A benign course of Parkinson's disease associated with different mutations in the glucocerebrosidase gene - A case series.

BACKGROUND: Subjects carrying mutations in the GBA gene, whether in one or two alleles pose a risk to develop Parkinson's disease (PD). This type of GBA-related PD has usually a severe course, and patients tend to develop cognitive deficits faster. Herein we describe 8 cases of GBA-PD patients with a markedly benign course (bGBA-PD), and compared them to the other regular GBA-PD group (rGBA-PD) and to mutation negative patients (MNP). METHODS: All patients who performed next generation testing were enrolled. We defined bGBA-PD as patients with disease duration of at least 5 years, with Hoehn and Yahr (HY) ≤3 and MoCA score of ≥24. Following a selection of patients with a disease duration of 3 years or longer up to 15 years or shorter, we compared the bGBA-PD group to the rGBA-PD and MNP in terms of demographic and clinical features. RESULTS: 166 patients (58 males) diagnosed with PD were genotyped. Twenty-five patients were GBA-PD, of whom 7 were defined as bGBA-PD. Seventeen patients were LRRK2-PD and 123 patients were MNP. The rate of Rapide Eye Movement Behavioral Disorder (RBD) was highest in the rGBA-PD group and lowest in the bGBA-PD group (100 % vs. 28.6 %) (p < 0.001). The bGBA-PD group scored significantly higher in the Montreal Cognitive Assessment (MoCA) than rGBA-PD and MNP (27.6 ± 2.0 vs. 19.8 ± 3.9 vs. 23.5 ± 3.7, respectively; p = 0.006). There were no significant differences in MDS-UPDRS part-3 among the groups. There was no specific GBA mutation which was more commonly associated with bGBA-PD. CONCLUSION: While GBA-PD has a severe course of disease, a subgroup of which shows a benign course with a relatively preserved cognitive function even years after onset. We might suggest that these cases have other pathomechanism leading to PD, including an incidental GBA carriership.

Adult Attention Deficit-Hyperactivity Disorder is associated with Lewy Body Disease and Cognitive Impairment: A prospective cohort Study With 15-year Follow-Up.

OBJECTIVES: Past reports have suggested that attention-deficit/hyperactivity disorder (ADHD) may be a risk factor for Lewy body disease (LBD). To confirm this relationship, we conducted the present study. DESIGN: A prospective observational cohort study with a follow-up to 15 years. SETTING: The subjects were recruited from cognitive neurology clinics, where they attended for a cognitive complaint or health check-up. PARTICIPANTS: Two groups of subjects: ADHD adults and healthy subjects. MEASUREMENTS: The risk of dementia and LBD was estimated with Kaplan-Meier analysis comparing for the presence or absence of ADHD with the log-rank test. Predictors of conversion were assessed through separate univariate and multivariate Cox regression analyses, adjusting for several variables. RESULTS: The baseline sample consisted of 161 subjects with ADHD and 109 without ADHD. At the end of the follow-up, 31 subjects developed dementia, 27 cases in the ADHD group and 4 in comparison group. Dementia with Lewy bodies (DLB) was the most frequent type (N:20) of which 19 corresponded to the ADHD group. The incidence of non-amnestic-MCI in the ADHD group was higher representing 67.1 % of these subjects (N:108), and 17.4% (N:19) of healthy cases. The hazard ratios for dementia and LBD in the multivariate adjusted model were 3.33 (95% CI 1.0915 to 10.1699) and 54.54 (95% CI 7.4849 to 397.5028), respectively in the ADHD group. CONCLUSIONS: This study showed that adult ADHD is independently associated with an increased risk of LBD, dementia, and na-MCI. Future studies should clarify this relationship to develop preventive measures for these patients.

A Lower Rate of Hyposmia in Non-Ashkenazi Jewish Patients with Parkinson's Disease: Motor and Non-motor Disease Characteristics in Different Ethnic Groups in Israel.

