographic appearance and pathological findings are presented. Focal dissection must henceforth be included in the differential diagnosis of peripheral cerebral artery aneurysms.

Ninety-two surgical procedures were performed in 82 patients for recurrent carotid artery stenosis. The etiology was recurrent atherosclerosis in 45 cases, myointimal hyperplasia in 20, organized thrombus without a significant underlying plaque in 20, and scarring along the proximal arteriotomy site in seven. The operations included a repeat endarterectomy in 66 cases and reconstruction with an interposition graft in 22. All five major neurological complications occurred in symptomatic patients, and included three instances of intraoperative embolization during exposure of the carotid artery. The majority of neurological complications occurred in symptomatic patients who had intraluminal thrombus confirmed at surgery. There were four perioperative deaths, due to cerebral hemorrhage in two patients and myocardial infarction in two. In the patients whose original surgery was performed at the Mayo Clinic, the risk of recurrent carotid artery stenosis was 3.1% with a primary closure compared to 1.6% when a patch graft was used. These results indicate that surgery for recurrent carotid artery stenosis is technically more difficult and carries a significantly higher risk than surgery for primary disease. The difficulty is due to the friable recurrent plaque associated with intraluminal thrombus, which increases the risk of embolization during carotid artery exposure. In the majority of patients with recurrent atherosclerosis, a repeat endarterectomy can be achieved. However, in some patients, there is scarring without a definite plane of cleavage between the recurrent disease and the underlying media, making an endarterectomy difficult. In these cases, excision of the diseased segment and reconstruction with an interposition graft is the best treatment. The findings presented here also suggest that closure of the original arteriotomy with a patch graft decreases the risk of recurrent carotid artery stenosis.

Meningeal and cortical biopsies were evaluated in 37 patients (25 men and 12 women; mean age, 54 yr) who had chronic meningitis of an unknown cause between 1985 and 1993 (the era of magnetic resonance imaging). Magnetic resonance imaging with gadolinium contrast was the most useful diagnostic imaging technique, demonstrating meningeal enhancement in 15 of 32 patients (47%). Only 2 of 32 (6%) computed tomographic scans revealed enhancement. A definitive diagnosis was made in 16 of 41 biopsies (39%), but in cases where enhancement was present on either magnetic resonance imaging or computed tomography, a diagnosis was obtained in 80% (12 of 15 cases). Only 2 of 22 biopsies (9%) from nonenhancing regions were diagnostic. Although the locations of enhancement were distributed evenly, biopsies through suboccipital and pterional craniotomies gave the highest diagnostic yields (50%). Furthermore, if the biopsies were obtained from enhancing regions, the yield of these two approaches increased to 84 and 100%, respectively. Of 18 cases in which biopsy samples were taken from both the meninges and cortex, only 1 had cortical involvement alone. The meninges were therefore diagnostic in 15 of the 16 definitive diagnostic cases (94%). Second biopsies were necessary in four cases, of which the three biopsies from enhancing regions were diagnostic. The most frequent causes of chronic meningitis were sarcoid (31%) and metastatic adenocarcinoma (25%).(ABSTRACT TRUNCATED AT 250 WORDS)

Background: Heritable disorders of connective tissue are recognized in a small minority of patients with neurovascular diseases. In this report, we review the neurovascular manifestations of four heritable connective tissue disorders: Ehlers-Danlos syndrome, Marfan's syndrome, osteogenesis imperfecta, and pseudoxanthoma elasticum, as well as two other systemic disorders with potential vascular manifestations: neurofibromatosis and polycystic kidney disease., Summary of Review: Typical neurovascular complications of Ehlers-Danlos syndrome are carotid-cavernous fistulae, intracranial aneurysms, and cervical artery dissections. Arterial dissections and intracranial aneurysms cause the majority of neurovascular symptoms in Marfan's syndrome. Neurovascular disease is uncommon in osteogenesis imperfecta, although carotid-cavernous fistulae and vertebral artery dissections have been reported. Neurovascular disease in pseudoxanthoma elasticum is characterized by intracranial aneurysms and cerebral ischemia caused by premature arterial occlusive disease. Intracranial occlusive arterial disease is the most common neurovascular manifestation of neurofibromatosis, followed by cervical arteriovenous fistulae and aneurysms and intracranial aneurysms. Intracranial aneurysms are the hallmark of polycystic kidney disease., Conclusions: Recognition of an underlying generalized connective tissue disorder may be of considerable importance, although marked phenotypic heterogeneity often complicates the diagnosis of these disorders. Conversely, the association of certain neurovascular anomalies with generalized connective tissue disorders and recognition of their basic molecular defect may offer clues to the etiology and pathogenesis of these neurovascular diseases in general.

