Fulminant Anti-GAD antibody encephalitis presenting with status epilepticus requiring aggressive immunosuppression.

Antibodies against glutamic acid decarboxylase (GAD) are reported in association with numerous neurological conditions including temporal lobe epilepsy and limbic encephalitis. We report a case of Anti-GAD-Antibody associated encephalitis presenting with epilepsia partialis continua (EPC) progressing to a fulminant encephalopathy preferentially affecting the frontal lobes associated with coma and refractory status epilepticus. The abnormalities identified on MRI included marked bilateral frontal lobe involvement which has not been reported in other auto-immune encephalitides and may be specific for Anti-GAD-Antibody associated encephalitis. Similar to the majority of cases of Anti-GAD associated neurological disturbance no underlying malignancy was identified. Treatment with high dose corticosteriods, IVIG and plasmapheresis had minimal response, but escalation of treatment with rituximab and cyclophosphamide was associated with clinical improvement, reducing antibody titers and resolution of MRI changes.

Long-Term Follow-Up Results following Elective Treatment of Unruptured Intracranial Aneurysms with the Pipeline Embolization Device.

BACKGROUND AND PURPOSE: Numerous reports of treatment of wide-neck aneurysms by flow diverters have been published; however, long-term outcomes remain uncertain. This article reports the imaging results of unruptured aneurysms treated electively with the Pipeline Embolization Device for up to 56 months and clinical results for up to 61 months. MATERIALS AND METHODS: One hundred nineteen aneurysms in 98 patients from 3 centers admitted between August 2009 and June 2011 were followed at 6-month, 1-year, and 2+-year postprocedural timeframes. Analyses on the effects of incorporated vessels, previous stent placement, aneurysm size, and morphology on aneurysm occlusion were performed. RESULTS: The 1- and 2+-year imaging follow-ups were performed, on average, 13 and 28 months postprocedure. At 2+-year follow-up, clinical data were 100% complete and imaging data were complete for 103/116 aneurysms (88.8%) with a 93.2% occlusion rate. From 0 to 6 months, TIA, minor stroke, and major stroke rates were 4.2%, 3.4%, and 0.8% respectively. After 6 months, 1 patient had a TIA of uncertain cause, with an overall Pipeline Embolization Device-related mortality rate of 0.8%. An incorporated vessel was significant for a delay in occlusion (P = .009) and nonocclusion at 6 months and 1 year, with a delayed mean time of occlusion from 9.1 months (95% CI, 7.1-11.1 months) to 16.7 months (95% CI, 11.4-22.0 months). Other factors were nonsignificant. CONCLUSIONS: The Pipeline Embolization Device demonstrates continued very high closure rates at 2+ years, with few delayed clinical adverse sequelae. The presence of an incorporated vessel in the wall of the aneurysm causes a delay in occlusion that approaches sidewall closure rates by 2 years.

Endovascular treatment of intracranial aneurysms with Guglielmi detachable coils: analysis of midterm angiographic and clinical outcomes.

BACKGROUND AND PURPOSE: The previous decade has witnessed increasing application of Guglielmi detachable coils (GDCs) for the treatment of intracranial aneurysms. However, the midterm angiographic and clinical outcomes are not well documented. We report here the angiographic and clinical outcomes of patients treated with GDCs over an 8-year period. METHODS: Between 1992 and 1998, 144 patients with 160 intracranial aneurysms were treated with GDCs. Clinical follow-up data were obtained from medical records, questionnaires, and telephone interviews. Angiographic studies were reviewed by 2 neuroradiologists to obtain consensus regarding the degree of aneurysm occlusion. RESULTS: Eighty-one patients had ruptured aneurysms; 63 had unruptured aneurysms. Technical success was achieved in 91% of patients, with complete aneurysm occlusion in 46%, neck remnants in 16%, and residual body filling in 38%. Angiographic follow-up revealed that residual body filling in some aneurysms was resolved, small neck remnants were stable, and the recanalization rate decreased with time. All 63 patients with unruptured aneurysms were discharged from hospital with independent clinical status (Glasgow Outcome Score, 1 or 2). For patients with ruptured aneurysms, discharge clinical status correlated with the Hunt & Hess clinical grade at the time of treatment. Clinical follow-up for a minimum of 2 years was available in 98.5% of patients. Ninety-four percent of patients treated for unruptured aneurysms were independent at 2 years, and 82% of Hunt & Hess grade I to II patients were independent. CONCLUSIONS: Coil embolization is a safe and effective treatment for both ruptured and unruptured aneurysms. Increasing angiographic stability is demonstrated in treated aneurysms up to 3 years from coil embolization. Therefore, follow-up angiography until this time is advisable.