BACKGROUND: Little is known about phenotypical variations among ethnic groups in patients with Parkinson's disease (PD) in Israel. Clinical characteristics of non-Ashkenazi Jews (NAJ) are scantly described. OBJECTIVES: To describe clinical aspects of PD in ethnic groups in Israel, focusing on NAJ and Ashkenazi Jews (AJ). METHODS: In this cross-sectional retrospective study, we collected demographic, genetic, and clinical characteristics of patients from different ethnic Jewish backgrounds. Ethnic groups included AJ; North African Jews (NAFJ); oriental Jews (OJ) originating from Iran, Iraq, and Buchara; Balkan Jews; Yemenite Jews (YJ); and Jews of mixed origin. Clinical characteristics included hyposmia, urinary complaints, constipation, and rapid eye movement sleep behavioral disorder. Cognitive complaints, motor features, levodopa-induced dyskinesia, and motor fluctuations were collected. Motor part of the MDS-UPDRS and Hoehn and Yahr scores were collected. RESULTS: The study comprised 174 PD Jewish patients (63.2% AJ, 56.4% males). The age at onset was 65.3 ± 10.2 years; 106 patients (60.9%) were genotyped (17 glucocerebrosidase [16.0%], 13 leucine-rich repeat kinase 2 [LRRK2] [12.3%]). Rates of hyposmia were significantly higher in AJ than NAJ (56.6% vs. 39.5%, respectively, P = 0.003). No significant differences were found in motor features in all variables. Of 13 AJ patients carrying the LRRK2 mutation, only one had hyposmia. Three patients with LRRK2 were NAJ. CONCLUSIONS: Hyposmia is less prevalent in PD patients of NAJ origin than in AJ. The rate of hyposmia in NAFJ patients is particularly low. The rate of other non-motor features is similar between NAJ and AJ patients.

The Downside of Botulinum Injections for Anterocollis: A Case Series and a Review of the Literature.

OBJECTIVES: This is a case series and a review of the literature of therapeutic outcomes of botulinum toxin (BT) injections for anterocollis. METHODS: Data collected included gender, age, age at onset, muscles targeted, and doses injected. Routine forms were filled out during each visit: Patient Global Impression of Change, Clinician Global Impression of Severity, Tsui scale. The effect duration and side effects (SEs) of the previous treatment were noted. RESULTS: We described 4 patients (3 men, 13 visits) with anterocollis, as primary postural abnormality of the neck, emphasizing the therapeutic response to BT injection. Mean age at onset was 75.3 ± 7.0 years, age at first injection was 80.7 ± 3.5 years. The mean total dose per treatment was 290.0 ± 95.6 units. Patient Global Impression of Change with any grade of favorable effect was reported in 27.3% of the treatments. In objective assessment, Global Impression of Severity and Tsui scores did not show a consistent tendency of improvement. Neck weakness was prevalent in 18.2% of the visits of the anterocollis group while no other SEs were noted. We found 15 articles describing experience with BT for anterocollis in 67 patients (19 in deep and 48 in superficial neck muscles). CONCLUSIONS: This case series describes the poor outcome of BT treatment for anterocollis, with low efficacy and bothersome SE. Levator scapulae injection for anterocollis is not effective and is highly associated with head drop and should perhaps be abandoned. Injection to the longus colli might give some benefit in non-responders.

Therapeutic Approach to Botulinum Injections for Hemifacial Spasm, Synkinesis and Blepharospasm.

The aim of this study was to show our therapeutic outcome of botulinum injection to the facial muscles and thereby to find the best therapeutic concept which should be embraced. The decision to treat the lower eyelid with 1-point or 2-points injection was randomly taken as there is no consensus regarding this debate. Injections of the lateral end of the upper eyelid were performed more laterally to the conventional injection point, just lateral to the conjunction of the upper and lower eyelids. Twenty-three patients (12 hemifacial spasm, 6 blepharospasm, 5 post facial palsy synkinesis) were enrolled. Data were retrieved from 112 visits between 2019 and 2022. Overall, 84.9% of the treatments had moderate or marked improvement. The most common side effect was facial weakness (11.8%). Neither ptosis nor diplopia were noted. Two-points regimen in the lower eyelid was associated with a lower risk of facial weakness (p = 0.01), compared to 1-point regimen, with a better therapeutic outcome as reflected by more favorable PGI-C scores (p = 0.04). Injection of the pretarsal segment of the upper eyelid, just onto or even lateral to the conjunction of the upper and lower eyelids, lowers the risk of ptosis.