Objective: To examine the techniques, reported experiences, and advantages and disadvantages associated with the endovascular treatment of intracranial aneurysms., Design: We review the endovascular techniques used for the treatment of intracranial aneurysms and the sequelae of subarachnoid hemorrhage, which have evolved during the past 10 years., Material and Methods: Two broad categories of endovascular therapy for intracranial aneurysms are described: occlusion of the parent artery and preservation of the parent artery by selective occlusion of the aneurysm with balloons or metallic coils. The Mayo protocol for testing tolerance of patients before permanent balloon occlusion of the parent artery is described, as are the types of aneurysms most amenable to this treatment. In addition, use of balloon angioplasty for cerebral vasospasm after subarachnoid hemorrhage is reviewed., Results: Recent improvements in microcatheter technology have facilitated the safe navigation of percutaneously introduced catheters in the intracranial circulation and selective catheterization of intracranial aneurysms. Surgically difficult aneurysms are now being treated with endovascular techniques more frequently than in the past. Early results from animal experiments and human trials have shown that selective occlusion of aneurysms with metallic coils may have a role in the treatment of intracranial aneurysms. Balloon angioplasty of symptomatic cerebral vasospasm has demonstrated improvement in neurologic function in approximately 70% of patients., Conclusion: As technology continues to improve and as greater experience is obtained, interventional neuroradiologists will continue to have an increasingly important role in the treatment of intracranial aneurysms.

The pathogenesis of ruptured intracranial aneurysms and cervical artery dissections is poorly understood but may be similar in these two disorders. We report four patients with alpha 1-antitrypsin deficiency who developed a ruptured intracranial aneurysm or spontaneous dissection of the cervical internal carotid artery. Three patients were heterozygous for the deficient allele (PiMZ or PiGZ) and one was homozygous (PiZZ). A deficiency of alpha 1-antitrypsin or one of the other protease inhibitors could result in degradation of the arterial wall through an imbalance between proteolytic enzymes and their inhibitors, thereby predisposing the arterial wall to dissection or aneurysm formation.

Twenty-three cases of transitional cavernous aneurysms are presented. Aneurysms of this subgroup (of a total of 118 cases of cavernous sinus aneurysms) arise entirely from within the cavernous sinus but project into the extracavernous intradural subarachnoid space, thus resembling other intracranial aneurysms in their increased risk of rupture. Six aneurysms were small (less than 15 mm), 6 were large (15 to 25 mm), and 11 were giant (more than 25 mm). Thirteen patients (57%) had a subarachnoid hemorrhage, nine patients (39%) had compressive symptoms, and one patient (4%) was asymptomatic. A direct surgical approach was performed successfully in 18 cases (78%), and indirect bypass methods were performed in 5 cases. The overall surgical outcome was excellent in 87% of the patients, with three complications (13%) including one fatality. It is suggested that this subgroup is a different entity from other cavernous or extracavernous aneurysms and should be managed aggressively with direct clipping whenever possible because of the increased risk of subarachnoid hemorrhage. A simplified numerical classification system of clinoidal-region aneurysms of the internal carotid artery (including transitional aneurysms) is also proposed.

