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Introduction Evidence regarding the impact of prophylactic implantation of Extracorporeal Membrane Oxygenation (ECMO) during coronary or structural procedures is limited. The purpose of this paper is to evaluate the in-hospital and medium term outcomes of ECMO support in complex percutaneous coronary or structural intervention. Methods The present is an observational prospective study including consecutive patients who underwent to prophylactic ECMO implantation for percutaneous coronary intervention (PCI), structural or combined interventions between July 2018 and July 2020 in Maria Pia Hospital GVM Care & Research, Turin, Italy. Primary endpoints were in-hospital and medium term all-cause death. Secondary endpoints were vascular complication, bleeding and procedural success. Results 27 patients were included with a mean age of 80 ± 6 years, 10 (37%) being diabetics and 19 (70%) with severe left ventricle dysfunction. Mean Logistic Euroscore was 28.7 ± 18.7. Seven patients (26%) underwent complex coronary revascularization, 7 (26%) combined PCI + TAVI, 5 (19%) combined PCI + Mitraclip, 5 (19%) TAVI alone and finally 3 (11%) combined TAVI + Mitraclip. The procedural success was 96%, with only one in-hospital death due to major vascular complication at the ECMO vascular access. At a mean follow-up of 11 ± 6.8 months 4 deaths were recorded (3 from not cardiovascular causes). Conclusions Prophylactic use of ECMO may be beneficial for high risk patients undergoing complex combined coronary/structural percutaneous interventions with good in-hospital and mid-term outcomes in term of safety and procedural success.

Background and Purpose— We investigated whether subarachnoid extension (SAHE) of intracerebral hemorrhage (ICH) is associated with hematoma expansion (HE). Methods— Retrospective analysis of patients with primary spontaneous ICH admitted at 3 academic hospitals in Italy. The study population was divided into a development and a replication cohort. SAHE was rated on baseline noncontrast computed tomography by investigators blinded to clinical data. The main outcome of interest was HE, defined as ICH growth >33% mL and/or >6 mL. Predictors of HE were explored with multivariable logistic regression stratified by ICH location (lobar versus nonlobar). Results— A total of 360 and 192 patients were included in the development and replication cohort, respectively. SAHE was identified with good interrater reliability ( K =0.82), and its frequency was 27.8% in the development and 24.5% in the replication cohort. In univariate analysis, HE was more common in patients with SAHE (52.0% versus 27.3%; P P =0.001) whereas there was no association with HE in nonlobar ICH (odds ratio, 0.55 [95% CI, 0.17–1.84]; P =0.334). The increased risk of HE in lobar ICH with SAHE was confirmed in the replication cohort (odds ratio, 3.46 [95% CI, 1.07–11.20]; P =0.038). Conclusions— SAHE predicts HE in lobar ICH. This may improve the stratification of HE risk in clinical practice or future trials targeting HE. Further research is needed to confirm our findings and characterize the underlying biological mechanisms.

Advanced MRI findings in two patients with probable chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids (CLIPPERS) are presented. Diffusion tensor imaging indices (fractional anisotropy and mean diffusivity), evaluated in both patients at baseline MRI examination before treatment and during follow-up, indicated white matter structural changes not only affecting the brainstem, which represents the primary site of inflammatory damage, but also projection (corona radiata) and associative tracts in both patients, while alterations within the corpus callosum were detected in patient 1# at follow-up. Susceptibility weighted imaging (SWI) revealed hypointense lesions in both patients, MRI spectroscopy (MRS) indicated a mildly increased Cho/NAA ratio with no evidence of lipids/lactate peaks, indicating that it may be used as a non-invasive marker to identify CLIPPERS cases suspected for progression to lymphoma.

