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OBJECTIVE There is a reported elevated risk of cerebral aneurysms in patients with intracranial dural arteriovenous fistulas (dAVFs). However, the natural history, rate of spontaneous regression, and ideal treatment regimen are not well characterized. In this study, the authors aimed to describe the characteristics of patients with dAVFs and intracranial aneurysms and propose a classification system. METHODS The Consortium for Dural Arteriovenous Fistula Outcomes Research (CONDOR) database from 12 centers was retrospectively reviewed. Analysis was performed to compare dAVF patients with (dAVF+ cohort) and without (dAVF-only cohort) concomitant aneurysm. Aneurysms were categorized based on location as a dAVF flow-related aneurysm (FRA) or a dAVF non–flow-related aneurysm (NFRA), with further classification as extra- or intradural. Patients with traumatic pseudoaneurysms or aneurysms with associated arteriovenous malformations were excluded from the analysis. Patient demographics, dAVF anatomical information, aneurysm information, and follow-up data were collected. RESULTS Of the 1077 patients, 1043 were eligible for inclusion, comprising 978 (93.8%) and 65 (6.2%) in the dAVF-only and dAVF+ cohorts, respectively. There were 96 aneurysms in the dAVF+ cohort; 10 patients (1%) harbored 12 FRAs, and 55 patients (5.3%) harbored 84 NFRAs. Dural AVF+ patients had higher rates of smoking (59.3% vs 35.2%, p < 0.001) and illicit drug use (5.8% vs 1.5%, p = 0.02). Sixteen dAVF+ patients (24.6%) presented with aneurysm rupture, which represented 16.7% of the total aneurysms. One patient (1.5%) had aneurysm rupture during follow-up. Patients with dAVF+ were more likely to have a dAVF located in nonconventional locations, less likely to have arterial supply to the dAVF from external carotid artery branches, and more likely to have supply from pial branches. Rates of cortical venous drainage and Borden type distributions were comparable between cohorts. A minority (12.5%) of aneurysms were FRAs. The majority of the aneurysms underwent treatment via either endovascular (36.5%) or microsurgical (15.6%) technique. A small proportion of aneurysms managed conservatively either with or without dAVF treatment spontaneously regressed (6.2%). CONCLUSIONS Patients with dAVF have a similar risk of harboring a concomitant intracranial aneurysm unrelated to the dAVF (5.3%) compared with the general population (approximately 2%–5%) and a rare risk (0.9%) of harboring an FRA. Only 50% of FRAs are intradural. Dural AVF+ patients have differences in dAVF angioarchitecture. A subset of dAVF+ patients harbor FRAs that may regress after dAVF treatment.

Purpose Radiologic follow-up of patients with a meningioma at the skull base or near the venous sinuses with magnetic resonance imaging (MRI) after stereotactic radiotherapy (SRT) and neurosurgical resection(s) can be difficult to interpret. This study evaluates the addition of 11C-methionine positron emission tomography (MET-PET) to the regular MRI follow-up. Methods This prospective pilot study included patients with predominantly WHO grade I meningiomas at the skull base or near large vascular structures. Previous SRT was part of their oncological treatment. A MET-PET in adjunct to their regular MRI follow-up was performed. The standardized uptake value (SUV) was determined for the tumor and the healthy brain, on the pre-SRT target delineation MET-PET and the follow-up MET-PET. Tumor-to-normal ratios were calculated, and 11C-methionine uptake over time was analyzed. Agreement between the combined MRI/MET-PET report and the MRI-only report was determined using Cohen’s κ. Results Twenty patients with stable disease underwent an additional MET-PET, with a median follow-up of 84 months after SRT. Post-SRT SUV T/N ratios ranged between 2.16 and 3.17. When comparing the pre-SRT and the post-SRT MET-PET, five categories of SUV T/N ratios did not change significantly. Only the SUVpeak T/Ncortex decreased significantly from 2.57 (SD 1.02) to 2.20 (SD 0.87) [p = 0.004]. A κ of 0.77 was found, when comparing the MRI/MET-PET report to the MRI-only report, indicating no major change in interpretation of follow-up data. Conclusion In this pilot study, 11C-methionine uptake remained remarkably high in meningiomas with long-term follow-up after SRT. Adding MET-PET to the regular MRI follow-up had no impact on the interpretation of follow-up imaging.

The clinical benefit of Deep Brain Stimulation (DBS) is associated with electrode positioning accuracy. Intraoperative assessment of clinical effect is therefore key. Evaluating this clinical effect in patients with dystonic head tremor, as opposed to limb tremor, is challenging because the head is fixed in a stereotactic frame. To clinically assess head tremor during surgery, surface electromyography (EMG) electrodes were bilaterally applied to the sternocleidomastoid and cervical paraspinal muscles. This case shows that intraoperative polymyography is an easy and useful tool to assess the clinical effect of DBS electrode positioning.

Background and purpose: The Unruptured Intracranial Aneurysm Treatment Score (UIATS) was built to harmonize the treatment decision making on unruptured intracranial aneurysms. Therefore, it may also function as a predictor of aneurysm progression. In this study, we aimed to assess the validity of the UIATS model to identify aneurysms at risk of growth or rupture during follow-up.Methods: We calculated the UIATS for a consecutive series of conservatively treated unruptured intracranial aneurysms, included in our prospectively kept neurovascular database. Computed tomography angiography and/or magnetic resonance angiography imaging at baseline and during follow-up was analyzed to detect aneurysm growth. We defined rupture as a cerebrospinal fluid or computed tomography-proven subarachnoid hemorrhage. We calculated the area under the receiver operator curve, sensitivity, and specificity, to determine the performance of the UIATS model.Results: We included 214 consecutive patients with 277 unruptured intracranial aneurysms. Aneurysms were followed for a median period of 1.3 years (range 0.3-11.7 years). During follow-up, 17 aneurysms enlarged (6.1%), and two aneurysms ruptured (0.7%). The UIATS model showed a sensitivity of 80% and a specificity of 44%. The area under the receiver operator curve was 0.62 (95% confidence interval 0.46-0.79).Conclusions: Our observational study involving consecutive patients with an unruptured intracranial aneurysm showed poor performance of the UIATS model to predict aneurysm growth or rupture during follow-up.

OBJECTIVE Cranial dural arteriovenous fistulas (dAVFs) are often treated with endovascular therapy, but occasionally a multimodality approach including surgery and/or radiosurgery is utilized. Recurrence after an initial angiographic cure has been reported, with estimated rates ranging from 2% to 14.3%, but few risk factors have been identified. The objective of this study was to identify risk factors associated with recurrence of dAVF after putative cure. METHODS The Consortium for Dural Arteriovenous Fistula Outcomes Research (CONDOR) data were retrospectively reviewed. All patients with angiographic cure after treatment and subsequent angiographic follow-up were included. The primary outcome was recurrence, with risk factor analysis. Secondary outcomes included clinical outcomes, morbidity, and mortality associated with recurrence. Risk factor analysis was performed comparing the group of patients who experienced recurrence with those with durable cure (regardless of multiple recurrences). Time-to-event analysis was performed using all collective recurrence events (multiple per patients in some cases). RESULTS Of the 1077 patients included in the primary CONDOR data set, 457 met inclusion criteria. A total of 32 patients (7%) experienced 34 events of recurrence at a mean of 368.7 days (median 192 days). The recurrence rate was 4.5% overall. Kaplan-Meier analysis predicted long-term recurrence rates approaching 11% at 3 years. Grade III dAVFs treated with endovascular therapy were statistically significantly more likely to experience recurrence than those treated surgically (13.3% vs 0%, p = 0.0001). Tentorial location, cortical venous drainage, and deep cerebral venous drainage were all risk factors for recurrence. Endovascular intervention and radiosurgery were associated with recurrence. Six recurrences were symptomatic, including 2 with hemorrhage, 3 with nonhemorrhagic neurological deficit, and 1 with progressive flow-related symptoms (decreased vision). CONCLUSIONS Recurrence of dAVFs after putative cure can occur after endovascular treatment. Risk factors include tentorial location, cortical venous drainage, and deep cerebral drainage. Multimodality therapy can be used to achieve cure after recurrence. A delayed long-term angiographic evaluation (at least 1 year from cure) may be warranted, especially in cases with risk factors for recurrence.