Cranial nerve palsies affecting ocular motor function in patients with spontaneous cervical internal carotid artery dissections are rare. Among 155 patients with spontaneous dissections of the cervical internal carotid artery, four (2.6%) had transient third, fourth, or sixth cranial nerve palsy. The third nerve was involved in two patients, the fourth nerve in one, and the sixth nerve in one. Three patients had ipsilateral headache or facial pain, one had bilateral headaches, and three had oculosympathetic palsy. None had any associated cerebral or retinal ischemic symptoms. Cervical internal carotid artery dissection should be included in the differential diagnosis of palsies of the third, fourth, or sixth cranial nerve, especially when associated with ipsilateral headache or facial pain. Interruption of the nutrient arteries supplying these cranial nerves could explain their involvement by internal carotid artery dissection.

Fifty-one patients with intracranial chordomas who were surgically treated between 1960 and 1984 were studied. Median patient age was 46 years, and 73% presented with diplopia or headache. Nineteen tumors were classified as the "chondroid" type. The extent of surgical removal was a biopsy in 11 patients and subtotal removal or greater in 40. Thirty-nine patients received postoperative radiation therapy. At the time of analysis, 17 patients were alive, and the estimated 5- and 10-year survival rates were 51% and 35%, respectively, for the group of 51 patients. Univariate analysis showed that: 1) patients undergoing resection lived longer (the 5-year survival rate was 36% for the 11 biopsy patients compared with 55% for the 40 patients who had resection; 2) patients who underwent postoperative radiotherapy tended to have longer disease-free survival times; and 3) overall and disease-free survival data were the same for patients with chondroid tumors and those with typical chordomas. Multivariate analysis showed that: 1) age was the factor most strongly associated with longer overall and disease-free survival; 2) diplopia was associated with longer survival; and 3) tumoral mitotic activity tended to be associated with shorter disease-free survival. One tumor metastasized to the cervical cord, and two tumors underwent anaplastic transformation. These data suggest that the prognosis in patients with chordomas is unfavorable, young age is the single factor most strongly associated with longer survival, surgical resection is beneficial, and postoperative radiotherapy may prolong disease-free survival.

In this study, we assessed the results of carotid endarterectomy in 357 patients with a carotid stenosis and contralateral carotid occlusion. The overall major neurologic morbidity was 0.6%, and the minor morbidity was 1.1%. The causes of four perioperative deaths (1.1%) were myocardial infarction in two patients, ruptured abdominal aortic aneurysm in one, and respiratory complications in one. Therefore, an excellent result was achieved in 97.2% of patients. With occlusion of the carotid artery for the endarterectomy, 165 patients (46%) had appreciable attenuation in intraoperative electroencephalographic findings and a decrease in cerebral blood flow to approximately 10 ml/100 g of brain tissue per min that necessitated placement of a shunt. This high percentage of profound electroencephalographic and blood flow changes during carotid occlusion suggests that the potential for collateral blood flow in this group of patients is minimal. These results demonstrate that a carotid endarterectomy can be performed at low risk in patients with a contralateral carotid occlusion. We advocate annual noninvasive carotid testing for patients with asymptomatic carotid stenosis and contralateral carotid occlusion. If progression of the stenosis is evident, a prophylactic endarterectomy should be considered because these patients may have a higher risk for cerebral infarction than do patients with a unilateral asymptomatic stenosis.