Introduction To describe clinical, neuroimaging, and laboratory features of a large cohort of Italian patients with reversible cerebral vasoconstriction syndrome. Methods In the setting of the multicenter Italian Project on Stroke at Young Age (IPSYS), we retrospectively enrolled patients with a diagnosis of definite reversible cerebral vasoconstriction syndrome according to the International Classification of Headache Disorders (ICHD)-3 beta criteria (6.7.3 Headache attributed to reversible cerebral vasoconstriction syndrome, imaging-proven). Clinical manifestations, neuroimaging, treatment, and clinical outcomes were evaluated in all patients. Characteristics of reversible cerebral vasoconstriction syndrome without typical causes (“idiopathic reversible cerebral vasoconstriction syndrome”) were compared with those of reversible cerebral vasoconstriction syndrome related to putative causative factors (“secondary reversible cerebral vasoconstriction syndrome”). Results A total of 102 patients (mean age, 47.2 ± 13.9 years; females, 85 [83.3%]) qualified for the analysis. Thunderclap headache at presentation was reported in 69 (67.6%) patients, and it typically recurred in 42 (60.9%). Compared to reversible cerebral vasoconstriction syndrome cases related to putative etiologic conditions (n = 21 [20.6%]), patients with idiopathic reversible cerebral vasoconstriction syndrome (n = 81 [79.4%]) were significantly older (49.2 ± 13.9 vs. 39.5 ± 11.4 years), had more frequently typical thunderclap headache (77.8% vs. 28.6%) and less frequently neurological complications (epileptic seizures, 11.1% vs. 38.1%; cerebral infarction, 6.1% vs. 33.3%), as well as concomitant reversible brain edema (25.9% vs. 47.6%). Conclusions Clinical manifestations and putative etiologies of reversible cerebral vasoconstriction syndrome in our series are slightly different from those observed in previous cohorts. This variability might be partly related to the coexistence of precipitating conditions with a putative etiologic role on disease occurrence.

Objective Cerebral venous thrombosis (CVT) is an uncommon form of stroke affecting mostly young individuals. Although genetic factors are thought to play a role in this cerebrovascular condition, its genetic etiology is not well understood. Methods A genome-wide association study was performed to identify genetic variants influencing susceptibility to CVT. A 2-stage genome-wide study was undertaken in 882 Europeans diagnosed with CVT and 1,205 ethnicity-matched control subjects divided into discovery and independent replication datasets. Results In the overall case-control cohort, we identified highly significant associations with 37 single nucleotide polymorphisms (SNPs) within the 9q34.2 region. The strongest association was with rs8176645 (combined p = 9.15 x 10(-24); odds ratio [OR] = 2.01, 95% confidence interval [CI] = 1.76-2.31). The discovery set findings were validated across an independent European cohort. Genetic risk score for this 9q34.2 region increases CVT risk by a pooled estimate OR = 2.65 (95% CI = 2.21-3.20, p = 2.00 x 10(-16)). SNPs within this region were in strong linkage disequilibrium (LD) with coding regions of the ABO gene. The ABO blood group was determined using allele combination of SNPs rs8176746 and rs8176645. Blood groups A, B, or AB, were at 2.85 times (95% CI = 2.32-3.52, p = 2.00 x 10(-16)) increased risk of CVT compared with individuals with blood group O. Interpretation We present the first chromosomal region to robustly associate with a genetic susceptibility to CVT. This region more than doubles the likelihood of CVT, a risk greater than any previously identified thrombophilia genetic risk marker. That the identified variant is in strong LD with the coding region of the ABO gene with differences in blood group prevalence provides important new insights into the pathophysiology of CVT. ANN NEUROL 2021

ObjectiveTo investigate the prevalence, predictors, and prognostic effect of hematoma expansion (HE) in patients with intracerebral hemorrhage (ICH) with unclear symptom onset (USO).MethodsWe performed a retrospective analysis of patients with primary spontaneous ICH admitted at 5 academic medical centers in the United States and Italy. HE (volume increase >6 mL or >33% from baseline to follow-up noncontrast CT [NCCT]) and mortality at 30 days were the outcomes of interest. Baseline NCCT was also analyzed for presence of hypodensities (any hypodense region within the hematoma margins). Predictors of HE and mortality were explored with multivariable logistic regression.ResultsWe enrolled 2,165 participants, 1,022 in the development cohort and 1,143 in the replication cohort, of whom 352 (34.4%) and 407 (35.6%) had ICH with USO, respectively. When compared with participants having a clear symptom onset, patients with USO had a similar frequency of HE (25.0% vs 21.9%, p = 0.269 and 29.9% vs 31.5%, p = 0.423). Among patients with USO, HE was independently associated with mortality after adjustment for confounders (odds ratio [OR] 2.64, 95% confidence interval [CI] 1.43–4.89, p = 0.002). This finding was similar in the replication cohort (OR 3.46, 95% CI 1.86–6.44, p < 0.001). The presence of NCCT hypodensities in patients with USO was an independent predictor of HE in the development (OR 2.59, 95% CI 1.27–5.28, p = 0.009) and replication (OR 2.43, 95% CI 1.42–4.17, p = 0.001) population.ConclusionHE is common in patients with USO and independently associated with worse outcome. These findings suggest that patients with USO may be enrolled in clinical trials of medical treatments targeting HE.

