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.

Safety of the pipeline embolization device in treatment of posterior circulation aneurysms.

BACKGROUND AND PURPOSE: The published results of treating internal carotid artery aneurysms with the PED do not necessarily apply to its use in the posterior circulation because disabling brain stem infarcts can be caused by occlusion of a single perforator. In this multicenter study, we assessed the safety of PED placement in the posterior circulation. MATERIALS AND METHODS: A prospective case registry was maintained of all posterior circulation aneurysms treated with PEDs at 3 Australian neurointerventional centers during a 27-month period. The objective was to assess the complications and aneurysm occlusion rates associated with posterior circulation PEDs. RESULTS: Thirty-two posterior circulation aneurysms were treated in 32 patients. No deaths or poor neurologic outcomes occurred. Perforator territory infarctions occurred in 3 (14%) of the 21 patients with basilar artery aneurysms, and in all 3, a single PED was used. Two asymptomatic intracranial hematomas were recorded. No aneurysm rupture or PED thrombosis was encountered. The overall rate of permanent neurologic complications was 9.4% (3/32); all 3 patients had very mild residual symptoms and a good clinical outcome. Aneurysm occlusion was demonstrated in 85% of patients with >6 months of follow-up and 96% of patients with >1 year of follow-up. CONCLUSIONS: The PED is effective in the treatment of posterior circulation aneurysms that are otherwise difficult or impossible to treat with standard endovascular or surgical techniques, and its safety is similar to that of stent-assisted coiling techniques. A higher clinical perforator infarction rate may be associated with basilar artery PEDs relative to the internal carotid artery.

Immediate and midterm results following treatment of unruptured intracranial aneurysms with the pipeline embolization device.

BACKGROUND AND PURPOSE: A number of flow-diverting devices have become available for endovascular occlusion of cerebral aneurysms. This article reports immediate and midterm results in treating unruptured aneurysms with the PED. MATERIALS AND METHODS: A prospective registry was established at 3 Australian neurointerventional units. Aneurysms were treated on the basis of unfavorable anatomy or recurrence following previous treatment. Aneurysms were treated with PED or PED and coils. Data including antiplatelet therapy, technical issues, complications, and imaging findings were recorded during at least a 6-month period. RESULTS: A total of 57 aneurysms in 54 patients were treated by 5 neurointerventional radiologists. Forty-one aneurysms were asymptomatic, and 16 patients had mass-induced neurological deficit. Clinical follow-up was available in 57 aneurysms with imaging follow-up at 6 months in 56. Permanent morbidity and mortality in the series was 0% at 6 months. Four TIAs and 1 small retinal branch occlusion occurred, but no stroke. The demonstrated aneurysm occlusion rate at 1 month was 61.9%, and the overall occlusion rate at 6 months was 85.7%. In cases previously untreated, the 6-month occlusion was 92.5%. Three of 6 aneurysms with a previous stent in situ were occluded. Two patients (3.5%) had asymptomatic in-construct stenosis of >50%. Acute aneurysm-provoked mass effect resolved or improved significantly in all cases. CONCLUSIONS: Use of the PED is safe and efficacious in difficult aneurysms with a high occlusion rate at 6 months, but lower occlusion rates were seen in a small population with previous stents in situ.

Endovascular treatment using predominantly stent-assisted coil embolization and antiplatelet and anticoagulation management of ruptured blood blister-like aneurysms.

BACKGROUND AND PURPOSE: BBA is a rare type of intracranial aneurysm that is difficult to treat both surgically and endovascularly and is often associated with a high degree of morbidity/mortality. The aim of this study was to present clinical and angiographic results, as well as antiplatelet/anticoagulation regimens, of endovascular BBA treatment by using predominantly stent-assisted coil embolization. MATERIALS AND METHODS: Thirteen patients (men/women, 6/7; mean age, 49.3 years) with ruptured BBAs were included from 2 different institutions. Angiographic findings, treatment strategies, anticoagulation/antiplatelet protocols, and clinical (mRS) and angiographic outcome were retrospectively analyzed. RESULTS: Eleven BBAs were located in the supraclinoid ICA, and 2 on the basilar artery trunk. Nine of 13 were ≤3 mm in the largest diameter, and 8/13 showed early growth before treatment. Primary stent-assisted coiling was performed in 11/13 patients, double stents and PAO in 1 patient, each. Early complementary treatment was required in 3 patients, including PAO in 2. In stent-placement procedures, altered periprocedural antiplatelet (11/12) and postprocedural heparin (6/12) protocols were used without evidence of thromboembolic events. Two patients had early rehemorrhage, including 1 major fatal SAH. Twelve of 13 BBAs showed complete or progressive occlusion at late angiographic follow-up. Clinical midterm outcome was good (mRS scores, 0-2) in 12/13 patients. CONCLUSIONS: Stent-assisted coiling of ruptured BBAs is technically challenging but can be done with good midterm results. Reduced periprocedural and postprocedural antiplatelet/anticoagulation protocols may be used with a low reasonable risk of thromboembolic complications. However, regrowth/rerupture remains a problem underlining the importance of early angiographic follow-up and re-treatment, including PAO if necessary.

Cerebral hyperperfusion syndrome post-carotid artery stenting.

Cerebral hyperperfusion syndrome is increasingly recognized as a complication in carotid artery stenting for severe internal carotid artery stenosis. This study reviews the cases of hyperperfusion syndrome occurring after this procedure. We reviewed our database of 170 cases of internal carotid artery stenting carried out at our hospital between January 1999 and June 2006. A radiology search was also carried out to identify those who had CT or MRI within 1 month of post-carotid artery stenting. We had four patients who developed cerebral hyperperfusion syndrome. One patient developed cerebral oedema, one patient had petechial intracerebral haemorrhage and two patients had large intracerebral haemorrhages, one of whom died. This gives a risk of 2.3% (95% confidence interval 2.27-2.323). All patients with cerebral haemorrhage presented within 6 h. Both patients with large intracerebral haemorrhage had carotid stenting within 3 weeks after presentation of symptoms and all had critically severe stenosis of 95% or more. In our series, large intracerebral haemorrhage has occurred only in patients who have been treated early. Cerebral hyperperfusion is an uncommon but serious complication post-carotid stenting. Further studies comparing early treatment of endarterectomy and carotid stenting are awaited.

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.

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.

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.

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.