An alternative theory is proposed to explain the brain edema and hemorrhage that may occur after resection of high-flow intracerebral arteriovenous malformations (AVM's). This theory, termed "occlusive hyperemia," is based on a retrospective analysis of operative dictations along with postoperative imaging studies (191 angiograms and 273 computerized tomography scans) in 295 cases of intracerebral AVM's operated on at the Mayo Clinic between 1970 and 1990. In this series, 34 cases (12%) of postoperative deterioration were documented, of which 15 were due to incomplete resection of the AVM. Of the remaining 19 cases, six had brain edema alone and 13 had hemorrhage with edema, despite complete excision of the AVM. In these 19 cases, the AVM's were greater than 6 cm in diameter in 10 patients, between 3 and 6 cm in six, and less than 3 cm in three. Obstruction of the venous drainage system was observed in 14 (74%) of the 19 cases. Ten of these 14 were due to obstruction of the primary venous drainage of the brain parenchyma immediately surrounding the lesions, while four were due to obstruction of other venous structures. In no case was a rapid circulation identified on postoperative angiograms. The flow pattern was slow or stagnant in former AVM feeders and their parenchymal branches. It is proposed that postoperative intracranial hemorrhage and/or brain edema in AVM patients may be due to: 1) obstruction of the venous outflow system of brain adjacent to the AVM, with subsequent passive hyperemia and engorgement; and 2) stagnant arterial flow in former AVM feeders and their parenchymal branches, with subsequent worsening of the existing hypoperfusion, ischemia, and hemorrhage or edema into these areas. Supportive hemodynamic evidence for this theory was derived from the literature.

A series of 280 cases of cerebral arteriovenous malformations (AVM's) treated surgically between June, 1970, and June, 1989, is reviewed with particular focus on the preoperative seizure history and follow-up seizure status. Follow-up evaluation (mean duration 7.5 years) was achieved in 98% of cases and was accomplished through re-examinations, telephone interviews, and written questionnaires. Overall, 89% of the surviving patients with a follow-up period of greater than 2 years were free of seizures at last examination. Of the 280 patients in this series, 163 had experienced no seizures preoperatively. A recent follow-up study (with a minimum duration of 2 years or to death) was available in 157 of these 163 cases; 21 patients had died. Of the 136 surviving patients, only eight (6%) were having new ongoing seizures. In the 128 (94%) who had remained seizure-free, 73% were receiving no anticonvulsant agents while 27% were taking anticonvulsant prophylaxis. The 2-year minimum follow-up study in 110 of the 117 patients with preoperative seizures revealed that eight (7%) had died. Of the 102 surviving patients, 85 (83%) were seizure-free (with 48% no longer receiving anticonvulsant therapy), while 17 (17%) still suffered intermittent seizures. However, of these 17 patients, 13 reported their seizures to be improved compared to preoperatively; the seizures were the same in two patients and were worse in two patients. An actuarial analysis was conducted comparing the life expectancy of patients following surgery for AVM's with the expected survival of a general white population of the same age and sex in the West Northcentral region of the United States. No statistically significant difference was found. There were seven perioperative deaths (three from cerebral hemorrhage, two from pulmonary emboli, and two from obstruction of venous drainage) and 22 deaths during the follow-up period. Of these 22 deaths, the cause was unknown in four patients, apparently unrelated to the AVM in 13, and directly or indirectly related to the patient's neurological condition prior to surgery or due to surgery performed for resection of the AVM in five. There was a statistically significant relationship between the size and location of the AVM and the clinical presentation. Patients with small AVM's (< 3 cm) were more likely to present with hemorrhage whereas those with large AVM's were more likely to present with seizures.(ABSTRACT TRUNCATED AT 400 WORDS)

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)

In a recent study from the Mayo Clinic on the natural history of intact saccular intracranial aneurysms, none of the aneurysms smaller than 10 mm in diameter ruptured. It was concluded that these aneurysms carry a negligible risk for future hemorrhage and that surgery for their repair could not be recommended. These findings and recommendations have been the subject of much controversy. The authors report three patients with previously documented asymptomatic intact saccular intracranial aneurysms smaller than 5 mm in diameter that subsequently ruptured. In Case 1, a 70-year-old man bled from a 4-mm middle cerebral artery aneurysm that had been discovered incidentally 2 1/2 years previously during evaluation of cerebral ischemic symptoms. A 10-mm internal carotid artery aneurysm and a contralateral 4-mm middle cerebral artery aneurysm had not ruptured. Case 2 was that of a 66-year-old woman who bled from a 4-mm pericallosal aneurysm that had been present 9 1/2 years previously when she suffered subarachnoid hemorrhage (SAH) from a 7 x 9-mm posterior inferior cerebellar artery aneurysm. Although the pericallosal aneurysm had not enlarged in the intervening years, a daughter aneurysm had developed. The third patient was a 45-year-old woman who bled from a 4- to 5-mm posterior inferior cerebellar artery aneurysm that had measured approximately 2 mm on an angiogram obtained 4 years previously; at that time she had suffered SAH due to rupture of a 5 x 12-mm posterior communicating artery aneurysm. These cases show that small asymptomatic intact saccular intracranial aneurysms are not innocuous and that careful consideration must be given to their surgical repair and long-term follow-up study.