OBJECTIVE Cranial dural arteriovenous fistulas (dAVFs) are rare lesions, hampering efforts to understand them and improve their care. To address this challenge, investigators with an established record of dAVF investigation formed an international, multicenter consortium aimed at better elucidating dAVF pathophysiology, imaging characteristics, natural history, and patient outcomes. This report describes the design of the Consortium for Dural Arteriovenous Fistula Outcomes Research (CONDOR) and includes characterization of the 1077-patient cohort. METHODS Potential collaborators with established interest in the field were identified via systematic review of the literature. To ensure uniformity of data collection, a quality control process was instituted. Data were retrospectively obtained. RESULTS CONDOR comprises 14 centers in the United States, the United Kingdom, the Netherlands, and Japan that have pooled their data from 1077 dAVF patients seen between 1990 and 2017. The cohort includes 359 patients (33%) with Borden type I dAVFs, 175 (16%) with Borden type II fistulas, and 529 (49%) with Borden type III fistulas. Overall, 852 patients (79%) presented with fistula-related symptoms: 427 (40%) presented with nonaggressive symptoms such as tinnitus or orbital phenomena, 258 (24%) presented with intracranial hemorrhage, and 167 (16%) presented with nonhemorrhagic neurological deficits. A smaller proportion (224 patients, 21%), whose dAVFs were discovered incidentally, were asymptomatic. Many patients (85%, 911/1077) underwent treatment via endovascular embolization (55%, 587/1077), surgery (10%, 103/1077), radiosurgery (3%, 36/1077), or multimodal therapy (17%, 184/1077). The overall angiographic cure rate was 83% (758/911 treated), and treatment-related permanent neurological morbidity was 2% (27/1467 total procedures). The median time from diagnosis to follow-up was 380 days (IQR 120–1038.5 days). CONCLUSIONS With more than 1000 patients, the CONDOR registry represents the largest registry of cranial dAVF patient data in the world. These unique, well-annotated data will enable multiple future analyses to be performed to better understand dAVFs and their management.

OBJECTIVE Current evidence suggests that intracranial dural arteriovenous fistulas (dAVFs) without cortical venous drainage (CVD) have a benign clinical course. However, no large study has evaluated the safety and efficacy of current treatments and their impact over the natural history of dAVFs without CVD. METHODS The authors conducted an analysis of the retrospectively collected multicenter Consortium for Dural Arteriovenous Fistula Outcomes Research (CONDOR) database. Patient demographics and presenting symptoms, angiographic features of the dAVFs, and treatment outcomes of patients with Borden type I dAVFs were reviewed. Clinical and radiological follow-up information was assessed to determine rates of new intracranial hemorrhage (ICH) or nonhemorrhagic neurological deficit (NHND), worsening of venous hyperdynamic symptoms (VHSs), angiographic recurrence, and progression or spontaneous regression of dAVFs over time. RESULTS A total of 342 patients/Borden type I dAVFs were identified. The mean patient age was 58.1 ± 15.6 years, and 62% were women. The mean follow-up time was 37.7 ± 54.3 months. Of 230 (67.3%) treated dAVFs, 178 (77%) underwent mainly endovascular embolization, 11 (4.7%) radiosurgery alone, and 4 (1.7%) open surgery as the primary modality. After the first embolization, most dAVFs (47.2%) achieved only partial reduction in early venous filling. Multiple complementary interventions increased complete obliteration rates from 37.9% after first embolization to 46.7% after two or more embolizations, and 55.2% after combined radiosurgery and open surgery. Immediate postprocedural complications occurred in 35 dAVFs (15.2%) and 6 (2.6%) with permanent sequelae. Of 127 completely obliterated dAVFs by any therapeutic modality, 2 (1.6%) showed angiographic recurrence/recanalization at a mean of 34.2 months after treatment. Progression to Borden-Shucart type II or III was documented in 2.2% of patients and subsequent development of a new dAVF in 1.6%. Partial spontaneous regression was found in 22 (21.4%) of 103 nontreated dAVFs. Multivariate Cox regression analysis demonstrated that older age, NHND, or severe venous-hyperdynamic symptoms at presentation and infratentorial location were associated with worse prognosis. Kaplan-Meier curves showed no significant difference for stable/improved symptoms survival probability in treated versus nontreated dAVFs. However, estimated survival times showed better trends for treated dAVFs compared with nontreated dAVFs (288.1 months vs 151.1 months, log-rank p = 0.28). This difference was statistically significant for treated dAVFs with 100% occlusion (394 months, log-rank p < 0.001). CONCLUSIONS Current therapeutic modalities for management of dAVFs without CVD may provide better symptom control when complete angiographic occlusion is achieved.

Stereotactic lesioning of the bilateral globus pallidus (GPi) was one of the first surgical treatments for medication‐refractory dystonia but has largely been abandoned in clinical practice after the introduction of deep brain stimulation (DBS). However, some patients with dystonia are not eligible for DBS. Therefore, we reviewed the efficacy, safety, and sustainability of bilateral pallidotomy by conducting a systematic review of individual patient data (IPD). Guidelines of the Preferred Reporting Items for Systematic Reviews and Meta‐Analyses and IPD were followed. In May 2020, Medline, Embase, Web of Science, and Cochrane Library were searched for studies reporting on outcome of bilateral pallidotomy for dystonia. If available, IPD were collected. In this systematic review, 100 patients from 33 articles were evaluated. Adverse events were reported in 20 patients (20%), of which 8 were permanent (8%). Pre‐and postoperative Burke‐Fahn‐Marsden Dystonia Rating Movement Scale scores were available for 53 patients. A clinically relevant improvement (>20%) of this score was found in 42 of 53 patients (79%). Twenty‐five patients with status dystonicus (SD) were described. In all but 2 the SD resolved after bilateral pallidotomy. Seven patients experienced a relapse of SD. Median‐reported follow‐up was 12 months (n = 83; range: 2–180 months). Based on the current literature, bilateral pallidotomy is an effective and relatively safe procedure for certain types of dystonia, particularly in medication‐refractory SD. Although due to publication bias the underreporting of negative outcomes is very likely, bilateral pallidotomy is a reasonable alternative to DBS in selected dystonia patients. © 2020 The Authors. Movement Disorders published by Wiley Periodicals LLC on behalf of International Parkinson and Movement Disorder Society.

Background: Thalamotomy is an endorsed treatment for medication-refractory tremor. It used to be the standard, but nowadays deep brain stimulation (DBS) has become the treatment option of choice. Nevertheless, DBS has the disadvantage of hardware failure, battery replacement, and frequent setting adjustment. Radiofrequency (RF) thalamotomy lacks these issues, is relatively inexpensive, and has a broad applicability in patients with significant comorbidity. Therefore, we analyzed the long-term patient-reported outcome of RF thalamotomy in a cohort of patients with an otherwise intractable tremor. Methods: A single-center cohort of 27 consecutive patients with intractable tremor was assessed after unilateral RF thalamotomy. Over time, 4 patients had died because of non-related causes. In total, 21 patients responded to a telephone survey to assess their personal judgment on postoperative tremor severity, using a validated tremor scale, adverse events, recurrence, and patient satisfaction. The median time between surgery and telephone survey was 39 months (range 12–126). Seven patients had an additional analysis with postoperative imaging, video-assisted electromyography tremor registration, and a self-reported treatment effect (SRTE) assessment. Results: Nineteen out of 21 patients (90.5%) reported absence or significant improvement of their tremor. The rating score (WHIGET/UPDRS-III) dropped significantly from a mean of 3.57 preoperatively to 1.05 postoperatively (p < 0.001). Eleven patients (52.4%) reported adverse events, but the majority (76.2%) did not consider the adverse events to be severe. SRTE assessment showed a direct postoperative effect of 89.6 of 100 points (SD 10.8), with a gradual decrease to 75.3 (SD 23.5) during follow-up. Conclusions: RF thalamotomy is a very effective long-term treatment for medication-refractory tremor and should therefore be considered in patients with a refractory unilateral tremor.

Background and ObjectivesWe combined individual patient data (IPD) from prospective cohorts of patients with unruptured intracranial aneurysms (UIAs) to assess to what extent patients with familial UIA have a higher rupture risk than those with sporadic UIA.MethodsFor this IPD meta-analysis, we performed an Embase and PubMed search for studies published up to December 1, 2020. We included studies that (1) had a prospective study design; (2) included 50 or more patients with UIA; (3) studied the natural course of UIA and risk factors for aneurysm rupture including family history for aneurysmal subarachnoid haemorrhage and UIA; and (4) had aneurysm rupture as an outcome. Cohorts with available IPD were included. All studies included patients with newly diagnosed UIA visiting one of the study centers. The primary outcome was aneurysmal rupture. Patients with polycystic kidney disease and moyamoya disease were excluded. We compared rupture rates of familial vs sporadic UIA using a Cox proportional hazard regression model adjusted for PHASES score and smoking. We performed 2 analyses: (1) only studies defining first-degree relatives as parents, children, and siblings and (2) all studies, including those in which first-degree relatives are defined as only parents and children, but not siblings.ResultsWe pooled IPD from 8 cohorts with a low and moderate risk of bias. First-degree relatives were defined as parents, siblings, and children in 6 cohorts (29% Dutch, 55% Finnish, 15% Japanese), totaling 2,297 patients (17% familial, 399 patients) with 3,089 UIAs and 7,301 person-years follow-up. Rupture occurred in 10 familial cases (rupture rate: 0.89%/person-year; 95% confidence interval [CI] 0.45–1.59) and 41 sporadic cases (0.66%/person-year; 95% CI 0.48–0.89); adjusted hazard ratio (HR) for familial cases 2.56 (95% CI 1.18–5.56). After adding the 2 cohorts excluding siblings as first-degree relatives, resulting in 9,511 patients, the adjusted HR was 1.44 (95% CI 0.86–2.40).DiscussionThe risk of rupture of UIA is 2.5 times higher, with a range from a 1.2 to 5 times higher risk, in familial than in sporadic UIA. When assessing the risk of rupture in UIA, family history should be taken into account.