Objective: Intradural Extramedullary (IDEM) tumors are usually treated with surgical excision. The aim of this study was to investigate the impact on clinical outcomes of pre-surgical clinical conditions, intraoperative neurophysiological monitoring (IONM), surgical access to the spinal canal, histology, degree of resection and intra/postoperative complications.Methods: This is a retrospective observational study analyzing data of patients suffering from IDEM tumors who underwent surgical treatment over a 12 year period in a double-center experience. Data were extracted from a prospectively maintained database and included: sex, age at diagnosis, clinical status according to the modified McCormick Scale (Grades I-V) at admission, discharge, and follow-up, tumor histology, type of surgical access to the spinal canal (bilateral laminectomy vs. monolateral laminectomy vs. laminoplasty), degree of surgical removal, use and type of IONM, occurrence and type of intraoperative complications, use of Ultrasonic Aspirator (CUSA), radiological follow-up.Results: A total number of 249 patients was included with a mean follow-up of 48.3 months. Gross total resection was achieved in 210 patients (84.3%) mostly in Schwannomas (45.2%) and Meningiomas (40.4%). IONM was performed in 162 procedures (65%) and D-wave was recorded in 64.2% of all cervical and thoracic locations (99 patients). The linear regression diagram for McCormick grades before and after surgery (follow-up) showed a correlation between preoperative and postoperative clinical status. A statistically significant correlation was found between absence of worsening of clinical condition at follow-up and use of IONM at follow-up (p = 0.01) but not at discharge. No associations were found between the choice of surgical approach and the extent of resection (p = 0.79), the presence of recurrence or residual tumor (p = 0.14) or CSF leakage (p = 0.25). The extent of resection was not associated with the use of IONM (p = 0.91) or CUSA (p = 0.19).Conclusion: A reliable prediction of clinical improvement could be made based on pre-operative clinical status. The use of IONM resulted in better clinical outcomes at follow-up (not at discharge), but no associations were found with the extent of resection. The use of minimally invasive approaches such as monolateral laminectomy showed to be effective and not associated with worse outcomes or increased complications.

Visceral pain, particularly chronic pain, is a significant problem for the physical, psychological, social, and economic aspects.

Bilateral absence of N20 peak in median nerve Somatosensory Evoked Potentials (SSEPs) is considered the most valid predictor of poor outcome in comatose survivors after cardiopulmonary resuscitation. We investigated the consistency in interpreting SSEP recordings in a multicentre study.44 SSEP recordings randomly extracted from 600 recordings of 392 patients included in the "Prognostication of Neurological outcome after Cardiac Arrest (ProNeCa) study" were blindly read by three expert neurophysiologists. Agreement between raters, and individual agreement of each rater vs. reference standard (RS), were calculated using Kappa Coefficients. Inter-rater reliability was calculated with Intra-class Correlation Coefficient (ICC).When raters had to evaluate the presence of N20 with normal amplitude, the inter-rater agreement was very high (Kappa = 0.84). In the case of N20 absence the agreement was good (Kappa = 0.66), but when N20 amplitude was low, the agreement decreased to moderate (Kappa = 0.579) becoming even weaker when it was "Non Assessable" (Kappa = 0.107). The agreement of each rater with the RS had a range from moderate to very good; rater1 Kappa = 0.589 (95%CI 0.397-0.781; p 0.001), rater2 Kappa = 0.644 (95%CI 0.460-0.828; p 0.001), rater3 Kappa = 0.859 (95%CI 0.698-1.000; p 0.001). The ICC was barely good, 0.682 (95%CI 0.539-0.798; p = 0.0075).Different health professionals, using different equipment in a multicentre study, had very good inter-rater agreement in interpreting SSEP records. The interpretation of "Non Assessable" SEPPs, mainly in relation to noise level, is still a crucial issue because it increases rater uncertainty. For this reason, it is important to focus on improving recording quality and interpretation of records.