A multiple-method comparative study using GC-MS, AMDIS and in-house-built software for the detection and identification of "unknown" volatile organic compounds in breath.

The human respiratory system is a highly complex matrix that exhales many volatile organic compounds (VOCs). Breath-exhaled VOCs are often "unknowns" and possess low concentrations, which make their analysis, peak digging and data processing challenging. We report a new methodology, applied in a proof-of-concept experiment, for the detection of VOCs in breath. For this purpose, we developed and compared four complementary analysis methods based on solid-phase microextraction and thermal desorption (TD) tubes with two GC-mass spectrometer (MS) methods. Using eight model compounds, we obtained an LOD range of 0.02-20 ng/ml. We found that in breath analysis, sampling the exhausted air from Tedlar bags is better when TD tubes are used, not only because of the preconcentration but also due to the stability of analytes in the TD tubes. Data processing (peak picking) was based on two data retrieval approaches with an in-house script written for comparison and differentiation between two populations: sick and healthy. We found it best to use "raw" AMDIS deconvolution data (.ELU) rather than its NIST (.FIN) identification data for comparison between samples. A successful demonstration of this method was conducted in a pilot study (n = 21) that took place in a closed hospital ward (Covid-19 ward) with the discovery of four potential markers. These preliminary findings, at the molecular level, demonstrate the capabilities of our method and can be applied in larger and more comprehensive experiments in the omics world.

Superficial Temporal Artery to Middle Cerebral Artery Cranial Bypass for Nonmoyamoya Steno-Occlusive Disease in Patients Who Failed Optimal Medical Treatment: A Case Series.

BACKGROUND: In the post-Carotid Occlusion Surgery Study (COSS) era, multiple reviews suggested subset groups of patients as potential candidates for superficial temporal artery to middle cerebral artery (STA-MCA) bypass. Among them are patients with recurrent strokes despite optimal medical therapy. There is a paucity of data on the outcome of bypass in these specific patients. OBJECTIVE: To examine the safety and efficacy of direct STA-MCA bypass in patients with nonmoyamoya, symptomatic steno-occlusive disease with impaired distal perfusion, who failed optimal medical management or endovascular treatment. METHODS: A retrospective review was performed to identify patients with cerebrovascular steno-occlusive disease who underwent bypass after symptomatic recurrent or rapidly progressive strokes, despite optimal conservative or endovascular treatment. RESULTS: A total of 8 patients (mean age 60 ± 6 yr) underwent direct or combined direct/indirect STA-MCA bypass between 2016 and 2019. All anastomoses were patent. One bypass carried slow flow. There were no procedure-related permanent deficits. One patient developed seizures which were controlled by medications. A total of 7 out of 8 patients were stable or improved clinically at last follow-up (mean 27.3 ± 13.8 mo) without recurrent strokes. One patient did not recover from their presenting stroke, experienced severe bilateral strokes 4 mo postoperatively, and subsequently expired. Modified Rankin Scale (mRS) improved in 6 patients (75%), remained stable in 1 patient (12.5%), and deteriorated in 1 (12.5%). Good long-term functional outcome was achieved in 5 patients (63%, mRS ≤ 2). CONCLUSION: Patients with symptomatic, hypoperfused steno-occlusive disease who fail optimal medical or endovascular treatment may benefit from cerebral revascularization. Direct or combined STA-MCA bypass was safe and provided favorable outcomes in this small series.

Acute bithalamic infarct manifesting as sleep-like coma: A diagnostic challenge.