Background and purpose: Delayed cerebral ischaemia (DCI) is a severe complication of aneurysmal subarachnoid hemorrhage that can significantly impact clinical outcome. Cerebral vasospasm is part of the pathophysiology of DCI and therefore a computed tomography angiography (CTA) Vasospasm Score was developed and an exploration was carried out of whether this score predicts DCI and subsequent poor outcome after aneurysmal subarachnoid hemorrhage. Methods: The CTA Vasospasm Score sums the degree of angiographic cerebral vasospasm of 17 intradural arterial segments. The score ranges from 0 to 34 with a higher score reflecting more severe vasospasm. Outcome measures were cerebral infarction due to DCI (CI-DCI), radiological and clinical DCI, and unfavorable functional outcome defined as a modified Rankin Scale >2 at 6 months. Receiver operating characteristic analyses were used to assess predictive value and to determine optimal cut-off scores. Inter-rater reliability was evaluated by Cohen's kappa coefficient. Results: This study included 59 patients. CI-DCI occurred in eight patients (14%), DCI in 14 patients (24%) and unfavorable outcome in 12 patients (20%). Median CTA Vasospasm Scores were higher in patients with (CI-)DCI and poor outcome. Receiver operating characteristic analysis revealed the highest area under the curve on day 5: CI-DCI 0.89 (95% confidence interval [CI] 0.79–0.99), DCI 0.68 (95% CI 0.50–0.87) and functional outcome 0.74 (95% CI 0.57–0.91). Cohen's kappa between the two raters was moderate to substantial (0.57–0.63). Conclusions: This study demonstrates that the CTA Vasospasm Score on day 5 can reliably identify patients with a high risk of developing (CI-)DCI and unfavorable outcome.

Background and Purpose: Dural arteriovenous fistulae can present with hemorrhage, but there remains a paucity of data regarding subsequent outcomes. We sought to use the CONDOR (Consortium for Dural Arteriovenous Fistula Outcomes Research), a multi-institutional registry, to characterize the morbidity and mortality of dural arteriovenous fistula–related hemorrhage. Methods: A retrospective review of patients in CONDOR who presented with dural arteriovenous fistula–related hemorrhage was performed. Patient characteristics, clinical follow-up, and radiographic details were analyzed for associations with poor outcome (defined as modified Rankin Scale score ≥3). Results: The CONDOR dataset yielded 262 patients with incident hemorrhage, with median follow-up of 1.4 years. Poor outcome was observed in 17.0% (95% CI, 12.3%–21.7%) at follow-up, including a 3.6% (95% CI, 1.3%–6.0%) mortality. Age and anticoagulant use were associated with poor outcome on multivariable analysis (odds ratio, 1.04, odds ratio, 5.1 respectively). Subtype of hemorrhage and venous shunting pattern of the lesion did not affect outcome significantly. Conclusions: Within the CONDOR registry, dural arteriovenous fistula–related hemorrhage was associated with a relatively lower morbidity and mortality than published outcomes from other arterialized cerebrovascular lesions but still at clinically consequential rates.

BACKGROUND AND PURPOSE: Aneurysm wall enhancement (AWE) of intracranial aneurysms on magnetic resonance imaging has been described in previous studies as a surrogate marker of instability. With this study, an updated literature overview and summary risk estimates of the association between AWE and different specific outcomes (i.e., rupture, growth or symptomatic presentation) for both cross-sectional and longitudinal studies are provided. METHODS: The PRISMA guideline was followed and a search was performed of PubMed and Embase to 1 January 2021 for studies that reported on AWE and aneurysm instability. In cross-sectional studies, AWE was compared between patients with stable and unstable aneurysms. In longitudinal studies, AWE of stable aneurysms was assessed at baseline after which patients were followed longitudinally. Risk ratios were calculated for longitudinal studies, prevalence ratios for cross-sectional studies and then the ratios were pooled in a random-effects meta-analysis. Also, the performance of AWE to differentiate between stable and unstable aneurysms was evaluated. RESULTS: Twelve studies were included with a total of 1761 aneurysms. In cross-sectional studies, AWE was positively associated with rupture (prevalence ratio 11.47, 95% confidence interval [CI] 4.05-32.46) and growth or symptomatic presentation (prevalence ratio 4.62, 95% CI 2.85-7.49). Longitudinal studies demonstrated a positive association between AWE and growth or rupture (risk ratio 8.00, 95% CI 2.14-29.88). Assessment of the performance of AWE showed high sensitivities, mixed specificities, low positive predictive values and high negative predictive values. CONCLUSIONS: Although AWE is positively associated with aneurysm instability, current evidence mostly supports the use of its absence as a surrogate marker of aneurysm stability.

BACKGROUND: Low-grade intracranial dural arteriovenous fistulas (dAVF) have a benign natural history in the majority of cases. The benefit from treatment of these lesions is controversial.OBJECTIVE: To compare the outcomes of observation versus intervention for low-grade dAVFs.METHODS: We retrospectively reviewed dAVF patients from institutions participating in the CONsortium for Dural arteriovenous fistula Outcomes Research (CONDOR). Patients with low-grade (Borden type I) dAVFs were included and categorized into intervention or observation cohorts. The intervention and observation cohorts were matched in a 1:1 ratio using propensity scores. Primary outcome was modified Rankin Scale (mRS) at final follow-up. Secondary outcomes were excellent (mRS 0-1) and good (mRS 0-2) outcomes, symptomatic improvement, mortality, and obliteration at final follow-up.RESULTS: The intervention and observation cohorts comprised 230 and 125 patients, respectively. We found no differences in primary or secondary outcomes between the 2 unmatched cohorts at last follow-up (mean duration 36 mo), except obliteration rate was higher in the intervention cohort (78.5% vs 24.1%, P < .001). The matched intervention and observation cohorts each comprised 78 patients. We also found no differences in primary or secondary outcomes between the matched cohorts except obliteration was also more likely in the matched intervention cohort (P < .001). Procedural complication rates in the unmatched and matched intervention cohorts were 15.4% and 19.2%, respectively.CONCLUSION: Intervention for low-grade intracranial dAVFs achieves superior obliteration rates compared to conservative management, but it fails to improve neurological or functional outcomes. Our findings do not support the routine treatment of low-grade dAVFs.

Background and Purpose— Early prediction of clinical outcome after aneurysmal subarachnoid hemorrhage (aSAH) is still lacking accuracy. In this observational cohort study, we aimed to develop and validate an accurate bedside prediction model for clinical outcome after aSAH, to aid decision-making at an early stage. Methods— For the development of the prediction model, a prospectively kept single-center cohort of 1215 aSAH patients, admitted between 1998 and 2014, was used. For temporal validation, a prospective cohort of 224 consecutive aSAH patients from the same center, admitted between 2015 and 2017, was used. External validation was performed using the ISAT (International Subarachnoid Aneurysm Trial) database (2143 patients). Primary outcome measure was poor functional outcome 2 months after aSAH, defined as modified Rankin Scale score 4–6. The model was constructed using multivariate regression analyses. Performance of the model was examined in terms of discrimination and calibration. Results— The final model included 4 predictors independently associated with poor outcome after 2 months: age, World Federation of Neurosurgical Societies grade after resuscitation, aneurysm size, and Fisher grade. Temporal validation showed high discrimination (area under the receiver operating characteristic curve, 0.90; 95% CI, 0.85–0.94), external validation showed fair to good discrimination (area under the receiver operating characteristic curve, 0.73; 95% CI, 0.70–0.76). The model showed satisfactory calibration in both validation cohorts. The SAFIRE grading scale was derived from the final model: size of the aneurysm, age, Fisher grade, world federation of neurosurgical societies after resuscitation. Conclusions— The SAFIRE grading scale is an accurate, generalizable, and easily applicable model for early prediction of clinical outcome after aSAH.

