The epidemiology of amyotrophic lateral sclerosis.

Amyotrophic lateral sclerosis (ALS) is the most common motor neuron disease in adults and is characterized by neurodegeneration of motor neurons in the brain and spinal cord. The incidence of ALS is approximately 1-2.6 cases per 100 000 persons annually, whereas the prevalence is approximately 6 cases per 100 000. The average age of onset of ALS is currently 58-60 years and the average survival from onset to death is 3-4 years. Between October 19, 2010 and December 31, 2011, there were an estimated 12 187 prevalent cases diagnosed with definite ALS in the USA alone. Sporadic ALS (90-95%) constitutes the large majority of cases, while the remaining 5-10% are hereditary and termed familial ALS. Sporadic ALS is suspected to involve genetic susceptibility to environmental risk factors. The purpose of this review is to present a clinical overview of ALS and provide an epidemiologic summary of personal and environmental risk factors shown to be related to the risk of disease. A discussion of the most recent research initiatives is also included.

Clip occlusion versus coil embolization for the treatment of cerebral aneurysms.

Advances in the endovascular treatment of intracranial aneurysms since the advent of detachable coil embolization continue to expand the spectrum of lesions amenable to minimally invasive therapy. The feasibility of treating a given cerebral aneurysm by a given open or endovascular modality does not necessarily make such an approach the optimal choice. Rather, any given cerebral aneurysm and patient should be carefully analyzed on a multitude of parameters which are based on available adjunctive technology, aneurysm morphology and characteristics, endovascular vs. microsurgical accessibility, and long-term angiographic outcome. In addition to patient age, co-morbid conditions, lesion size and attendant risk, one must also consider patient preference with respect to length of hospital stay, recovery duration and required follow-up and radiographic assessments. The relentless improvements in minimally invasive embolization therapies ranging from coiling with adjunctive balloon and stent support to the emerging role of flow-diversion must be balanced against the lower cost and complexity associated with longitudinal clinical and radiographic follow-up requirements of microsurgical clipping. This review will consider the various factors involved in the determination of optimal modality choice based on an assessment of clinical, morphological and anatomical considerations. In this rapidly evolving field, the quest for maximizing protection from aneurysm rupture at the lowest neurological cost dictates that a balance be maintained between technical virtuosity and procedural safety of either microsurgical clipping or endovascular repair to insure that the advantages of the selected modality not be negated by its associated shortcomings.

Stent-assisted coiling of intracranial bifurcation aneurysms leads to immediate and delayed intracranial vascular angle remodeling.

BACKGROUND AND PURPOSE: Wide-neck bifurcating aneurysms are increasingly treated with intracranial stent-assisted coiling by using shape-memory alloy microstents. We sought to investigate the short- and long-term effects of intracranial stent implantation on the geometry and angular conformation of the stent-coiled vascular bifurcation. MATERIALS AND METHODS: Thirty patients underwent stent-mediated coiling for 31 bifurcation aneurysms by using 31 self-expanding Neuroform (n = 14) and Enterprise (n = 17) stents (17 women; mean age, 56 years). The angle (δ) between the stented mother and daughter vessels at the bifurcation was measured by using multiplanar imaging of reconstructed rotational conventional angiography volumes and was compared by using matched-pair statistics. Neuroform and Enterprise longitudinal stent stiffness was measured in vitro at an increasing bending angle θ (θ = 180°-δ). RESULTS: Stent deployment increased the bifurcation angle δ from 101.5° to 119.8° postprocedurally and to 137.3° (P < .0001) at latest follow-up, resulting in effective straightening; the angular remodeling was greater in distal-versus-proximal arteries (anterior cerebral > MCA > BA > ICA), inversely proportional to mother-vessel diameter and proportional to pretreatment bending angle θ. At follow-up, angle δ continued to significantly expand, with remodeling being greater in the early period (1-6 versus >7 months) and more pronounced with the longitudinally stiffer closed-cell Enterprise compared with the open-cell Neuroform stent. CONCLUSIONS: Stent placement across bifurcation aneurysms leads to a significant biphasic angular remodeling related to stent type and vessel caliber, altering morphology to mimic sidewall lesions, a phenomenon needing consideration during procedural planning. Future work is needed to uncover the hemodynamic implications of this structural change and any possible effect on aneurysm-recurrence rates.

Parent vessel size and curvature strongly influence risk of incomplete stent apposition in enterprise intracranial aneurysm stent coiling.