Spontaneous dissections of the internal carotid arteries are uncommon but are not rare. They constitute a fairly common cause of ischemic stroke in young patients (young in terms of the age at which strokes generally occur). The common presenting manifestations are (1) unilateral headaches followed after a period of delay by focal cerebral ischemic symptoms or (2) unilateral headaches and ipsilateral incomplete Horner's syndrome. These may or may not be associated with subjective or objective bruits. In rare instances, spontaneous dissections of the internal carotid arteries may present as lower cranial nerve palsies and cause dysphonia, dysarthria, dysphagia, and numbness of the throat. Affected patients may initially present to the otolaryngologist or be referred to one. This article describes eight patients with spontaneous dissections of the internal carotid arteries and lower cranial nerve palsies, and the pertinent literature is reviewed.

The authors report a series of seven patients with myelopathy who were found to have spinal dural arteriovenous (AV) fistulas in which the nidus was located at some distance from the spinal cord. The nidus was intracranial in three cases and involved a sacral nerve root sheath in the other four; in each case, the arterialized draining vein led into the coronal plexus of medullary veins. A lack of normal draining radicular veins was noted in all cases. Magnetic resonance images were obtained in four patients and demonstrated spinal cord tissue changes only in the lower thoracic cord in three cases and in the cervical cord in one, all consistent with an ischemic process secondary to venous hypertension. Five patients were managed surgically by division of the draining vein, with improvement of the neurological deficit in all. One patient was treated by embolization alone and had stabilization of her deficit. The remaining patient in the series died of unrelated systemic disease before the spinal dural AV fistula could be treated. These cases support the theory that venous hypertension is the dominant pathophysiological mechanism involved in spinal dural AV fistulas independent of their location. In patients with a suspected spinal dural AV fistula, lumbar and thoracic spinal angiography will reveal the site of the fistula in the majority of cases (88% in this series). In the remaining patients, the possibility of a remote fistula must be considered. The lack of normal venous drainage of the cord following injection in the artery of Adamkiewicz is the most reliable indicator of venous hypertension in the cord and can be helpful in making the decision to proceed with a search for a cranial or sacral arterial supply.

A neonate presented with anatomically discrete cerebral arteriovenous fistulae located in the right sylvian fissure and the cerebellar vermis that were initially detected by prenatal ultrasonography. Following delivery of the baby by caesarean section, both malformations were treated by surgical obliteration. These intracranial vascular lesions were associated with cardiac anomalies and a periductal coarctation of the aorta, which was treated with a left subclavian rotational arterial pedicle repair. Follow-up examination of the infant at age 13 months demonstrated an excellent clinical result with normalization of the circulation. The pathophysiology of this syndrome is discussed and the literature reviewed.

The pathogenesis of intracranial aneurysms and spontaneous cervical artery dissection is incompletely understood but a primary arteriopathy, possibly similar in both disorders, may be of importance. To investigate the frequency of intracranial aneurysms in patients with spontaneous cervical artery dissection, the angiograms of 164 patients who were diagnosed at the Mayo Clinic as having spontaneous extracranial carotid or vertebral artery dissection were reviewed. Thirteen intracranial aneurysms were detected in nine (5.5%) of the 164 patients: eight (8.8%) of the 91 female patients and one (1.4%) of the 73 male patients. The frequency of intracranial aneurysms in these patients was significantly higher (p less than 0.01) than that observed in a recent angiographic study from the same institution, estimating the frequency of intracranial aneurysms in the general population (1.1%). The significance of these findings is discussed.