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The BAT, BRAIN, and HEP scores have been proposed to predict hematoma expansion (HE) with noncontrast computed tomography (NCCT). We sought to validate these tools and compare their diagnostic performance. We retrospectively analyzed two cohorts of patients with primary intracerebral hemorrhage. HE expansion was defined as volume growth > 33% or > 6 mL. Two raters analyzed NCCT scans and calculated the scores, blinded to clinical and imaging data. The inter-rater reliability was assessed with the interclass correlation statistic. Discrimination and calibration were calculated with area under the curve (AUC) and Hosmer–Lemeshow χ2 statistic, respectively. AUC comparison between different scores was explored with DeLong test. We also calculated the sensitivity, specificity, positive, and negative predictive values of the dichotomized scores with cutoffs identified with the Youden’s index. A total of 230 subjects were included, of whom 86 (37.4%) experienced HE. The observed AUC for HE were 0.696 for BAT, 0.700 for BRAIN, and 0.648 for HEP. None of the scores had a significantly superior AUC compared with the others (all p > 0.4). All the scores had good calibration (all p > 0.3) and good-to-excellent inter-rater reliability (interclass correlation > 0.8). BAT ≥ 3 showed the highest specificity (0.81), whereas BRAIN ≥ 6 had the highest sensitivity (0.76). The BAT, BRAIN, and HEP scores can predict HE with acceptable discrimination and require just a baseline NCCT scan. These tools may be used to stratify the risk of HE in clinical practice or randomized controlled trials.

Background Infective endocarditis (IE) can be complicated by intracranial bleeding (ICB) caused by different pathologic mechanisms. The occurrence of ICB in patients with IE significantly influences therapeutic decisions and has a negative impact on outcome. Case Report We describe the clinical courses of 3 patients with aortic prosthetic valve IE presenting with ICB. Patients 1 and 2 experienced subarachnoid hemorrhage (SAH) and intracerebral hemorrhage (ICH), respectively, caused by rupture of an intracranial infectious aneurysm (IIA). Both underwent endovascular treatment of IIA with good outcome. In patient 3, ICB was the hemorrhagic conversion of an acute ischemic lesion from septic brain embolization. In the subacute phase of the disease, aortic valve replacement was performed, with excellent outcome. Why Should an Emergency Physician be Aware of This? ICB is a relevant complication and sometimes the first clinical feature of IE. Imaging of brain vessels should be performed to investigate the pathologic mechanism underlying ICB. The prevalence of IIA is probably underestimated and may influence the therapeutic strategy. Cerebrovascular imaging may therefore also be considered in asymptomatic subjects with left-sided IE. Withdrawal of anticoagulant treatment and delay of cardiac surgery are recommended in all cases of IE complicated by ICB. Because of the impact of ICB on IE management and outcome, a high level of clinical suspicion and prompt recognition and treatment of this complication are necessary.

Objective Although a concern exists that 3-hydroxy-3-methylglutaryl coenzyme A reductase inhibitors (statins) might increase the risk of intracerebral haemorrhage (ICH), the contribution of these agents to the relationship between serum cholesterol and disease occurrence has been poorly investigated. Methods We compared consecutive patients having ICH with age and sex-matched stroke-free control subjects in a case–control analysis, as part of the Multicenter Study on Cerebral Haemorrhage in Italy (MUCH-Italy), and tested the presence of interaction effects between total serum cholesterol levels and statins on the risk of ICH. Results A total of 3492 cases (mean age, 73.0±12.7 years; males, 56.6%) and 3492 control subjects were enrolled. Increasing total serum cholesterol levels were confirmed to be inversely associated with ICH. We observed a statistical interaction between total serum cholesterol levels and statin use for the risk of haemorrhage (Interaction OR (IOR), 1.09; 95% CI 1.05 to 1.12). Increasing levels of total serum cholesterol were associated with a decreased risk of ICH within statin strata (average OR, 0.87; 95% CI 0.86 to 0.88 for every increase of 0.26 mmol/l of total serum cholesterol concentrations), while statin use was associated with an increased risk (OR, 1.54; 95% CI 1.31 to 1.81 of the average level of total serum cholesterol). The protective effect of serum cholesterol against ICH was reduced by statins in strictly lobar brain regions more than in non-lobar ones. Conclusions Statin therapy and total serum cholesterol levels exhibit interaction effects towards the risk of ICH. The magnitude of such effects appears higher in lobar brain regions.