Stent angioplasty for cervical carotid artery stenosis in high-risk symptomatic NASCET-ineligible patients.

BACKGROUND AND PURPOSE: Although the North American Symptomatic Carotid Endarterectomy Trial (NASCET) has shown carotid endarterectomy (CEA) to be protective compared with medical therapy alone, its stringent eligibility criteria excluded patients with severe medical, angiographic, and neurological risk factors. We sought to determine the safety and efficacy of stent angioplasty in this high-risk subset for whom the perioperative morbidity and mortality of surgery are elevated. METHODS: Twenty-eight consecutive symptomatic NASCET-ineligible patients (10 female; median age, 72.2 years) underwent microcatheter-based carotid stent angioplasty. Half of the patients had sustained a previous stroke. Classification of surgical risk by Sundt criteria yielded no patients in grade 1, 3 patients in grade 2 (10.7%), 8 in grade 3 (28.6%), and 17 (60.7%) in grade 4. Stratification of stroke risk for medical therapy according to the European Carotid Surgery Trial (ECST) 5-point score showed 8 patients with a score of 3 (28.6%), 12 with 4 (42.8%), and 8 with 5 (28.6%). Follow-up was obtained in all patients at a median of 14 months. RESULTS: The procedure was technically successful in all cases (100%), with immediate stenosis reduction from a mean of 80.3% to 2.7%. There were no periprocedural deaths, 1 major stroke (3.6%), no minor strokes, and 3 transient ischemic attacks (10.7%). In-hospital complications included 2 nonfatal myocardial infarctions, 1 case of acute renal failure, and 1 groin hematoma requiring transfusion. There were 5 deaths during the follow-up period, all beyond 30 days after the procedure: 3 from cardiac causes, 1 from lung cancer, and 1 following unrelated surgery. The patient with major stroke died at 7.8 months during rehabilitation. No surviving patients had further strokes, and all except 1 (95.5%) remained asymptomatic. Anatomic follow-up in 20 patients showed occlusion in 2 (10%) (1 symptomatic, 1 asymptomatic) and intimal hyperplasia in 3 asymptomatic patients (15%). CONCLUSIONS: The clinical results and sustained freedom from symptoms and stroke during the short available follow-up period suggest that stent angioplasty may be useful in the treatment of symptomatic cervical carotid stenosis in high-risk patients despite a notable incidence of restenosis.

Use of glycoprotein IIb-IIIa inhibitor for a thromboembolic complication during Guglielmi detachable coil treatment of an acutely ruptured aneurysm.

Thrombotic occlusion of the anterior communicating and right anterior cerebral arteries occurred during embolization of an acutely ruptured aneurysm of the anterior communicating artery. Traditional management, including superselective infusion of a fibrinolytic agent, was unsuccessful in reestablishing normal vessel patency. Therefore, an intravenous dose of abciximab was administered. Serial angiography showed that normal vessel patency was reestablished within 10 min. There were no adverse events related to abciximab administration, and the patient recovered from the procedure without neurologic deficit.

Simultaneous subarachnoid hemorrhage and carotid cavernous fistula after rupture of a paraclinoid aneurysm during balloon-assisted coil embolization.

We describe an iatrogenic perforation of a paraclinoid aneurysm during balloon-assisted coil embolization that resulted in simultaneous subarachnoid contrast extravasation and a carotid cavernous fistula. The causative factors specifically related to the balloon-assisted method that led to aneurysm rupture are discussed as well as strategies for dealing with this complication.

Application of a rheolytic thrombectomy device in the treatment of dural sinus thrombosis: a new technique.

We present a novel application of a transvascular rheolytic thrombectomy system in the treatment of symptomatic dural sinus thrombosis in a 54-year-old woman with somnolence and left-sided weakness. The diagnosis of bilateral transverse and superior sagittal sinus thrombosis was made and the patient was treated with anticoagulant therapy. After an initial period of improvement, she became comatose and hemiplegic 8 days after presentation. After excluding intracerebral hemorrhage by MR imaging, we performed angiography and transfemoral venous thrombolysis with a hydrodynamic thrombectomy catheter, followed by intrasinus urokinase thrombolytic therapy over the course of 2 days. This technique resulted in dramatic sinus thrombolysis and near total neurologic recovery. Six months after treatment, the patient showed mild cognitive impairment and no focal neurologic deficit. Our preliminary experience suggests that this technique may play a significant role in the endovascular treatment of this potentially devastating disease.