Background and Purpose: The familial occurrence of intracranial aneurysms and cervical artery dissections has been described in different families and supports the hypothesis that a primary arteriopathy may play a role in the pathogenesis of these disorders. Although the basis for this arteriopathy is generally not believed to be similar among cases of intracranial aneurysms and cervical artery dissections, several similarities exist in the epidemiology of these disorders and a common underlying arterial abnormality may be suspected., Summary of Reports: The medical records of all 175 patients with spontaneous dissections of the cervical arteries who were seen at the Mayo Clinic between 1970 and 1989 were reviewed to identify families in which intracranial aneurysms and cervical dissections coexisted. Three families were identified in which intracranial aneurysms and cervical artery dissections were observed among siblings. These families are described in detail., Conclusions: The familial occurrence of intracranial aneurysms and cervical artery dissections within the same families provides support to the importance of a common underlying arteriopathy in the pathogenesis of both these disorders. The underlying vascular defect may, at least in some cases, be inherited.

Neurofibromatosis is of particular interest to neurosurgeons because of the various central and peripheral nervous system tumors and, more rarely, cervicocerebral arterial lesions associated with the disease. In the present paper, we report two patients with neurofibromatosis Type 1 (von Recklinghausen's disease) who had anomalies of the extracranial vertebral arteries. A large extracranial vertebral artery aneurysm was incidentally discovered in the first patient, a 43-year-old woman, after rupture of a subclavian artery aneurysm. The second patient, a 28-year-old woman, had an enlarging neck mass and was found to have an extensive extracranial vertebral artery arteriovenous fistula. The vascular lesions associated with neurofibromatosis Type 1 in general and those arising from the extracranial vertebral artery in particular are reviewed.

A 28-year-old man had a 5-month history of focal and generalized neurologic symptoms culminating in a thoracic myelopathy. Evaluation revealed granulomatous angiitis of the spinal cord in association with occult nodular sclerosing Hodgkin's disease. In previous reports, manifestations indicative of intracranial involvement have dominated the clinical presentation of granulomatous angiitis associated with Hodgkin's disease. Successful therapy for Hodgkin's disease may result in marked improvement of associated granulomatous angiitis, whereas the lack or failure of therapy results in a uniformly fatal outcome. Definitive antemortem diagnosis of granulomatous angiitis requires a biopsy of involved tissue. The cause of granulomatous angiitis, as well as the nature of its association with Hodgkin's disease, remains unexplained.

Spontaneous bilateral carotid-cavernous fistulae and cervical artery dissection is reported in a 20-year-old woman with Ehlers-Danlos syndrome Type IV. The clinical features of 16 previously published cases of spontaneous carotid-cavernous fistulae associated with Ehlers-Danlos syndrome Type IV are reviewed, for a total of 17 cases. The mean age of the 14 women and three men was 31.6 years. Only direct fistulae were encountered. Diagnostic neuroangiography carried morbidity and mortality rates of 36% and 12%, respectively; neuroradiological treatment resulted in death in one of six patients. The possible value of desmopressin in the management of these patients is discussed. In view of the risks of arterial puncture and surgery, the authors emphasize the importance of early recognition of Ehlers-Danlos syndrome.

The case of an ectopic craniopharyngioma arising from a seed of tissue deposited along the operative track is reported. The uniqueness of this lesion is addressed. Ideal therapy and controversies regarding radiation therapy of craniopharyngiomas are discussed in light of this new variation in recurrence.

Modern aneurysm clips with newer metallurgic properties and state-of-the-art designs have evolved through the years. Regretfully, manufacturers are still not required to provide product information regarding clip strength, and when such information is supplied, there is no interbrand consistency to how it is obtained. This experiment compares the closing pressures of 5 commonly used brands of aneurysm clips: Yasargil, Sugita, Sundt-Kees, Vari-Angle McFadden, and Heifetz. Interclip comparison is also made of opening pressures, that is, the arterial pressure required to open an aneurysm clip. Findings show a distinct difference in closing forces among these brands of aneurysm clips. The study of opening forces indicates that the important factors determining clip release or displacement were the failure of the clip to cross the vessel completely and intrinsic characteristics of the vascular wall.