Cerebral venous thrombosis is associated with intracranial hemorrhage. The occurrence of brain bleeding complicates management and is associated with worse outcomes. We aimed to identify clinical and imaging risk factors for intracerebral hemorrhage. We hypothesized that higher clot burden would be associated with a higher risk of intracerebral hemorrhage. Methods: We performed a retrospective analysis of an international, multicenter cohort of patients with cerebral venous thrombosis who underwent CT within 2 weeks of symptom onset and who had a subsequent confirmed diagnosis of CVT on MRV, CTV or DSA. Clinical and imaging features were compared between patients with and without intracranial hemorrhage. Clot burden was assessed by counting the number of thrombosed venous sinuses and veins on confirmatory imaging. Results: We recruited 260 patients with cerebral venous thrombosis from 10 institutions in Europe and Mexico. The mean age was 42 and 74% were female. Intracranial hemorrhage was found in 102 (39%). Intracerebral hemorrhage occurred in 64 (63%), small juxtacortical hemorrhage in 30 (29%), subarachnoid hemorrhage in 24 (24%) and subdural hemorrhage in 11 (11%). Combinations of these types of hemorrhage occurred in 23 (23%). Intraparenchymal hemorrhages in multiple locations occurred in 6 (6%). Older age, presence of hematological disease and superior sagittal thrombosis involvement were significantly associated with presence of hemorrhage. The number of thrombosed venous sinuses was not associated with intracerebral hemorrhage (average number of sinuses/veins involved with hemorrhage 2.1 versus 1.9 without hemorrhage, p=0.4). Conclusion: The high rate of intracranial hemorrhage in cerebral venous thrombosis is not explained by widespread involvement of the venous sinuses. Superior sagittal sinus involvement is associated with the presence of intracranial hemorrhage.

[Objective] To assess the added diagnostic value of semiquantitative imaging markers on noncontrast CT scans in cerebral venous thrombosis (CVT)., [Methods] In a retrospective, multicenter, blinded, case-control study of patients with recent onset (, [Results] We enrolled 285 patients with CVT and 303 controls from 10 international centers. Sensitivity of visual impression of thrombosis ranged from 41% to 73% and specificity ranged from 97% to 100%. Attenuation measurement had an area under the curve (AUC) of 0.78 (95% confidence interval [CI] 0.74–0.81). After adjustment for hematocrit, the AUC remained 0.78 (95% CI 0.74–0.81). The analysis of attenuation ratios of affected vs unaffected sinuses had AUC of 0.83 (95% CI 0.8–0.86). Adding this imaging marker significantly improved discrimination, but sensitivity when tolerating a false-positive rate of 20% was not higher than 76% (95% CI 0.70–0.81)., [Conclusion] Semiquantitative analysis of attenuation values for diagnosis of CVT increased sensitivity but still failed to identify 1 out of 4 CVT., [Classification of evidence] This study provides Class II evidence that visual analysis of plain CT with or without attenuation measurements has high specificity but only moderate sensitivity for CVT.

Objective Intraoperative electrical stimulation of the spinal cord evokes not only high amplitude cortical somatosensory evoked potentials (ScEPs: spinal cord-evoked potentials) recorded from the scalp but also early potentials. It has been postulated that the early potential recorded from the scalp could be generated by antidromic stimulation of the cortico-spinal tract (ACSP: Antidromic Corticospinal tract Potential or “anti D-wave”). In this study, we aimed to investigate the anti D-wave evoked by epidural stimulation in a population of neurologically uncompromised/compromised patients during spine and spinal cord surgery. To better define its origin, we examined its spatial distribution, response to stimulation modification and recording parameters, and finally, we correlated anti D-waves and corresponding epidural recorded D-waves after a transcranial electric stimulation at the same level of spinal cord stimulation. Methods Tibial nerve somatosensory evoked potentials (SEPs), transcranially elicited muscle motor evoked potentials (m-MEPs), epidural motor evoked potentials (D-wave), and cortical spinal cord-evoked potentials (ScEPs) as well as Antidromic Cortico Spinal tract Potential (anti D-wave) were intraoperatively recorded in 30 subjects with different degrees of neurological involvement. ScEPs and anti D-wave were evoked by epidural stimulation of the spinal cord, both cranially and caudally to the surgery site and were recorded over the scalp at the midline. The effects of the stimulus rate and high pass filter were also tested. Results The anti D-wave was recordable in all neurologically intact patients and was clearly isolated from the ScEPs by its very short latency; recordings of the anti D-wave were limited to the anterior midline, and its amplitude was only slightly reduced by increasing the stimulus rate or by changing the high pass filter, and its latency was slightly longer than that of the D-wave latency. In neurologically compromised patients, the anti D-wave and D-wave exhibit a similar behaviour, both of which were present in neurologically intact or moderately compromised patients and absent in patients with quadri/paraplegia. In a patient with paraplegia due to T8 meningioma and in neurologically intact patients in whom the cauda/conus stimulated the anti D-wave, cortical ScEPs were absent when the stimulation was performed caudally to the surgical site. Conclusions We provide evidence that the anti D-wave behaviour and its parameters have a close correlation with the behaviour of the D-wave; specifically, its distribution, response to filtering, stimulus rate, and absence in paraplegic patients. Significance The presented data demonstrate that the anti D-wave is generated by the antidromic stimulation of fast neurons of the corticospinal tract, and consistent findings have been previously published in animals.