Bilateral thalamic infarction (BTI) typically presents as a sleep-like coma (SLC) without localizing signs, posing a diagnostic challenge that may lead the treating physician to search for toxic or metabolic causes and delay treatment. We review our experience with BTI of different etiologies, and emphasize the critical role of timely imaging, diagnosis, and management in a series of 12 patients with a presentation of SLC and acute BTI who were managed in our Medical Centers from 2006-2015. In 11/12, urgent head CT scans showed normal brain tissue, while diffusion-weighted (DWI) MRI revealed symmetric bilateral thalamic hyperintense lesions with variable degrees of brainstem involvement. In 1/12, CT scans revealed a contralateral subacute stroke from a thalamic infarct 1month earlier with a unilateral hyperintense lesion on DWI-MRI. From clinical and imaging findings (DWI-MRI, CT angiography and venography), etiology was attributed to embolic causes (cardio-embolism, artery-to-artery mechanism), small vessel disease, or deep sinus vein thrombosis secondary to dural arteriovenous (AV) fistula. Three patients had good outcomes after prompt diagnosis and optimal treatment in <3hours (intravenous tissue plasminogen activator in two patients cardio-embolic etiology and neuro-endovascular repair in one patient with venous infarction due to a dural AV fistula). The diagnosis was made beyond the therapeutic window in seven patients, who were left with significant neurological sequelae. Higher awareness of BTI presenting as SLC is warranted. Optimal patient management includes urgent DWI-MRI. In cases of BTI, further imaging workup is indicated to provide a comprehensive assessment for etiology. Early diagnosis and prompt, targeted intervention are crucial.

Emergent revascularization of acute tandem vertebrobasilar occlusions: Endovascular approaches and technical considerations-Confirming the role of vertebral artery ostium stenosis as a cause of vertebrobasilar stroke.

Patients suffering from acute atherothrombotic occlusion of the proximal vertebral artery (VA) and concomitant basilar artery (BA) occlusion present a grim prognosis. We describe our experience in the endovascular recanalization of tandem vertebrobasilar occlusions using endovascular techniques. The BA was accessed through the normal VA (clean-road) or the occluded, thrombotic VA (dirty-road), and stentriever-based thrombectomy was performed using antegrade or reverse revascularization variants. Seven patients underwent successful stentriever-assisted mechanical thrombectomy of the BA and five sustained concomitant VA revascularization. Stroke onset to endovascular intervention initiation (time-to-treatment) ranged from 4.5-13hours (mean 8.6). In two of seven patients, the BA occlusion was approached with a 'clean-road' approach via the contralateral VA; in five of seven patients, a 'dirty-road' approach via the occluded VA was used. Mean time-to-recanalization was 66minutes (range 55-82). There were no perforations, iatrogenic vessel dissections, or other technical complications. Four patients presented mild-to-moderate disability (modified Rankin Scale [mRS] 0-3) at 3months, one remained with moderate-to-severe disability (mRS 4), and two patients died on days 9 and 23 after their strokes. Follow-up ranged from 6-45months (mean 24months). In selected patients with acute VA-BA occlusion, stentriever-based thrombectomy performed through either the patent or the occluded VA, may be feasible, effective, and safe. Clinical outcomes in these patients seem to equipoise the neurological outcome of patients with successful revascularization for isolated BA occlusion. This unique pair of occlusions confirms the role of VA ostium stenosis as a cause of vertebrobasilar stroke.

Emergency endovascular revascularization of tandem occlusions: Internal carotid artery dissection and intracranial large artery embolism.

Internal carotid artery dissection (ICAD) with concomitant occlusive intracranial large artery emboli is an infrequent cause of acute stroke, with poor response to intravenous thrombolysis. Reports on the management of this entity are limited. We present our recent experience in the endovascular management of occlusive ICAD and major intracranial occlusion. Consecutive anterior circulation acute stroke patients meeting Medical Center criteria for endovascular management of ICAD from June 2011 to June 2015 were included. Clinical, imaging, and procedure data were collected retrospectively under Institutional Review Board approval. The endovascular procedure for carotid artery revascularization and intracranial stent thrombectomy is described. Six patients met inclusion criteria (National Institutes of Health Stroke Scale score 12-24, time from symptom onset 2-8hours). Revascularization of the extracranial carotid dissection and stent thrombectomy were achieved in 5/6 patients, resulting in complete recanalization (Thrombolysis in Myocardial Infarction flow grade 3 in a mean 2.7hours), and modified Rankin Scale score 0-2 at 90 day follow-up. In one patient, attempts to microcatheterize the true arterial lumen failed and thrombectomy was therefore not feasible. No arterial dissection, arterial rupture or accidental stent detachment occurred, and there was no intracerebral hemorrhage or hemorrhagic transformation. Our preliminary data on this selected subgroup of patients suggest the presented approach is safe, feasible in a significant proportion of patients, and efficacious in achieving arterial recanalization and improving patient outcome. Crossing the dissected segment remains the most important limiting factor in achieving successful ICA recanalization. Further evaluation in larger series is warranted.