A prospective study was done of complications associated with 134 consecutive diagnostic spinal cord arteriograms in 96 patients (63 men and 33 women aged 17-78 years). Patients were examined for either arteriovenous malformation (n = 88) or tumor (n = 8), as indicated by myelography. Among the complications, 11 (8.2%) were local, five (3.7%) were systemic nonneurologic, and three (2.2%) were neurologic (two were associated with full recovery in less than 24 hours, and one was associated with full recovery in less than 1 week). No specific clinical or technical factors were significantly associated with the development of neurologic complications. Details of the clinical profile, angiographic technique, and pathologic findings for each patient were recorded and analyzed with respect to the potential risk for arteriographic complications. Diagnostic spinal cord arteriography had an acceptable risk within the range of other neuroangiographic diagnostic procedures.

Venous stasis retinopathy associated with carotid occlusive disease has been found to diminish or even disappear after carotid artery bypass surgery (superficial temporal artery to middle cerebral artery anastomosis). We present photographic documentation of these changes and subsequent resolution of the retinopathy in a patient who had bypass surgery. This resolution reflects the increased perfusion to the retina and to the cerebral hemisphere.

The authors conducted a long-term follow-up study of 168 patients to define the natural history of clinically unruptured intracranial arteriovenous malformations (AVM's). Charts of patients seen at the Mayo Clinic between 1974 and 1985 were reviewed. Follow-up information was obtained on 166 patients until death, surgery, or other intervention, or for at least 4 years after diagnosis (mean follow-up time 8.2 years). All available cerebral arteriograms and computerized tomography scans of the head were reviewed. Intracranial hemorrhage occurred in 31 patients (18%), due to AVM rupture in 29 and secondary to AVM or aneurysm rupture in two. The mean risk of hemorrhage was 2.2% per year, and the observed annual rates of hemorrhage increased over time. The risk of death from rupture was 29%, and 23% of survivors had significant long-term morbidity. The size of the AVM and the presence of treated or untreated hypertension were of no value in predicting rupture.

Spontaneous dissections of the extracranial internal carotid artery are diagnosed more frequently as their clinical and angiographic features are more widely recognized. Familial occurrence of spontaneous dissection of the internal carotid artery has not been previously reported. We describe the occurrence of this entity in a mother and daughter and also in a father and son. The familial occurrence of spontaneous dissection of the internal carotid artery raised the possibility of an inherited disorder of the blood vessel wall that predisposes the artery to this disorder. Fibromuscular dysplasia is suspected to be the underlying arterial disease, but other unknown arteriopathies cannot be excluded.

Selected patients with acute or continuing ischemic symptoms from occlusions or inaccessible stenotic lesions of the internal carotid artery or middle cerebral artery have been considered candidates for a carotid artery-middle cerebral artery bypass procedure at our institution since July 1974. We report herein an 8-year experience through June 1982 with 415 operations in 403 patients in whom a branch of the superficial temporal artery was anastomosed to a branch of the middle cerebral artery. Patients selected for operation usually had had more than one form of ischemic symptom. The primary indication for operation was transient ischemic attacks, and the most common vascular pathologic condition was internal carotid artery occlusion. Preoperatively, 183 patients were taking antiplatelet agents and 157 were taking anticoagulants. Neurologic function 6 months postoperatively was equal to or better than the function preoperatively in 95% of survivors. The bypass pedicle was patent in 99% of patients studied. The mortality and morbidity associated with the surgical procedure varied on the basis of the patient's preoperative neurologic condition but were 1% and 4%, respectively, for the entire group at 30 days postoperatively. Of the 54 deaths during the entire follow-up period, 27 were cardiogenic and 6 were from ischemic stroke. On the basis of patient-months of follow-up, stroke was 8 times more likely to occur within 6 months after operation than thereafter.