BackgroundAlthough the liquid embolic agent, Onyx, is often the preferred embolic treatment for cerebral dural arteriovenous fistulas (DAVFs), there have only been a limited number of single-center studies to evaluate its performance.ObjectiveTo carry out a multicenter study to determine the predictors of complications, obliteration, and functional outcomes associated with primary Onyx embolization of DAVFs.MethodsFrom the Consortium for Dural Arteriovenous Fistula Outcomes Research (CONDOR) database, we identified patients who were treated for DAVF with Onyx-only embolization as the primary treatment between 2000 and 2013. Obliteration rate after initial embolization was determined based on the final angiographic run. Factors predictive of complete obliteration, complications, and functional independence were evaluated with multivariate logistic regression models.ResultsA total 146 patients with DAVFs were primarily embolized with Onyx. Mean follow-up was 29 months (range 0–129 months). Complete obliteration was achieved in 80 (55%) patients after initial embolization. Major cerebral complications occurred in six patients (4.1%). At last follow-up, 84% patients were functionally independent. Presence of flow symptoms, age over 65, presence of an occipital artery feeder, and preprocedural home anticoagulation use were predictive of non-obliteration. The transverse-sigmoid sinus junction location was associated with fewer complications, whereas the tentorial location was predictive of poor functional outcomes.ConclusionsIn this multicenter study, we report satisfactory performance of Onyx as a primary DAVF embolic agent. The tentorium remains a more challenging location for DAVF embolization, whereas DAVFs located at the transverse-sigmoid sinus junction are associated with fewer complications.

BackgroundBeta-based adaptive Deep Brain Stimulation (aDBS) is effective in Parkinson's disease (PD), when assessed in the immediate post-implantation phase. However, the potential benefits of aDBS in patients with electrodes chronically implanted, in whom changes due to the microlesion effect have disappeared, are yet to be assessed.MethodsTo determine the acute effectiveness and side-effect profile of aDBS in PD compared to conventional continuous DBS (cDBS) and no stimulation (NoStim), years after DBS implantation, 13 PD patients undergoing battery replacement were pseudo-randomised in a crossover fashion, into three conditions (NoStim, aDBS or cDBS), with a 2-min interval between them. Patient videos were blindly evaluated using a short version of the Unified Parkinson's Disease Rating Scale (subUPDRS), and the Speech Intelligibility Test (SIT).ResultsMean disease duration was 16 years, and the mean time since DBS-implantation was 6.9 years. subUPDRS scores (11 patients tested) were significantly lower both in aDBS (p=

OBJECTIVE The risk-to-benefit profile of treating an unruptured high-grade dural arteriovenous fistula (dAVF) is not clearly defined. The aim of this multicenter retrospective cohort study was to compare the outcomes of different interventions with observation for unruptured high-grade dAVFs. METHODS The authors retrospectively reviewed dAVF patients from 12 institutions participating in the Consortium for Dural Arteriovenous Fistula Outcomes Research (CONDOR). Patients with unruptured high-grade (Borden type II or III) dAVFs were included and categorized into four groups (observation, embolization, surgery, and stereotactic radiosurgery [SRS]) based on the initial management. The primary outcome was defined as the modified Rankin Scale (mRS) score at final follow-up. Secondary outcomes were good outcome (mRS scores 0–2) at final follow-up, symptomatic improvement, all-cause mortality, and dAVF obliteration. The outcomes of each intervention group were compared against those of the observation group as a reference, with adjustment for differences in baseline characteristics. RESULTS The study included 415 dAVF patients, accounting for 29, 324, 43, and 19 in the observation, embolization, surgery, and SRS groups, respectively. The mean radiological and clinical follow-up durations were 21 and 25 months, respectively. Functional outcomes were similar for embolization, surgery, and SRS compared with observation. With observation as a reference, obliteration rates were higher after embolization (adjusted OR [aOR] 7.147, p = 0.010) and surgery (aOR 33.803, p < 0.001) and all-cause mortality was lower after embolization (imputed, aOR 0.171, p = 0.040). Hemorrhage rates per 1000 patient-years were 101 for observation versus 9, 22, and 0 for embolization (p = 0.022), surgery (p = 0.245), and SRS (p = 0.077), respectively. Nonhemorrhagic neurological deficit rates were similar between each intervention group versus observation. CONCLUSIONS Embolization and surgery for unruptured high-grade dAVFs afforded a greater likelihood of obliteration than did observation. Embolization also reduced the risk of death and dAVF-associated hemorrhage compared with conservative management over a modest follow-up period. These findings support embolization as the first-line treatment of choice for appropriately selected unruptured Borden type II and III dAVFs.

Background: Deep brain stimulation (DBS) of the globus pallidus pars interna (GPi) and the subthalamic nucleus (STN) are established treatment option in Parkinson's disease (PD). If DBS does not provide the desired effect, re-operation to the alternative target is a treatment option, but data on the effect of re-operation are scarce. Objectives: The objective of this study is to evaluate the clinical effect of re-operation the alternative target after failure of initial STN or GPi DBS for Parkinson's disease. Materials and Methods: We descriptively analyzed the baseline characteristics, the effect of initial surgery and re-operation of eight NSTAPS (Netherlands SubThalamic and Pallidal Stimulation) patients and six previously published cases that underwent re-operation to a different target. Results: In the NSTAPS cohort, two of the eight patients showed more than 30% improvement of off-drug motor symptoms after re-operation. The initial DBS leads of these patients were off target. In the cases from the literature, 30% off-drug motor improvement was seen in all three patients re-operated from GPi to STN and none of the three patients re-operated from STN to GPi. Only one of the three cases from the literature where any improvement was seen with the operation had a confirmed on target lead location after the first surgery, while the other two patients did not undergo post-operative imaging after the first surgery. Conclusions: Re-operation to a different target due to lack of effect appears to have a limited chance of leading to objective improvement if the leads were correctly placed during initial surgery.

OBJECTIVEVarious international and national gastrointestinal guidelines take different positions on whether ventriculoperitoneal shunt (VPS) insertion is a contraindication to percutaneous endoscopic gastrostomy (PEG). The objective of this meta-analysis was to try to answer the question of whether VPS insertion is a contraindication to PEG.METHODSA systematic review of the literature was performed according to the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) criteria. Electronic databases PubMed and Embase were searched using variations of the terms “ventriculo-peritoneal shunt” and “percutaneous (endoscopic) gastrostomy.” This search resulted in 70 studies, 9 of which were relevant. These were cross-referenced, and 1 additional study was found, resulting in 10 studies in this systematic review.RESULTSThe 10 relevant studies in adult cohorts included 208 patients. All studies save one were retrospective and, in general, poor quality. Among the studies with relevant data, there were 26 (12.5% of 208 cases) VPS infections and 4 (4.4% of 90 cases) VPSs that malfunctioned. In 137 patients the VPS had been placed before the PEG tube, with a VPS infection rate of 4.4%. More VPS infections occurred among the 55 patients who first had a PEG and a subsequent VPS (21.8%) and in the 16 patients who had simultaneous PEG tube and VPS placement (50%). The heterogeneity of the studies in this analysis prohibited statistical comparisons of the timing of VPS and PEG tube placement.CONCLUSIONSThis systematic review indicated that VPS placement in combination with a PEG has a high but acceptable VPS complication rate. Therefore, VPS insertion should not be considered a contraindication to the placement of a PEG tube. Preferably, a PEG tube should be placed after the VPS. Waiting 7–10 days between VPS insertion and a PEG seems reasonable, but this could not be corroborated in this review.

OBJECTIVEMicrovascular decompression (MVD) is regarded as a valid treatment modality in neurovascular conflicts (NVCs) causing, for example, trigeminal neuralgia and hemifacial spasms. An NVC of the cochleovestibular nerve might cause tinnitus and/or vertigo; however, general acceptance of MVD for this indication is lacking. The aim of this study was to investigate the effectiveness, safety, and prognostic factors for success of MVD of the cochleovestibular nerve.METHODSA systematic review and meta-analysis of individual patient data (IPD) were conducted according to the Preferred Reporting Items for Systematic Reviews and Meta-Analyses and Individual Patient Data (PRISMA-IPD) guidelines. By a comprehensive search (conducted in January 2016) in MEDLINE, EMBASE, and Google Scholar, eligible studies were identified. The collected outcome was a global measurement of improvement of 1) tinnitus, 2) vertigo, and 3) tinnitus combined with vertigo. For the meta-analysis, IPD were collected from the papers and/or from the authors. IPD were analyzed with logistic regression analysis while accounting for study clustering.RESULTSThirty-five studies (572 patients) were included. The level of evidence provided by these studies was low. In 28% of patients with tinnitus and 32% of patients with vertigo, complete relief following MVD was reported. Patients with both tinnitus and vertigo had complete relief in 62% of cases. In 11% of patients, ≥ 1 complications were reported. Meta-analysis of IPD (165 patients) demonstrated that patients with both tinnitus and vertigo had a higher chance of success (OR 3.8, 95% CI 1.45–10.10) than patients with tinnitus alone. No other variables were significantly related to success.CONCLUSIONSDue to low success rates, MVD cannot be considered as a standard treatment method for tinnitus or vertigo. Moreover, a substantial complication rate was found. However, patients with combined symptoms had a higher chance of success. When combined symptoms occur, it is more likely that an NVC is the underlying pathology and MVD might be appropriate. Due to the low level of evidence in the included studies, this conclusion must be taken with caution. Further validation is necessary to evaluate whether patients with combined symptoms are indeed better candidates for MVD.