BACKGROUND AND PURPOSE: Flexible microstents, such as the closed-cell EN, have facilitated adjunctive coiling of intracranial aneurysms. Little data are available on the ability of the stent struts to maintain vessel-wall apposition once deployed in the tortuous cerebral vasculature and the prevalence of ISA. The purpose of this study was to evaluate the relationship between geometric features of the parent vessel at the stent deployment site and prevalence of ISA. MATERIALS AND METHODS: Postprocedural 3T-MRA was performed in a cohort of 39 patients undergoing EN stent-assisted intracranial aneurysm coiling. 3T-MRA was analyzed for the presence of ISA and supplemented by angiographic C-arm FPCT (DynaCT). Parent vessel diameter, curvature radius, and stent-subtended arc angle were measured at the site of deployment and analyzed for prediction of ISA in the ICA. RESULTS: 3T-MRA uncovered a unique crescent flow pattern (CS) outside the EN struts, which was confirmed by FPCT to indicate ISA resulting from EN crimping. ISA was detected on 3T-MRA in 19/39 patients (49%). Univariate analysis revealed ISA in the ICA to correlate with a large stent-subtended angle, a small curvature radius, and a large diameter but not stent length or jailing versus a sequential technique. Multivariate analysis identified ISA to correlate with vessel-curvature radius (OR, 253; P = .009), stent-subtended angle (OR, 225; P = .005), and parent vessel diameter (OR, 8.49; P = .044). CONCLUSIONS: In this study, ISA was detectable by 3T-MRA in a significant proportion of patients undergoing EN stent-assisted coiling of ICA aneurysms in a vessel geometry- and stent-deployment location-dependent manner. This characteristic of EN coiling at this potentially tortuous location should be taken into account when selecting an endovascular strategy.

Endovascular treatment of a ruptured dual aperture cavernous aneurysm.

The authors describe the diagnosis and endovascular management of a multiaperture, ruptured cavernous internal carotid artery aneurysm causing a carotid cavernous fistula (CCF) using both transarterial and transvenous techniques. Although uncommon, recognition of the imaging characteristics of such a lesion will aide in successful management and improve treatment outcome. To the authors' knowledge, CCF due to a ruptured cavernous aneurysm with multiple shunts has not been previously reported.

Public-private partnerships in healthcare: criteria for success.

Public-private partnerships present opportunities for healthcare providers to meet the dual challenges of shrinking public funding and increasing demand for care. Partnering with private sector organizations can provide new sources of capital, expertise and technology. Successful partnerships require specific skills and strategies. This article describes a pilot study that explored the literature and the experiences of participants in a small sample of interviews to compile a set of criteria for successful public-private healthcare partnerships.

Primary stenting for high-grade basilar artery stenosis.

We report two patients with symptomatic high-grade stenosis of the basilar artery refractory to appropriate maximal medical therapy in whom endovascular stenting was performed successfully without preliminary balloon angioplasty. Excellent angiographic results were achieved and there were no procedural or periprocedural complications. The patients were asymptomatic and neurologically intact at a mean clinical follow-up of 6.5 months. Primary stenting of basilar artery stenosis may be an alternative to balloon angioplasty for patients with symptomatic lesions refractory to medical therapy or in whom anticoagulation is contraindicated.

Carotid artery stent placement for atherosclerotic disease: rationale, technique, and current status.

Carotid arterial endarterectomy is considered to be the standard for the treatment of atherosclerotic carotid arterial occlusive disease. This has been validated with results of several randomized controlled trials in which its effectiveness has been demonstrated over that of the best nonsurgical therapy. In the past several years, however, carotid angioplasty with stent placement has emerged as a potential alternative to carotid endarterectomy. This article represents a critical examination of the rationale for carotid revascularization; the history of endovascular techniques for the treatment of carotid atherosclerosis, beginning with balloon angioplasty and evolving to the use of stents; and the evidence supporting the effectiveness of the endovascular approach. A brief description of the current technical aspects of carotid artery stent placement is presented. The future status of the endovascular approach will be determined with randomized trials in which carotid artery stent placement is directly compared with endarterectomy, as well as by the potential for further innovation and improvement in endovascular devices, technique, and safety.

Endovascular management of extracranial carotid artery dissection achieved using stent angioplasty.