Whether to resume antithrombotic treatment after oral anticoagulant–related intracerebral haemorrhage (OAC-ICH) is debatable. In this study, we aimed at investigating long-term outcome associated with OAC resumption after warfarin-related ICH, in comparison with secondary prevention strategies with platelet inhibitors or antithrombotic discontinuation. Participants were patients who sustained an incident ICH during warfarin treatment (2002–2014) included in the Multicenter Study on Cerebral Hemorrhage in Italy. Primary end-point was a composite of ischemic stroke/systemic embolism (SE) and all-cause mortality. Secondary end-points were ischemic stroke/SE, all-cause mortality and major recurrent bleeding. We computed individual propensity score (PS) as the probability that a patient resumes OACs or other agents given his pre-treatment variables, and performed Cox multivariable analysis using Inverse Probability of Treatment Weighting (IPTW) procedure. A total of 244 patients qualified for the analysis. Unlike antiplatelet agents, OAC resumption was associated with a lower rate of the primary end-point (weighted hazard ratio [HR], 0.21; 95% confidence interval [CI], 0.09–0.45), as well as of overall mortality (weighted HR, 0.17; 95% CI, 0.06–0.45) and ischemic stroke/SE (weighted HR, 0.19; 95% CI, 0.06–0.60) with no significant increase of major bleeding in comparison with patients receiving no antithrombotics. In the subgroup of patients with atrial fibrillation, OACs resumption was also associated with a reduction of the primary end-point (weighted HR, 0.22; 95% CI, 0.09–0.54), and the secondary end-point ischemic stroke/SE (weighted HR, 0.09; 95% CI, 0.02–0.40). In conclusion, in patients who have an ICH while receiving warfarin, resuming anticoagulation results in a favorable trade-off between bleeding susceptibility and thromboembolic risk.

Although sparse observational studies have suggested a link between migraine and cervical artery dissection (CEAD), any association between the 2 disorders is still unconfirmed. This lack of a definitive conclusion might have implications in understanding the pathogenesis of both conditions and the complex relationship between migraine and ischemic stroke (IS).To investigate whether a history of migraine and its subtypes is associated with the occurrence of CEAD.A prospective cohort study of consecutive patients aged 18 to 45 years with first-ever acute ischemic stroke enrolled in the multicenter Italian Project on Stroke in Young Adults was conducted between January 1, 2000, and June 30, 2015. In a case-control design, the study assessed whether the frequency of migraine and its subtypes (presence or absence of an aura) differs between patients whose IS was due to CEAD (CEAD IS) and those whose IS was due to a cause other than CEAD (non-CEAD IS) and compared the characteristics of patients with CEAD IS with and without migraine.Frequency of migraine and its subtypes in patients with CEAD IS vs non-CEAD IS.Of the 2485 patients (mean [SD] age, 36.8 [7.1] years; women, 1163 [46.8%]) included in the registry, 334 (13.4%) had CEAD IS and 2151 (86.6%) had non-CEAD IS. Migraine was more common in the CEAD IS group (103 [30.8%] vs 525 [24.4%], P = .01), and the difference was mainly due to migraine without aura (80 [24.0%] vs 335 [15.6%], P .001). Compared with migraine with aura, migraine without aura was independently associated with CEAD IS (OR, 1.74; 95% CI, 1.30-2.33). The strength of this association was higher in men (OR, 1.99; 95% CI, 1.31-3.04) and in patients 39.0 years or younger (OR, 1.82; 95% CI, 1.22-2.71). The risk factor profile was similar in migrainous and non-migrainous patients with CEAD IS (eg, hypertension, 20 [19.4%] vs 57 [24.7%], P = .29; diabetes, 1 [1.0%] vs 3 [1.3%], P .99).In patients with IS aged 18 to 45 years, migraine, especially migraine without aura, is consistently associated with CEAD. This finding suggests common features and warrants further analyses to elucidate the underlying biologic mechanisms.