Background.-The term "cluster-tic syndrome" is used for the rare ipsilateral co-occurrence of attacks of cluster headache and trigeminal neuralgia. Medical treatment should combine treatment for cluster headache and trigeminal neuralgia, but is very often unsatisfactory.Case.-Here, we describe a 41-year-old woman diagnosed with cluster-tic syndrome who underwent microvascular decompression of the trigeminal nerve, primarily aimed at the "trigeminal neuralgia" part of her pain syndrome. After venous decompression of the trigeminal nerve both a decrease in trigeminal neuralgia and cluster headache attacks was seen. However, the headache did not disappear completely. Furthermore, she reported a decrease in pain intensity of the remaining cluster headache attacks.Discussion.-This case description suggests that venous vascular decompression in cluster-tic syndrome can be remarkably effective, both for trigeminal neuralgia and cluster headache.

OBJECTIVEUnruptured intracranial aneurysms are common incidental findings on brain imaging. Short-term follow-up for conservatively treated aneurysms is routinely performed in most cerebrovascular centers, although its clinical relevance remains unclear. In this study, the authors assessed the extent of growth as well as the rupture risk during short-term follow-up of conservatively treated unruptured intracranial aneurysms. In addition, the influence of patient-specific and aneurysm-specific factors on growth and rupture risk was investigated.METHODSThe authors queried their prospective institutional neurovascular registry to identify patients with unruptured intracranial aneurysms and short-term follow-up imaging, defined as follow-up MRA and/or CTA within 3 months to 2 years after initial diagnosis. Medical records and questionnaires were used to acquire baseline information. The authors measured aneurysm size at baseline and at follow-up to detect growth. Rupture was defined as a CT scan–proven and/or CSF-proven subarachnoid hemorrhage (SAH).RESULTSA total of 206 consecutive patients with 267 conservatively managed unruptured aneurysms underwent short-term follow-up at the authors’ center. Seven aneurysms (2.6%) enlarged during a median follow-up duration of 1 year (range 0.3–2.0 years). One aneurysm (0.4%) ruptured 10 months after initial discovery. Statistically significant risk factors for growth or rupture were autosomal-dominant polycystic kidney disease (RR 8.3, 95% CI 2.0–34.7), aspect ratio > 1.6 or size ratio > 3 (RR 10.8, 95% CI 2.2–52.2), and initial size ≥ 7 mm (RR 10.7, 95% CI 2.7–42.8).CONCLUSIONSSignificant growth of unruptured intracranial aneurysms may occur in a small proportion of patients during short-term follow-up. As aneurysm growth is associated with an increased risk of rupture, the authors advocate that short-term follow-up is clinically relevant and has an important role in reducing the risk of a potential SAH.

IntroductionAdaptive deep brain stimulation (aDBS), based on the detection of increased beta oscillations in the subthalamic nucleus (STN), has been assessed in patients with Parkinson’s disease (PD) during the immediate postoperative setting. In these studies, aDBS was shown to be at least as effective as conventional DBS (cDBS), while stimulation time and side effects were reduced. However, the effect of aDBS on motor symptoms and stimulation-induced side effects during the chronically implanted phase (after the stun effect of DBS placement has disappeared) has not yet been determined.Methods and analysisThis protocol describes a single-centre clinical study in which aDBS will be tested in 12 patients with PD undergoing battery replacement, with electrodes implanted in the STN, and as a proof of concept in the internal globus pallidus. Patients included will be allocated in a pseudo-randomised fashion to a three-condition (no stimulation/cDBS/ aDBS), cross-over design. A battery of tests will be conducted and recorded during each condition, which aim to measure the severity of motor symptoms and side effects. These tests include a tablet-based tapping test, a subscale of the Movement Disorder Society-unified Parkinson’s disease rating scale (subMDS-UPDRS), the Speech Intelligibility Test (SIT) and a tablet-based version of the Stroop test. SubMDS-UPDRS and SIT recordings will be blindly assessed by independent raters. Data will be analysed using a linear mixed-effects model.Ethics and disseminationThis protocol was approved by the Ethical Committee of the University Medical Centre Groningen, where the study will be carried out. Data management and compliance to research policies and standards of our centre, including data privacy, storage and veracity, will be controlled by an independent monitor. All the scientific findings derived from this protocol are aimed to be made public through publication of articles in international journals.Trial registration numberNTR 5456; Pre-results.

A 73-year-old woman with a history of hypertension and diabetes presented with sudden headache and right-sided periorbital ecchymosis (figure). There was no history of trauma. Neurologic examination was unremarkable. Ophthalmologic examination revealed normal vision and intact ocular movements and no papilledema or retinal hemorrhage. CT angiography showed subarachnoid hemorrhage (SAH) from a right ophthalmic artery aneurysm (figure). The patient underwent successful endovascular treatment and made a full recovery. In addition to skull fracture and cavernous sinus thrombosis, SAH is an important differential diagnosis of sudden severe headache with periorbital ecchymosis.

Background: ISAT demonstrated that 1 year after aneurysmal subarachnoid haemorrhage (aSAH), coiling resulted in a significantly better clinical outcome (death or dependency at 1 year) than clipping. After 5 years, this difference did not reach statistical significance but mortality (secondary endpoint) was still higher in the clipping group. Here, we present additional analyses, reporting the outcome after excluding pre-treatment death and/or re-bleeding. Methods: Outcome measures were death or dependency, and death alone, 1 and 5 years after treatment. We investigated outcome after i. exclusion of all pre-treatment deaths ii. exclusion of all pre-treatment rebleedings iii. 'as-treated' analysis and iv. as-treated analysis including good-grade patients (WFNS 0-2). Treatment differences were assessed using relative risks. Exploratory analyses were used to investigate the relation between treatment delay and outcome. Findings: After exclusion of pre-treatment deaths, coiling was favourable over clipping at 1-year follow-up for death or dependency (RR 0·77; 95%CI 0·67-0·89), but not for death alone (RR 0·88; 95%CI 0·66-1·19). After 5 years, no significant differences were observed, neither for death or dependency (RR 0·88; 95%CI 0·77-1·02), nor death alone (RR 0·82; 95%CI 0·64-1·05). Analyses ii. and iii showed a similar picture. In good-grade patients coiling remained favourable over clipping in long-term. Time between randomisation and treatment was significantly longer in the clipping arm (mean 1·7 versus 1·1 days; p

Cerebral vasospasm in the first 2 weeks after aneurysmal subarachnoid hemorrhage is recognized as a major predictor of delayed cerebral ischemia. The routine screening for cerebral vasospasm with either transcranial Doppler or CT angiography has been advocated, although its diagnostic value has not yet been determined. Our study investigated the diagnostic accuracy of detecting vasospasm by transcranial Doppler and CT angiography for the prediction of delayed cerebral ischemia and functional outcome. Additionally, agreement between transcranial Doppler and CT angiography was determined.Design: Prospective diagnostic accuracy study.Settings: Neurocritical care unit and neurosurgical ward at a tertiary academic medical center.Patients: Between 2013 and 2016, 59 consenting patients were included.Intervention: Patients undergo both transcranial Doppler and CT angiography for detection of cerebral vasospasm on days 5 and 10 after aneurysmal subarachnoid hemorrhage. Delayed cerebral ischemia was defined as secondary neurologic deterioration, not explained otherwise. Unfavorable outcome was defined modified Rankin Scale > 2 at 6 months.Measurements and Main Results: On transcranial Doppler, cerebral vasospasm was observed in 26 patients (45%). On CT angiography, vasospasm was observed in 54 patients (95%). The agreement between transcranial Doppler and CT angiography was 0.47. Delayed cerebral ischemia occurred in 16 patients (27%); unfavorable outcome in 12 patients (20%). Transcranial Doppler predicted delayed cerebral ischemia with a sensitivity of 0.44 (day 5) and 0.50 (day 10), with a specificity of 0.67 (day 5) and 0.57 (day 10). CT angiography predicted delayed cerebral ischemia with a sensitivity of 0.81 (day 5 and 10) and with a specificity of 0.070 (day 5) and 0.00 (day 10). The highest accuracy for predicting unfavorable outcome was on day 5 (0.61 for transcranial Doppler vs 0.27 for CT angiography).Conclusion: The diagnostic accuracy of both CT angiography and transcranial Doppler for detection of cerebral vasospasm as well as prediction of delayed cerebral ischemia and functional outcome is limited. The agreement between CT angiography and transcranial Doppler is low.