Emergency revascularization of acute internal carotid artery occlusion: Follow the spike, it guides you.

The present study sought to examine the incidence of the angiographic "spike sign" and to assess its predictive significance for achieving carotid revascularization in 54 patients with acute internal carotid artery (ICA) occlusions that required urgent endovascular revascularization. Clinical and imaging files of consecutive patients with ICA occlusion who were treated in a tertiary care academic medical center from 2011-2015 were retrospectively examined under Institutional Review Board approval with a waiver of the requirement for informed consent. All proximal ICA occlusions were treated by stent-assisted carotid angioplasty, and all distal embolic occlusions were managed with stent-assisted mechanical thrombectomy. The study included 24 patients with acute ICA occlusion (group 1) and 30 patients with tandem ICA-intracranial occlusions (group 2). The spike sign was seen in 16/24 patients in group 1 (67%), and successful ICA revascularization was achieved in 14/16 (88%). The sign was seen in 26/30 patients in group 2 (87%), and ICA revascularization was successful in all 26 (100%). The remaining 12 patients had no spike sign, and ICA revascularization was successful in only 7/12 (58%). The spike sign is a transient finding that represents the proximal patent remnant of the stenotic corridor in fresh clot. Acute ICA occlusion frequently leaves the spike sign as a marker of the recent thrombotic event. The spike vertex points to the "path of least resistance" for the guidewire to cross the occlusion and engage the true arterial lumen, a critical step during ICA endovascular revascularization.

Sandfly virus seroconversion associated with neurologic presentation.

OBJECTIVE: To describe the clinical presentation and unique neurologic manifestations of sandfly viruses (SFVs) in the Jerusalem area. METHODS: We identified all patients with acute seroconversion to SFV at the Hadassah-Hebrew University Medical Centers during the years 2008-2013 and retrospectively collected and analyzed the clinical and imaging data. RESULTS: Nine patients (ranging from 1.5 to 85 years old) were identified. Presentation included acute neurologic disease, mostly with fever, change in consciousness and behavior, seizures, headache, meningitis, limb paresis, or myelitis. Eight patients had clinical signs of meningitis, meningoencephalitis, or encephalitis alone. Four patients had myelitis. MRI identified pathologic symmetrical changes in the basal ganglia, thalami, and other deep structures in 5 patients, and additional myelitis of the spine was noted on imaging in 3 patients. Seven patients had long-term follow-up: 4 completely recovered and 3 had remaining neurologic sequelae, among them 1 with permanent severe brain damage. CONCLUSION: Neurologic involvement associated with acute SFV infections is considered to be benign. However, in this series, all 9 patients presented with significant neurologic pathology associated with a unique finding of myelitis and symmetrical basal ganglia, thalami, or white matter involvement. Thus, acute SFV infection should be included in the differential diagnosis in febrile onset of neurologic manifestations and neuroradiologic changes.

Asystole in the epilepsy unit.

BACKGROUND: Early identification of cardiac asystole as a reason for syncope is of uttermost significance, as insertion of a cardiac pacemaker can save the patient's life and prevent severe injury. The aim of this work was to emphasize the subtle and unusual presentations of asystole in patients evaluated in epilepsy units. METHODS: We reviewed the clinical presentation, ECG and EEG data of a series of seven patients who were evaluated in four epilepsy units and were diagnosed with asystole. RESULTS: Three patients had unusual clinical manifestations of cardiac asystole, resembling epileptic seizures. Three patients had asystole induced by epileptic seizures and in one patient the diagnosis was not clear. All patients except one were implanted with a pacemaker and improved clinically. CONCLUSIONS: Seizure-induced asystole is a rare complication of epilepsy and asystole may clinically mimic epileptic seizures. A high level of suspicion and thorough prolonged cardiac and EEG monitoring are mandatory for reaching the right diagnosis. As the diagnosis is rare and difficult to reach, a flow chart to assist diagnosis is suggested.