BACKGROUND AND PURPOSE: Dissection of the carotid artery can, in certain cases, lead to significant stenosis, occlusion, or pseudoaneurysm formation, with subsequent hemodynamic and embolic infarcts, despite anticoagulant therapy. We sought to determine the therapeutic value of stent-supported angioplasty retrospectively in this subset of patients who are poor candidates for medical therapy. METHODS: Five men and five women (age range, 37-83 years; mean age, 51.2 years) with dissection of the internal (n=9) and common (n=1) carotid artery were successfully treated with percutaneous endovascular balloon angioplasty and stent placement. The etiology was spontaneous in five, iatrogenic in three, and traumatic in two. Seven of the treated lesions were left-sided and three were right-sided. RESULTS: The treatment significantly improved dissection-related stenosis from 74+/-5.5% to 5.5+/-2.8%. Two occlusive dissections were successfully recanalized using microcatheter techniques during the acute phase. Multiple overlapping stents were needed in four patients to eliminate the inflow zone and false lumen and establish an angiographically smooth outline within the true lumen. There was one case of retroperitoneal hemorrhage, but there were no procedural transient ischemic attacks (TIAs), minor or major strokes, or deaths (0%). Clinical outcome at latest follow-up (16.5+/-1.9 months) showed significant improvements compared with pretreatment modified Rankin score (0.7+/-0.3 vs 1.8+/-0.44) and Barthel index (99.5+/-0.5 vs 80.5+/-8.9). One delayed stroke occurred in a treated patient with contralateral carotid occlusion following a hypotensive uterine hemorrhage at 8 months; the remaining nine patients have remained free of TIA or stroke. CONCLUSION: In select cases of carotid dissection associated with critical hemodynamic insufficiency or thromboembolic events that occur despite medical therapy, endovascular stent placement appears to be a safe and effective method of restoring vessel lumen integrity, with good clinical outcome.

Endovascular treatment of cerebral artery aneurysms during pregnancy: report of three cases.

Historically, surgical management of cerebral aneurysms during pregnancy has been controversial. Debate originally focused on early versus late intervention, before or after delivery of the fetus. More recently, treatment has centered on rapid intervention. We describe the endovascular treatment of cerebral artery aneurysms with Guglielmi detachable coils in three pregnant women.

Cerebral hyperperfusion syndrome after percutaneous transluminal stenting of the craniocervical arteries.

OBJECTIVE: Cerebral hyperperfusion syndrome is a recognized complication of carotid endarterectomy, with a reported incidence of 0.3 to 1.2%. The incidence of cerebral hyperperfusion after endovascular revascularization procedures of the craniocervical arteries remains unknown. We evaluated the incidence of cerebral hyperperfusion syndrome in our endovascular revascularization series. To our knowledge, there are no previous studies evaluating the incidence of hyperperfusion syndrome after percutaneous transluminal angioplasty/stenting. METHODS: Between March 1996 and February 2000, 140 patients underwent percutaneous transluminal angioplasty/stenting of the craniocervical arteries at our institution. In all patients, selective bilateral arteriography of the carotid and vertebral arteries was performed to document the sites of craniocervical stenosis and collateral blood flow and the results of the endovascular revascularization procedure. We then reviewed all pertinent medical records, arteriographic films, and sectional imaging studies to determine the incidence of cerebral hyperperfusion in this series. RESULTS: Seven patients (5.0%) developed clinical or radiological manifestations of cerebral hyperperfusion. In the target group, percutaneous transluminal stenting achieved a 90 to 100% reduction in stenotic lesions (mean stenosis, 91%) of the carotid (n = 5) and vertebral (n = 2) arteries. All seven patients remained neurologically stable immediately after treatment. There was delayed development of clinical and radiographic findings, suggestive of cerebral hyperperfusion. Six patients showed evidence of ipsilateral hemispheric edema, including two patients who developed intracranial hemorrhage (one parenchymal, one parenchymal and subarachnoid) documented by computed tomographic brain scans. Symptoms resolved within 72 hours in the four patients without hemorrhage. The two patients with hemorrhage recovered during a more protracted period (range, 3 wk to 6 mo). There were no long-term sequelae or deaths during a cumulative follow-up of 84 months (mean follow-up, 12 mo). CONCLUSION: Hyperperfusion syndrome is an uncommon but potentially serious complication of extracranial and intracranial angioplasty and stenting procedures. The clinical manifestations are similar to hyperperfusion syndrome after carotid endarterectomy; however, the prevalence may be greater in the high-risk cohort commonly referred for endovascular treatment. Our findings suggest that patients undergoing endovascular stenting procedures should be closely monitored for evidence of hyperperfusion, with careful monitoring of blood pressure, heart rate, and anticoagulation. Further research is needed to confirm that cerebral hyperperfusion is the pathogenesis of this condition.

Stent-supported coil embolization: the treatment of fusiform and wide-neck aneurysms and pseudoaneurysms.