Introduction: Dystonia-deafness syndrome is a distinct clinical presentation within the dystonia-spectrum. Although several genetic and acquired causes have been reported, etiology remains unknown in the majority of patients. Objectives: To describe two patients with dystonia-deafness syndrome due to a beta-actin gene mutation. Methods: We report on disease course, genetic testing, and management of 2 patients, mother and daughter, presenting with dystonia-deafness syndrome. Results: After exclusion of known dystonia-deafness syndrome causes, whole-exome sequencing revealed a beta-actin gene mutation (p.Arg183Trp) in both patients. Although beta-actin gene mutations are generally associated with developmental Baraitser-Winter syndrome, dystonia-deafness syndrome has been reported once in identical twin brothers. Bilateral GPi-DBS led to a significant decrease of dystonia and regain of independency in our patients. Conclusion: The p.Arg183Trp mutation in the beta-actin gene is associated with the clinical presentation of dystonia-deafness syndrome, even with only minimal or no developmental abnormalities of Baraitser-Winter syndrome. GPi-DBS should be considered to ameliorate the invalidating dystonia in these patients. (C) 2016 International Parkinson and Movement Disorder Society.

Background: To systematically evaluate the effectiveness of deep brain stimulation of the globus pallidus internus (GPi-DBS) in dystonia on pre-operatively set functional priorities in daily living.\ud Methods: Fifteen pediatric and adult dystonia patients (8 male; median age 32y, range 8–65) receiving GPi-DBS were recruited. All patients underwent a multidisciplinary evaluation before and 1-year post DBS implantation. The Canadian Occupational Performance Measure (COPM) first identified and then measured changes in functional priorities. The Burke-Fahn-Marsden Dystonia Rating Scale (BFMDRS) was used to evaluate dystonia severity.\ud Results: Priorities in daily functioning substantially varied between patients but showed significant improvements on performance and satisfaction after DBS. Clinically significant COPM-score improvements were present in 7/8 motor responders, but also in 4/7 motor non-responders.\ud Discussion: The use of a patient-oriented approach to measure GPi-DBS effectiveness in dystonia provides an unique insight in patients’ priorities and demonstrates that tangible improvements can be achieved irrespective of motor response.\ud Highlights\ud • Functional priorities in life of dystonia patients and their caregivers vary greatly\ud • The effect of DBS on functional priorities did not correlate with motor outcome\ud • Half of the motor ‘non-responder’ patients reported important changes in their priorities\ud • The effect of DBS in dystonia should not be measured by motor outcome alone

Tinnitus is a common problem, with a prevalence of about 8% in the general population. A pulsatile tinnitus occurs in less than 10% of tinnitus patients. It has an extensive differential diagnosis. In general, the underlying pathology for a pulsatile tinnitus is found in up to 70% of the cases.1 Most frequently, the diagnosis can be made based on non‐invasive imaging techniques (CT or CT‐angiography (CTA); MRI or MR‐angiography (MRA)). However, evaluation of flow dynamics is limited on MRI/MRA and CTA and particularly dural arteriovenous fistulas (dAVFs) are difficult to recognise. With an estimated incidence of 0.16 per 100 000 adults per year dAVFs are a rare intracranial vascular malformation.2 In more than 10% of the cases of patients with a dAVF, a pulsatile tinnitus is the only initial symptom.3 A dAVF with cortical venous involvement (Borden 2 or 3) can, if left untreated, have an aggressive natural disease course with neurological sequelae and even death.4 Therefore, patients with pulsatile tinnitus lacking a clear diagnose after non‐invasive imaging should be referred for digital substraction angiography (DSA), which is considered as the gold standard for detection of dAVFs (Figure ​(Figure1).1). On the other hand, DSA is an invasive imaging modality, with a procedural risk of 1%‐2% of neurological complications, of which 0.5% are permanent.5 In a tertiary care setting, a dAVF was found in 24% of patient with pulsatile tinnitus that underwent a DSA.6 As such, the majority of patients with pulsatile tinnitus indicated for a DSA are unnecessarily exposed to these risks. It would be valuable to have a screening tool to narrow indication for a DSA in patients with pulsatile tinnitus. Open in a separate window Figure 1 Lateral projection of a digital subtraction angiography examination demonstrating a dural arteriovenous fistula (dAVF)

BACKGROUND: Deep brain stimulation (DBS) is an established treatment for refractory essential tremor (ET). Initially, the target of choice was the thalamic ventralis intermedius nucleus (VIM). However, the zona incerta (ZI) has been suggested as a superior target. Both targets are considered safe and effective, but a direct comparison between these targets is lacking.METHODS: We analyzed a single-center cohort of 44 patients with ET treated with DBS between 1998 and 2017, targeting the VIM and/or ZI. Patient-reported outcome on the Washington Heights-Inwood Genetic Study of Essential Tremor rating scale, adverse events, and stimulation-induced side effects were assessed.RESULTS: Patient-reported outcome of ZI DBS (-2.2 +/- 1.2; 18 patients with 28 electrodes) was superior to VIM DBS (-1.2 +/- 1.4; 10 patients with 19 electrodes) (P CONCLUSIONS: In this study, ZI DBS was superior to VIM DBS in terms of patient-reported effectiveness. There was a comparable number of complications between both targets. This finding further supports ZI over VIM as the principal DBS target in essential tremor.

Background and Purpose— Aneurysmal subarachnoid hemorrhage (aSAH) is a devastating type of stroke associated with high morbidity and mortality. One of the most feared complications is an early rebleeding before aneurysm repair. Predictors for such an often fatal rebleeding are largely unknown. We therefore aimed to determine predictors for an early rebleeding after aSAH in relation with time after ictus. Methods— This observational prospective cohort study included all consecutive patients admitted with aSAH between January 1998 and December 2014 (n=1337) at our University Neurovascular Center. Clinical predictors for rebleeding ≤24 hours were identified using multivariable Cox regression analyses. Kaplan–Meier analyses were applied to evaluate the time of rebleeding ≤72 hours after aSAH. Results— A modified Fisher grade of 3 to 4 was a predictor for an in-hospital rebleeding ≤24 hours after ictus (adjusted hazard ratio, 4.4; 95% confidence interval, 2.1–10.6; P P Conclusions— In our opinion, timing of treatment of aSAH patients, especially those with an modified Fisher grade of 3 or 4 in a good clinical condition, should be reconsidered. These aSAH patients might be regarded a medical emergency, requiring aneurysm repair as soon as possible. In this respect, our findings should provoke the debate on timing of aneurysm repair, especially in patients considered to be at high risk for rebleeding.

BACKGROUND:There is an increasing tendency to treat spinal dural arteriovenous fistulas (SDAVFs) endovascularly despite the lack of clear evidence favoring embolization over surgery.OBJECTIVE:To compare the initial failure and recurrence rates of primary treatment of SDAVFs by surgery and endovascular techniques.METHODS:A meta-analysis using the PRISMA (Preferred Reporting Items for Systematic Reviews and Meta-Analyses) standard was performed. All the English literature from 2004 onward was evaluated. From each article that compared the 2 treatment modalities, the odds ratio (OR) was calculated. Combined ORs were calculated with Review Manager 5.3 of The Cochrane Collaboration.RESULTS:A total of 35 studies harboring 1112 patients were assessed. Initial definitive fistula occlusion was observed in 588 of 609 surgical patients (96.6%; 95% confidence interval [CI], 94.8-97.8) vs 363 of 503 endovascularly treated patients (72.2%; 95% CI, 68.1-75.9; P

Objectives/Hypothesis: The management of a sporadic vestibular schwannoma (VS) has changed with the introduction of stereotactic radiosurgery (SRS). Because functional outcome is important, particularly regarding the facial nerve, a policy of near-total surgical resection of a large-size VS has emerged, minimizing damage to the facial nerve. The debate remains whether the surgical remnant should be treated immediately or after established growth. Study Design: Retrospective case series. Methods: A consecutive cohort of 55 patients underwent a retrosigmoid craniotomy and near-total removal of a large-size VS at our university medical center between 2005 and 2011 and had a follow-up of a least 3 years. Documented growth of the VS remnant after surgery necessitating adjuvant SRS was the primary outcome measure using analysis of variance. Results: In 45 patients (81.8%), a small tumor remnant was left during surgery. The mean preoperative tumor volume was 12.2 cm(3) (range, 1.13-50.16 cm(3)); the mean volume of the remnant was 0.22 cm(3) (range, 0-1.52 cm(3)). The mean post-operative follow-up time was 35.4 months (range, 3-76 months). Salvage SRS was deemed necessary in seven patients (13.0%). The size of the postoperative tumor remnant was a significant predictor for the necessity of postoperative adjuvant SRS. Normal facial nerve function (House-Brackmann [HB] I) was preserved in 30 patients (57.7%), 17 patients (32.7%) experienced a permanent mild facial nerve deficit (HB II, III), and five patients (9.6%) experienced a severe facial nerve deficit (HB grade IV-VI). Conclusions: Initial observation after near total surgical removal of VS is a feasible strategy, with only a minority requiring salvage radiosurgery during follow-up.