Background and Purpose: The aim of this study was to investigate for a possible association between both prestroke CHA(2)DS(2)-VASc score and the severity of stroke at presentation, as well as disability and mortality at 90 days, in patients with acute stroke and atrial fibrillation (AF). Methods: This prospective study enrolled consecutive patients with acute ischemic stroke, AF, and assessment of prestroke CHA2DS2-VASc score. Severity of stroke was assessed on admission using the National Institutes of Health Stroke Scale (NIHSS) score (severe stroke: NIHSS >= 10). Disability and mortality at 90 days were assessed by the modified Rankin Scale (mRS = 3). Multiple logistic regression was used to correlate prestroke CHA(2)DS(2)-VASc and severity of stroke, as well as disability and mortality at 90 days. Results: Of the 1020 patients included in the analysis, 606 patients had an admission NIHSS score lower and 414 patients higher than 10. At 90 days, 510 patients had mRS >= 3. A linear correlation was found between the prestroke CHA(2)DS(2)-VASc score and severity of stroke (P = .001). On multivariate analysis, CHA(2)DS(2)-VASc score correlated with severity of stroke (P = .041) and adverse functional outcome (mRS = 3) (P = .001). A logistic regression with the receiver operating characteristic graph procedure (C-statistics) evidenced an area under the curve of .60 (P = .0001) for severe stroke. Furthermore, a correlation was found between prestroke CHA(2)DS(2)-VASc score and lesion size. Conclusions: In patients with AF, in addition to the risk of stroke, a high CHA(2)DS(2)-VASc score was independently associated with both stroke severity at onset and disability and mortality at 90 days.

The aim of this study was to test the hypothesis that patients with spontaneous cervical artery dissection (CeAD) have increased arterial tortuosity, and the objective quantification of such a tortuosity may aid in the identification of subjects at increased risk of disease. In the setting of a hospital-based, case-control study, we used the vertebral tortuosity index (VTI) measured on magnetic resonance angiography, a validated method for the assessment and quantification of arterial tortuosity, to compare the degree of tortuosity in a series of consecutive patients with spontaneous CeAD and of age- and sex-matched patients with ischemic stroke unrelated to CeAD (non-CeAD IS) and stroke-free subjects. The study group was composed of 102 patients with CeAD (mean age, 44.5 ± 7.8 years; 66.7% men), 102 with non-CEAD IS, and 102 stroke-free subjects. The VTI was higher in the group of patients with CeAD (median, 7.3; 25th–75th percentile, 10.2) compared with that of non-CeAD IS (median, 3.4; 25th–75th percentile, 4.4) and of stroke-free subjects (median, 4.0; 25th–75th percentile, 2.9; p ≤ 0.001), and was independently associated to the risk of CeAD (OR, 1.18; 95% CI, 1.09–1.29) in multivariable regression analysis. The degree of tortuosity also tended to be higher in CeAD patients who experienced short-term recurrence (5.8%; median, 20.2; 25th–75th percentile, 31.2) than in those without recurrent events (median, 7.2; 25th–75th percentile, 9.4; p = 0.074). CeAD patients exhibit increased arterial tortuosity. This might have potential implications for better understanding of the pathophysiology of the disease as well as clinical utility in evaluation, prognostication, and decision-making of affected individuals.

The combined recordings of epidural-(D wave) and muscle motor evoked potentials (m-MEPs) have been proposed in many studies in intramedullary spinal cord tumour (IMSCT) surgery, although not all agree. Furthermore, the usefulness of the intraoperative monitoring of motor systems using these methods in other types of spine surgery has not yet been clearly confirmed. The aim of this study is to test the impact of intraoperative D wave on the monitorability and motor outcome in spine surgery.Intraoperative recording of posterior tibial nerve somatosensory potentials, lower limb m-MEPs (LLm-MEPs) and epidurally recorded D wave caudally to the surgical level was attempted in a total of 103 spine and spinal cord surgeries (23 IMSCT, 55 extramedullary spinal cord tumours and 25 myelopathies).There was a 97.1 %, overall monitorability where at least 1 of the 3 modalities was applicable in 100 surgical procedures. Baseline LLm-MEPs were recorded bilaterally in 85 cases and unilaterally in 11. A caudal D wave was recorded in 97 cases. Transient, or persistent intraoperative modifications occurred in 14/23 IMSCT, 5/55 extramedullary spinal cord tumours and in 2/25 myelopathies. The presence of a persistent stable caudal D wave was predictive of a good motor outcome even when the LL-MEPs were absent and/or when lost during surgery.Not only is intraoperative D wave recording to be considered mandatory in IMSCT surgery but it should also be attempted in other types of spine/spinal cord surgeries.