OBJECTIVE: We describe a consecutive series of patients treated with endovascular stent-supported coil embolization for symptomatic or enlarging wide-neck and fusiform aneurysms and pseudoaneurysms of the carotid and vertebrobasilar arteries. METHODS: Seven stent-supported coil embolization procedures were performed for seven aneurysms in seven consecutive patients. There were five pseudoaneurysms, one dissecting aneurysm, and one berry aneurysm. Four aneurysms were located in the carotid artery, and three were located in the vertebrobasilar system. Three aneurysms were intracranial. Four patients were symptomatic, and three had angiographic evidence of increasing aneurysm size. RESULTS: Technical success was achieved in six (86%) of seven patients. Entanglement of a coil with the stent struts necessitated partial coil delivery into the parent artery in one patient, but there were no neurological or other adverse sequelae. The 30-day rate of periprocedural stroke or mortality was 0%. At a mean clinical follow-up of 14.5 months, neurological status was at baseline or better in all patients. To date, all treated patients remain clinically asymptomatic with oral administration of aspirin only. CONCLUSION: Stent-supported coil embolization represents an emerging therapeutic alternative to surgery for the treatment of symptomatic or enlarging wide-neck and fusiform aneurysms and pseudoaneurysms of the cervical and vertebrobasilar arteries, which are not amenable to conventional unsupported coil embolization. Experience with greater numbers of patients and long-term follow-up are required to further validate this technique.

Balloon-assist technique for endovascular coil embolization of geometrically difficult intracranial aneurysms.

OBJECTIVE: The balloon-assist or neck-remodeling technique is an adjunctive method devised for the endovascular coil embolization of aneurysms characterized by a wide neck or unfavorable geometric features. Since its initial description, there have been few data to corroborate its utility, efficacy, and safety in aneurysm embolization. METHODS: Twenty patients (19 female patients and 1 male patient) with 22 aneurysms (19 unruptured aneurysms and 3 ruptured aneurysms) underwent balloon-assisted coil embolization. The balloon-assist technique was performed in the same treatment session after conventional coil embolization had failed in 55% of cases (12 of 22 cases) and was the primary treatment in 45% of cases. The majority of aneurysms were located in the supraclinoid carotid artery (13 paraophthalmic and 3 superior hypophyseal aneurysms). The mean angiographic measurements included a fundus of 8.7 +/- 3.7 mm, a neck of 5.3 +/- 2.2 mm, and a comparatively unfavorable fundus/neck ratio of 1.33 +/- 0.23. RESULTS: Technical success was achieved in 77% of cases (17 of 22). The rate of aneurysm obliteration at the end of the procedures was 97 +/- 3.8%. Angiographic follow-up data (mean follow-up period, 10.3 mo) obtained for 89% of the treated aneurysms (15 of 17) confirmed stable mean occlusion of 97.8 +/- 3.8%. Technical complications included two cases of asymptomatic distal vessel thromboembolism, which resolved angiographically within 24 hours, and one case of intraprocedural rupture of an arteriovenous malformation-related feeder artery aneurysm, which resulted in no neurological deficits and required no further treatment (transient complication rate, 13.6%; 3 of 22 cases). There were no deaths and no procedure-related 30-day or permanent morbidity. CONCLUSION: The balloon-assist method of coil embolization is characterized by promising intermediate-term angiographic and clinical outcomes and acceptable morbidity and mortality rates. Although this adjunctive method requires the use of an additional microcatheter and consequently involves a higher level of technical complexity, it extends the range of aneurysms that can be successfully treated with electrolytically detachable coils via an endovascular approach.

Hemorrhagic complications in vein of Galen malformations.

The authors report on a series of spontaneous intracranial hemorrhages associated with vein of Galen aneurysmal malformations (VGAMs). Thirty-four children with VGAMs have been treated at this institution since 1986. Eight children (24%) harbored the mural-type malformation, and 26 (76%) had the choroidal-type lesion. Two children (25%) with mural lesions and 1 (4%) with a choroidal lesion suffered hemorrhagic complications. Two presented with acute intracranial hemorrhage. A third child developed acute intracranial hemorrhage due to delayed dural sinus thrombosis after endovascular treatment of his choroidal-type VGAM. The subjects ranged in age from 13 days to 17 months at the time of presentation. Each patient underwent rapid radiological evaluation and treatment with endovascular surgery. Post-procedural arteriography demonstrated complete occlusion of the malformation in each patient. For the 3 patients with hemorrhage, follow-up has taken place over 49-, 107-, and 43-month intervals, respectively. Vein of Galen aneurysmal malformations can present with acute intracranial hemorrhage or develop delayed intracranial hemorrhage but respond to treatment using standard endovascular techniques. The presence of hemorrhage does not de facto portend a poor prognosis.