OBJECTIVE: There is an ongoing search for conditions that induce spontaneous subarachnoid hemorrhage (SAH). The seasonal pattern of SAH is shown in a large meta-analysis of the literature, but its explanation remains undecided. There is a clear need for sound meteorologic data to further elucidate the seasonal influence on SAH. Because of the stable and densely monitored atmospheric situation in the north of the Netherlands, we reviewed our unique cohort on the seasonal incidence of SAH and the association between SAH and local atmospheric changes.METHODS: Our observational cohort study included 1535 patients with spontaneous SAH admitted to our neurovascular center in the north of the Netherlands between 2000 and 2015. Meteorologic data could be linked to the day of the ictus. To compare SAH incidences over the year and to test the association with meteorologic conditions, incidence rate ratios (IRRs) with corresponding 95% confidence intervals (Cis) were used, calculated by Poisson regression analyses.RESULTS: Atmospheric pressure variations were significantly associated with aneurysmal SAH. In particular, the pressure change on the second and third day before the ictus was independently correlated to a higher incidence of aneurysmal SAH (IRR, 1.11; 95% CI, 1.00-1.23). The IRR for aneurysmal SAH in July was calculated 0.67 (95% CI, 0.49-0.92) after adjustment for temperature and atmospheric pressure changes.CONCLUSIONS: Atmospheric pressure variations are a delayed trigger for aneurysmal SAH. Also, a significantly decreased incidence of aneurysmal SAH was noted in July.

Introduction: In tinnitus treatment, there is a tendency to shift from a "one size fits all" to a more individual, patient-tailored approach. Insight in the heterogeneity of the tinnitus spectrum might improve the management of tinnitus patients in terms of choice of treatment and identification of patients with severe mental distress. The goal of this study was to identify subgroups in a large group of tinnitus patients.Methods: Data were collected from patients with severe tinnitus complaints visiting our tertiary referral tinnitus care group at the University Medical Center Groningen. Patientreported and physician-reported variables were collected during their visit to our clinic. Cluster analyses were used to characterize subgroups. For the selection of the right variables to enter in the cluster analysis, two approaches were used: (1) variable reduction with principle component analysis and (2) variable selection based on expert opinion.Results: Various variables of 1,783 tinnitus patients were included in the analyses. Cluster analysis (1) included 976 patients and resulted in a four-cluster solution. The effect of external influences was the most discriminative between the groups, or clusters, of patients. The "silhouette measure" of the cluster outcome was low (0.2), indicating a "no substantial" cluster structure. Cluster analysis (2) included 761 patients and resulted in a three-cluster solution, comparable to the first analysis. Again, a "no substantial" cluster structure was found (0.2).Conclusion: Two cluster analyses on a large database of tinnitus patients revealed that clusters of patients are mostly formed by a different response of external influences on their disease. However, both cluster outcomes based on this dataset showed a poor stability, suggesting that our tinnitus population comprises a continuum rather than a number of clearly defined subgroups.

OBJECTIVE Currently, early prediction of outcome after spontaneous subarachnoid hemorrhage (SAH) lacks accuracy despite multiple studies addressing this issue. The clinical condition of the patient on admission as assessed using the World Federation of Neurosurgical Societies (WFNS) grading scale is currently considered the gold standard. However, the timing of the clinical assessment is subject to debate, as is the contribution of additional predictors. The aim of this study was to identify either the conventional WFNS grade on admission or the WFNS grade after neurological resuscitation (rWFNS) as the most accurate predictor of outcome after SAH. METHODS This prospective observational cohort study included 1620 consecutive patients with SAH admitted between January 1998 and December 2014 at our university neurovascular center. The primary outcome measure was a poor modified Rankin Scale score at the 2-month follow-up. Clinical predictors were identified using multivariate logistic regression analyses. Area under the receiver operating characteristic curve (AUC) analysis was used to test discriminative performance of the final model. An AUC of > 0.8 was regarded as indicative of a model with good prognostic value. RESULTS Poor outcome (modified Rankin Scale Score 4–6) was observed in 25% of the patients. The rWFNS grade was a significantly stronger predictor of outcome than the admission WFNS grade. The rWFNS grade was significantly associated with poor outcome (p < 0.001) as well as increasing age (p < 0.001), higher modified Fisher grade (p < 0.001), larger aneurysm size (p < 0.001), and the presence of an intracerebral hematoma (OR 1.8, 95% CI 1.2–2.8; p = 0.002). The final model had an AUC of 0.87 (95% CI 0.85–0.89), which indicates excellent prognostic value regarding the discrimination between poor and good outcome after SAH. CONCLUSIONS In clinical practice and future research, neurological assessment and grading of patients should be performed using the rWFNS to obtain the best representation of their clinical condition.

Object Microvascular decompression (MVD) is considered the method of choice to treat idiopathic trigeminal neuralgia (TN) refractory to medical treatment. However, repeat MVD for recurrent TN is not well established. In this paper, the authors describe a large case series in which patients underwent repeat MVD for recurrent TN, focusing on outcome, risk factors, and complication rates. Methods Between 1990 and 2012, a total of 33 consecutive patients underwent repeat MVD for recurrent TN at the University Medical Center Groningen. The authors performed a retrospective chart review and telephone interviews. Risk factors were analyzed by binary logistic regression analysis. Results After 12 months of follow-up, 22 (67%) operations were successful, of which 19 patients were completely free of pain without medication. With multivariate analysis significant risk factors for success were older age (OR 1.11, p < 0.01) and direct absence of pain after repeat MVD (OR 25.2, p < 0.01). Previous neurodestructive procedures did not influence success rates. Facial numbness occurred in 9 patients (27%), while other morbidity was minimal. There was no mortality. Conclusions This study demonstrates that repeat MVD is a feasible therapeutic option with good chances of success, even in patients who have undergone neurodestructive procedures. Complication rates, particularly facial numbness, can be avoided if only a limited neurolysis is performed.

OBJECTIVE: The authors' aim was to investigate cognitive outcome in patients with aneurysmal and angiographically negative subarachnoid hemorrhage (aSAH and anSAH), by comparing them to healthy controls and to each other. Besides investigating cognitive functions as memory and attention, they focused on higher-order prefrontal functions, namely executive functioning (EF) and emotion recognition.METHOD: Patients and healthy controls were assessed with tests measuring memory (15 Words Test, Digit Span), attention and processing speed (Trail Making Test A and B), EF (Zoo Map, Letter Fluency, Dysexecutive Questionnaire), and emotion recognition (Facial Expressions of Emotion Stimuli and Tests). Between-groups comparisons of test performances were made.RESULTS: Patients with aSAH scored significantly lower than healthy controls on measures of memory, processing speed, and attention, but anSAH patients did not. In the higher-order prefrontal functions (EF and emotion recognition), aSAH patients were clearly impaired when compared to healthy controls. However, anSAH patients did not perform significantly better than aSAH patients on the majority of the tests.CONCLUSIONS: In the subacute phase after SAH, cognitive functions, including the higher-order prefrontal functions EF and emotion recognition, were clearly impaired in aSAH patients. Patients with anSAH did not perform better than aSAH patients, which indicates that these functions may also be affected to some extent in anSAH patients. Considering the importance of these higher-order prefrontal functions for daily life functioning, and following the results of the present study, tests that measure emotion recognition and EF should be part of the standard neuropsychological assessment after SAH. (PsycINFO Database Record

OBJECTIVE: Tinnitus is a common entity that may lead to severe impairment in quality of life. An adequate treatment modality for severe tinnitus is currently lacking. Neurostimulation of the auditory tract may serve as a promising adjunct in tinnitus treatment. The aim is to investigate the effect of direct stimulation on the cochleovestibular nerve for intractable tinnitus.METHODS: This study was conducted at the University Medical Center Groningen, The Netherlands. We studied 10 patients with severe, unilateral, intractable tinnitus, who were implanted with a cuff electrode around the cochleovestibular nerve between 2001 and 2013. All patients had preoperative ipsilateral hearing loss. Tinnitus Handicap Inventory (THI) scores and audiometric values were collected. Treatment success was determined based on the self-assessment of satisfactory usage by each patient.RESULTS: The mean preoperative tinnitus duration was 8.0 +/- 5.9 years. The preoperative THI score was 71 +/- 18 points. During mean follow-up of 49 months, the mean THI reduction was 24 +/- 26 points (P = 0.02). Treatment was regarded successful in 6 patients (60%). In these patients, tinnitus did not disappear, but transformed into a more bearable sound. In 4 patients, transient complications occurred, and 1 patient experienced permanent vertigo postoperatively. Furthermore, hearing deterioration was a result of implantation in 86% of the patients.CONCLUSIONS: Direct neurostimulation resulted in treatment success in a small majority of the patients, with a significant decrease in THI score. However, because of a high risk of additional hearing damage, this technique seems not viable for patients with moderate hearing loss.