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.

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.

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.

Intraventricular contrast medium leakage during ethanol embolization of an arteriovenous malformation.

We report the unusual phenomenon of abrupt intraventricular contrast medium leakage from the choroid plexus occurring during ethanol embolization of a periventricular arteriovenous malformation. There was no evidence of any associated intraventricular hemorrhage to suggest that leakage arose from a vessel perforation, as was first suspected. Intraventricular contrast medium leakage has been reported previously in the setting of ependymitis, and it is likely that similar pathogenetic mechanisms apply in this case. To our knowledge, this is the first reported case of intraventricular contrast medium leakage occurring during an embolization procedure.

Embolization of the meningohypophyseal trunk as a cause of diabetes insipidus.

We present an unusual case of diabetes insipidus occurring after selective embolization of 50% dextrose and pure ethanol into an enlarged left meningohypophyseal trunk (MHT) supplying a dural carotid cavernous fistula. The inferior hypophyseal artery was not opacified during the selective preembolization MHT injection; however, diabetes insipidus developed abruptly a few hours after the procedure. The patient required intranasal 1-deamino-(8-D-arginine)-vasopressin for approximately 3 months, after which his symptoms resolved. The hazards of using liquid embolic agents, especially ethanol, in the cavernous branches of the internal carotid artery should always be borne in mind.

Treatment of an intracranial aneurysm using a new three-dimensional-shape Guglielmi detachable coil: technical case report.

OBJECTIVE AND IMPORTANCE: Coil embolization of wide-necked aneurysms is currently difficult, when using a conventional endovascular approach without resorting to complex adjunctive techniques. CLINICAL PRESENTATION: A 41-year-old woman with a history of systemic lupus erythematosus and hypertension refractory to treatment presented with an unruptured right ophthalmic segment aneurysm of the internal carotid artery having an unfavorable neck-to-fundus ratio. INTERVENTION: A new type of Guglielmi detachable coil (Target Therapeutics, Fremont, CA), consisting of a series of omega loops, spontaneously forms a three-dimensional cage after deployment and was used to successfully treat the aneurysm, which was angiographically stable at the 3-month follow-up. CONCLUSION: The new Guglielmi detachable coil may be useful in the embolization of aneurysms having an unfavorable geometry, which would otherwise not be amenable to endovascular treatment without adjunctive techniques.

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.

Tandem intracranial stent deployment for treatment of an iatrogenic, flow-limiting, basilar artery dissection: technical case report.

OBJECTIVE AND IMPORTANCE: Intimal dissection constitutes one of the complications associated with angioplasty of intracranial vessels. We present a case of iatrogenic dissection of the entire basilar artery, which was induced by angioplasty and stenting of symptomatic, focal, intracranial vertebral artery stenosis, and its successful treatment with tandem deployment of a downstream stent. CLINICAL PRESENTATION: A 61-year-old, hypertensive, renal transplant recipient presented with orthostatic vertebrobasilar insufficiency that was refractory to medical management, including anticoagulation therapy. Angiography revealed an occluded right vertebral artery and focal, high-grade, left intracranial vertebral artery stenosis. Magnetic resonance imaging showed multiple posterior fossa infarctions. The left intracranial vertebral artery stenosis was successfully treated with primary stent deployment and balloon angioplasty, with symptom resolution. On postprocedure Day 2, the patient noted worsening right hemiparesis. INTERVENTION: Subsequent angiography revealed a flow-limiting, windsock-type, basilar artery dissection beginning at the distal end of the left vertebral artery stent and extending to the origin of the left posterior cerebral artery. A tandem stent was navigated intracranially and deployed past the first one, successfully sealing the dissection inflow zone and reconstituting normal flow to the top of the basilar artery. A clinical follow-up examination at 3 months revealed no further orthostatic symptoms and only mild residual right-sided weakness. CONCLUSION: This is the first description of iatrogenic stent-induced dissection of the entire basilar artery that was successfully treated by inflow zone control via tandem intracranial stent deployment.