Endovascular stenting for carotid artery stenosis: preliminary experience using the shape-memory- alloy-recoverable-technology (SMART) stent.

We describe our initial clinical experience using the newly available self-expanding, Nitinol, shape-memory-, alloy-recoverable-technology (SMART) stent in treating carotid artery occlusive disease. Five stents were used in four carotid arteries in four consecutive patients with carotid stenosis of at least 70%. Technical success (<20% residual stenosis) was achieved in all cases. No procedural complications specifically related to use of the SMART stent were encountered. All patients remained symptom-free, with no evidence of transient ischemic attacks or new strokes during an average follow-up period of 6 months. Excellent performance of the SMART stent for the endovascular treatment of carotid artery stenosis has been shown based on our early experience. Validation with greater numbers and longer-term follow-up is required. The specific technical characteristics, potential advantages, and disadvantages of this stent are discussed and compared with other currently used carotid artery stents.

Endovascular treatment of noncarotid extracranial cerebrovascular disease.

The last two decades have witnessed a growing application of endovascular techniques for the treatment of atherosclerotic disease of the extracranial vertebral arteries, subclavian arteries, and brachiocephalic artery. Beginning with simple balloon angioplasty, these minimally invasive techniques have now progressed to the use of stent-supported angioplasty. Stent-supported angioplasty is currently providing a therapeutic alternative to traditional methods of open surgery for revascularization of these vessels and increasing the therapeutic options available for patients who have failed maximal medical therapy. Additionally, endovascular techniques are also being used successfully to treat a variety of nonatherosclerotic diseases affecting the noncarotid extacranial arteries, such as inflammatory, radiation, and anastomotic-graft strictures; acute intimal dissection; traumatic and spontaneous arteriovenous fistulas; and aneurysms or pseudoaneurysms. Continued innovation and refinement of endovascular devices and techniques will inevitably improve technical success rates, reduce procedure-related complications, and broaden the endovascular therapeutic spectrum for extracranial cerebrovascular disease.

Patient presentation, angiographic features, and treatment of strangulation-induced bilateral dissection of the cervical internal carotid artery. Report of three cases.

Domestic violence leading to strangulation by an abusive spouse can cause carotid artery dissection. This phenomenon is rare and has been described in only three previous instances. The authors present their management strategies in three additional cases. Three young women aged 24 to 43 years were victims of manual strangulation committed by their spouses 3 months to 1 year before presentation. Two of the patients suffered delayed cerebral infarctions before presentation and angiography demonstrated focal, mirror-image severe residual stenoses in the high-cervical internal carotid artery (ICA), which were characteristic of a healed chronic dissection; there was no evidence of fibromuscular dysplasia. One of these patients underwent unilateral percutaneous angioplasty with stent placement, and the other underwent bilateral percutaneous angioplasty. Both patients have recovered from their strokes and remain clinically stable at 8 and 20 months posttreatment, respectively. The third patient presented with bilateral ischemic frontal watershed infarctions resulting from an occluded left ICA and a severely narrowed right ICA. Given the extent of the established infarctions, this case was managed with a long-term regimen of anticoagulation medications, and the patient remains neurologically impaired. These cases illustrate the susceptibility of the manually compressed ICA to traumatic injury as a result of domestic violence. They identify bilateral symmetrical ICA dissection as a consistent finding and the real danger of delayed stroke as a consequence of strangulation. Endovascular therapy in which percutaneous angioplasty and/or stent placement are used can be useful in treating residual focal stenoses to improve cerebral perfusion and to lower the risk of embolic or ischemic stroke.

Subclavian arteritis and pseudoaneurysm formation secondary to stent infection.

Technically uncomplicated percutaneous angioplasty and stent placement of a left subclavian artery stenosis was performed in a 56-year-old man for treatment of subclavian steal syndrome and vertebrobasilar insufficiency. Six days later the patient was readmitted with Staphylococcus aureus bacteremia and stigmata of septic emboli isolated to the ipsilateral hand. Nine days later he had computed tomography (CT) evidence of a contrast-enhancing phlegmon surrounding the stent. Despite clinical improvement and resolution of bacteremia on intravenous antibiotic therapy, the phlegmon progressed, and at day 21 a pseudoaneurysm was angiographically confirmed. The patient underwent surgical removal of the stented arterial segment and successful autogenous arterial reconstruction. The possible contributory factors leading to stent infection were prolonged right femoral artery access and an infected left arm venous access. Although the role of prophylactic antibiotics remains to be defined, it may be important in cases where the vascular access sheath remains in place for a prolonged period of time.