BACKGROUND: In deep brain stimulation (DBS), accurate placement of the lead is critical. Target definition is highly dependent on visual recognition on magnetic resonance imaging (MRI). We prospectively investigated whether the 7T MRI enabled better visualization of targets and led to better placement of leads compared with the 1.5-T and the 3-T MRI.METHODS: Three patients with PD (mean, 55 years) were scanned on 1.5-, 3-, and 7-T MRI before surgery. Tissue contrast and signal-to-noise ratio were measured. Target coordinates were noted on MRI and during surgery. Differences were analyzed with post-hoc analysis of variance.RESULTS: The 7-T MRI demonstrated a significant improvement in tissue visualization (P CONCLUSIONS: Although the 7-T MRI enables a significant better visualization of the DBS target in patients with PD, we found no clinical benefit for the placement of the DBS leads.

Object Chronic sinusitis can be a debilitating disease with significant impact on quality of life. Frontal sinusitis has a relatively low prevalence, but complications can be severe due to its anatomical location. After failure of conservative measures, typically endoscopic procedures are performed to improve the drainage of the frontal sinus. The cranialization of the frontal sinus is the final surgical measure, in which the affected frontal sinus is truly removed. In this study the authors describe the surgical technique of cranialization of the frontal sinus for refractory chronic frontal sinusitis, systematically search the literature for its application, and assess patient satisfaction in a cohort of consecutively treated patients after long-term follow-up. Methods A consecutive cohort of 15 patients with refractory chronic frontal sinusitis was treated by cranialization of the frontal sinus and followed over a 20-year period (1989–2008) for the direct results and complications of the surgery. Long-term follow-up (mean 6.5 years) was obtained to assess the long-term effects of the cranialization. Results In all patients the signs and symptoms of chronic frontal sinusitis responded very well to the cranialization. Five patients had surgical complications, of which 2 were serious. One patient died of an unrelated cause and 1 patient was lost to follow-up. The remaining 13 patients had a long-term follow-up, which revealed that 12 of them thought that their life was better after the surgical procedure. Conclusions Cranialization of the frontal sinus deserves consideration as the final remedy for refractory chronic frontal sinusitis after definite failure of other options.

Objective In recent years the endovascular treatment of intracranial aneurysms (coiling) has progressively gained recognition, particularly after the publication of the International Subarachnoid Aneurysm Trial (ISAT) in 2002. Despite the fact that in ISAT middle cerebral artery (MCA) aneurysms were clearly underrepresented, the study is often used as an argument to favor coiling above surgery in MCA aneurysms. Taken into account that MCA aneurysms are very well accessible for surgery, a contemporary assessment of the benefits of a preferred surgical strategy for MCA aneurysms was performed in a tertiary neurovascular referral center. Methods A prospectively kept single-center database of 151 consecutive patients with an MCA aneurysm was reviewed over a 6-year period (2001–2006). Long-term follow-up after surgical treatment of a ruptured MCA aneurysm was obtained in 74 out of 77 (96%) patients. The outcome was compared with relevant series in the literature. Results After a mean follow-up of 4.7 years, 59 out of 74 surgically treated patients (80%) with a ruptured MCA aneurysm had a good outcome (mRankin 0–2). All patients with an unruptured MCA aneurysm also had a good outcome after clipping. This is well-matched with the findings of the literature search, and competitive with the endovascular results. Conclusion Surgical clipping is recommended as the principal treatment strategy for MCA aneurysms. This is not only ethically defendable in view of the surgical results but also in line with a strategy to maintain surgical experience within centralized neurovascular centers.

The development of an intracranial aneurysm (IA) is a multifactorial process, involving genetic and environmental factors. The presence of IA or aneurysmal subarachnoid hemorrhage (aSAH) in twins is particularly interesting, since both genetic and environmental factors can be studied. It also raises the question of whether, when one twin is affected, the other asymptomatic twin should be examined for an IA. We report on a monozygotic (MZ) twin-pair with aSAH in both twins and we review all reported cases of IA in MZ twins. Including our case, we found only 14 MZ twin-pairs in which both twins harbored an IA, suggesting a heavy underreporting in the medical literature. In this small group, a high concordance was noted in the sites of IAs. In MZ twins, the preferred sites for IAs are the branching arteries, while aneurysms arising from fusion arteries are rare. These sites differ from the preferential sites seen in series of familial IAs and series of sporadic IAs. We therefore hypothesize that the twinning process might play a significant role in the development of IAs in MZ twins. To further explore and substantiate this, the large twin registries should be studied. Although IAs in MZ twins with a negative family history for IAs should not be regarded as familial IAs, screening of the asymptomatic twin should be seriously considered if one MZ twin presents with an aSAH or an IA, because of the high fatality rates reported in asymptomatic (and not screened) MZ twin-halves.

Post-transplant lymphoproliferative disorder (PTLD) is a serious complication after solid-organ transplantation. We report a lung transplant recipient presenting with lower limb weakness as a result of extradural cord compression from PTLD. Diagnosis was made by laminectomy of T-3 with partial removal of the epidural mass. Further treatment consisted of chemoradiotherapy. The patient recovered completely. To our knowledge, this is the first reported case of PTLD presenting with signs and symptoms of spinal cord compression. The differential diagnosis of spinal cord compression in a patient who has had a transplant should include primary presentation of PTLD.

Object Dural arteriovenous fistulas (DAVFs) are distinct neurovascular entities. Although their exact origins are unknown, venous thrombosis and venous hypertension are likely to be major inducing factors. To address the relationship between DAVFs and thrombophilic factors, the authors conducted a case-control study at a single institution and performed a metaanalysis of the literature. Methods Forty patients with DAVFs at Toronto Western Hospital were recruited to complete a questionnaire and to donate blood samples for factor V Leiden mutation and factor II G20210A mutation screening and assessment of coagulation factors. The questionnaire was designed to collect information on each participant's specific history of venous thrombosis, medications, and race. A control group of 33 healthy volunteers agreed to the same protocol. A MEDLINE search of the literature from 1966 to the present was conducted and three relevant series were found. The results of the present study were pooled with the data from the literature. Results Combining institutional results with the results from the literature yielded a total of 121 patients and 178 control group members. Thrombophilic mutations were present in 16 patients and four healthy volunteers, with an odds ratio (OR) of 4.69 for factor V Leiden (95% confidence interval [CI] 1.24–17.69) and an OR of 10.87 for the prothrombin G20210A allele (95% CI 1.32–89.51). Levels of the basic coagulation profile, fibrinogen, and factor VIII were within normal limits. Conclusions Patients with the factor V Leiden and factor II G20210A mutations are at a higher risk for DAVFs. However, because these mutations are not implicated in the vast majority of DAVFs, routine screening is not recommended.

Object. In the present study the authors compared the incidence and risk factors for external drainage—related bacterial meningitis (ED-BM) by using ventricular and lumbar catheters. Methods. A cohort of 230 consecutive patients with ED was evaluated. Cerebrospinal fluid samples were obtained daily for microbiological culture, and ED-BM was defined based on culture results in combination with clinical symptoms. The incidence of ED-BM was 7% in lumbar and 15% in ventricular drains. Independent risk factors included site leakage, drain blockage, and most importantly duration of ED. Despite a higher infection rate, ventricular catheters did not have a significant higher risk of infection after correcting for duration of drainage. Conclusions. Analysis of data in the present study showed that the incidence of ED-associated death is low (0.45%) in patients who do not receive continuous antibiotic prophylaxis during ED.