Ischemic complications after tailored carotid artery stenting in different subpopulations with high-grade stenosis: feared but rare.

Although the procedural and postoperative safety profile of carotid artery stenting (CAS) has been steadily improving, many centers still recommend carotid endarectomy (CEA) over CAS. We assessed outcomes (procedural and postoperative stroke) following tailored CAS in a cohort of patients managed at a single academic medical center. Outcomes for patients with carotid artery stenosis treated from 2005-2013 with CAS were retrospectively reviewed. Stenosis was assessed with Doppler ultrasonography and/or CT angiogram, and angiography. Symptomatic and asymptomatic patients were dichotomized (based on the North American Symptomatic Carotid Endarterectomy Trial [NASCET] and the Asymptomatic Carotid Atherosclerosis Study [ACAS]). CAS technique was chosen based on angiographic and clinical characteristics; procedures were performed with/without pre-angioplasty, cerebral protection, and post-dilation. Endpoints were cumulative incidence of ipsilateral stroke, myocardial infarction, and death within 30 days (primary) or 12 months (secondary). Overall 249 patients (151 men/98 women; mean age 69.9 years) with 254 carotid stenoses were included; 148 lesions (58%) were asymptomatic, and 106 (42%) were symptomatic. CAS was successfully performed in all lesions. At 30 days, ipsilateral transient ischemic attack (TIA)/minor stroke was seen in 6/104 (5.8%) symptomatic patients and no asymptomatic patients; there was no myocardial infarction or ipsilateral major stroke. At 12 months, there was ipsilateral TIA/minor stroke in an additional 3/98 (3.1%) symptomatic and 1/127 (0.8%) asymptomatic patients, and major stroke in 1/98 (1%). The incidence of stroke after CAS compares favorably with rates reported after CEA. The majority of peri-procedural ischemic events following CAS are TIA/minor strokes causing only transient or minor functional impact; major disabling stroke is rare with current techniques.

Occlusion site does not impact outcome in patients with carotid stroke undergoing endovascular reperfusion.

BACKGROUND: It remains unclear whether occlusion site impacts outcome in patients with acute carotid artery occlusions. METHODS: Patients with acute carotid artery occlusion that underwent endovascular reperfusion treatments were prospectively enrolled. Patients with extracranial carotid bifurcation occlusions were compared with those with intracranial carotid-T-occlusions. Collected data included demographics, risk factor profile, and procedure-related variables. Neurological deficits were studied with the National Institutes of Health Stroke Scale and outcome was studied with the modified Rankin Score at day 90 after stroke and dichotomized into favorable (≤2) or unfavorable (>3). Recanalization status was studied with the thrombolysis in cerebral infarction scale. RESULTS: We included 51 patients (33 with extracranial bifurcation occlusion and 18 with intracranial T-occlusion). Patients with T lesions were significantly older (median 74 versus 56 years, P = 0.02), more frequently had atrial fibrillation (61% versus 18%; P = 0.005) and cardioembolism (78% versus 21% P = 0.001), smoked less often (6% versus 42%; P = 0.01), and less often required stent implantation (11% versus 48%; P = 0.015). However, neurological severity, other procedure and peri-procedure-related variables including recanalization rates and percentages of symptomatic hemorrhages did not differ between the groups. Mortality rates (24% versus 23%) and chances for favorable outcomes (33% versus 24%) did not significantly differ. On multivariate logistic regression analysis, occlusion location was not a significant modifier of outcome. CONCLUSIONS: Despite differences in stroke risk factors and treatments used between patients with extracranial bifurcation and intracranial T-occlusions, lesion location in itself does not influence outcome in patients with acute carotid artery occlusion treated with endovascular reperfusion.

Extracranial carotid artery stenting followed by intracranial stent-based thrombectomy for acute tandem occlusive disease.

OBJECTIVE: Acute tandem occlusions of the extracranial internal carotid artery (ICA) and a major intracranial artery respond poorly to intravenous tissue plasminogen activator (tPA) and present an endovascular challenge. We describe our experience with emergency stent-assisted ICA angioplasty and intracranial stent-based thrombectomy of tandem occlusions. METHODS: Procedures were performed from March 2010 to December 2013. National Institutes of Health Stroke Score (NIHSS) and Alberta Stroke Program Early CT Score (ASPECTS), occlusion sites, collateral supply, procedural details, and outcomes were retrospectively reviewed with IRB waiver of informed consent. RESULTS: 24 patients, mean age 66 years, mean admission NIHSS 20.4, and mean ASPECTS 9 were included. Occlusion sites were proximal ICA-middle cerebral artery (MCA) trunk in 17 patients, proximal ICA-ICA terminus in six, and ICA-MCA-anterior cerebral artery in one. Stent-assisted cervical ICA recanalization was achieved in all patients, with unprotected pre-angioplasty in 24/24, unprotected stenting in 16/24 (67%), and protected stenting in 8/24 (33%), followed by stent-thrombectomy in 25 intracranial occlusions. There was complete recanalization/complete perfusion in 19/24 (79%), complete recanalization/partial perfusion in 3/24 (13%), and partial recanalization/partial perfusion in 2/24 (8%) with no procedural morbidity/mortality. Mean time to therapy was 3.8 h (range 2-5.5) and mean time to recanalization was 51 min (range 38-69). At 3-month follow-up, among 17/22 surviving patients (77%), 13/17 (76%) were modified Rankin Scale (mRS) 0-2 and 3/17 (18%) were mRS 3. CONCLUSIONS: In acute tandem ICA-MCA/distal ICA occlusions, extracranial stenting followed by intracranial stent-based thrombectomy appears feasible, effective, and safe. Further evaluation of this treatment strategy is warranted.

Urgent off-label use of the pipeline flow diverter stent in selected ischemic cerebrovascular conditions: thrombotic segments and tortuous arteries.

OBJECTIVE: Flow diverter stents were originally designed for the endovascular management of certain types of cerebral aneurysms; however, these devices present characteristics that make them more suitable that regular carotid stents or neurostents for the management of selected ischemic cerebrovascular conditions. METHODS: Eight patients with steno-occlusive disease of the internal carotid (ICA) or vertebral (VA) arteries underwent endovascular reconstruction by means of flow diverter stent implant at our center. Five patients presented with ICA steno-occlusive lesions that involved tortuous segments not amenable to regular carotid stent placement and three patients presented with severe and complex proximal VA dissections. RESULTS: In all cases the procedures were considered technically successful. Flow diverter stent implant allowed recanalization of the treated vessels (stenosis of 89±10.5% was improved to 26±13%) without procedure related complications. At the 3 month clinical and radiological follow-up, patients either improved or remained stable, and showed stent patency. One patient presented with asymptomatic occlusion of the revascularized artery at 13 months, emphasizing the need for prolonged antiplatelet therapy. CONCLUSIONS: This preliminary series of patients with high risk steno-occlusive lesions affecting tortuous arterial segments or presenting with heavy thrombotic load managed by the implant of flow diverter stents shows that this approach is feasible, safe, and effective in achieving arterial recanalization. Further studies will elucidate the role of this technique in ischemic cerebrovascular settings.

Endovascular thrombectomy and stenting in the management of carotid fibromuscular dysplasia presenting with major ischemic stroke.

Fibromuscular dysplasia (FMD) affecting the internal carotid artery (ICA) is considered a benign condition; however, retinal and cerebral ischemic events may occur. We present a patient with carotid FMD presenting with a major ischemic stroke due to major embolic occlusion of the ICA in conjunction with a hemodynamic component caused by narrowing and beading of the lumen associated with FMD. The patient was successfully treated with intracranial stent-assisted thrombectomy followed by cervical ICA stenting that aimed to reconstruct and angioplasty the FMD-affected arterial segment. Recently, stent-based thrombectomy has emerged as the most effective endovascular option for the rapid revascularization of major intracranial occlusions; however, to our knowledge, its use in a rare case of FMD-associated major stroke has not yet been reported.