Heparin-induced thrombocytopenia (HIT) is an adverse effect of heparin therapy which can be responsible for thrombotic events with embolic consequences. Although ischemic stroke is a well known consequence of HIT, few cases of cerebral ischemia of arterial origin have been reported so far. A 38-year-old man was admitted because of acute multiple ischemic strokes and pulmonary embolism which occurred during treatment with low molecular weight heparin as prophylactic therapy for orthopedic surgery. Neuroimaging showed occlusion of the right common carotid artery with multiple acute cerebral infarcts. Testing for anti-platelet factor 4 antibodies confirmed the diagnosis. Systematic review of the literature revealed 55 cases of arterial stroke and three cases of carotid artery occlusion caused by HIT. Although arterial ischemic stroke is a rare complication of HIT, a high level of suspicion and a prompt diagnosis of this coagulation disorder are necessary to avoid life-threatening thromboembolic complications.

The transforming growth factor β (TGFβ) superfamily consists of multipotential secreting cytokines that mediate many key events in normal cellular growth and development, including differentiation, proliferation, motility, organization and death. TGFβs act as ligand for 3 classes of cell surface receptors, the transmembrane serine-threonine kinase receptors, TGFβ receptor type I (TGFβRI) and type 2 (TGFβRII), and TGFβRIII receptors which include an ubiquitous extracellular β-glycan and the membrane glycoprotein endoglin (CD105). Binding of TGFβs to their receptors initiates diverse cellular responses resulting in the phosphorilation of Smad proteins, which then translocate to the nucleus and regulate the transcription of target genes. Perturbation of TGFβ signaling has been implicated in various human disorders including cancer, fibrosis and auto-immune diseases. Recently, mutations in TGFβR1 and TGFβR2 genes have been found in association with a continuum of clinical features with widespread vascular involvement. The extreme of clinical severity is represented by the Loeys-Dietz syndrome (LDS), an autosomal dominant disorder characterized by hypertelorism, bifid uvula, and/or cleft palate, and aggressive arteriopathy causing arterial tortuosity as well as life-threatening complications such as vascular aneurysms and dissections. Elastin disarray, loss of elastic fibre architecture and increased collagen expression in the arterial wall are the pathologic hallmark of LDS. In the present review article we will provide details on the activation of TGFβ cascade, on the clinical features of LDS, as well as on the mechanisms of TGFβ signaling perturbation leading to this condition and the potential role of the antagonism of TGFβ activity in disease management.

Objective To evaluate the prognostic value of EEG and SEPs association in post-anoxic comatose patients at different recording time from cardiac arrest (CA) a italian multicentric study. Methods Comatose patients after CA were included.EEG and SEPs were recorded within 12 h and at 72 h after CA. EEG was classified into “non-continuous” (low voltage, isoelectric, burst-suppression) and “continuous” (other patterns except epileptiform). SEPs were dichotomized into “bilaterally absent” (BA) and “present”. Outcome was evaluated at 6 months by GOS. “Recovery of consciousness” was considered good outcome. Results At the moment four center took part to the study and 230 patients were included. EEG and SEPs were recorded in 92 within 12 h in 102 at 72 h. “Continuous” EEG pattern at 12 h always predicted good outcome, “non-continuous” pattern at 72 h always predicted poor outcome. BA SEPs always predicted poor outcome. Early “continuous” EEG pattern was always associated with present SEPs. Conclusion EEG is a reliable time-dependent predictor of good outcome (within 12 h) and poor outcome (after 72 h). SEP is time-independent predictor of poor outcome. Early “continuous” EEG and BA SEPs are never associated together. Significance The combination of EEG/SEP findings allows prediction of good and poor outcome (within 12 h after CA) and of poor outcome (after 48–72 h). Recording of EEG and SEPs in the same patients allows always an increase in the number of cases correctly classified and an increase of the reliability of prognostication in a single patient due to concordance of patterns.

Hemodynamic responses to noxious stimuli during general anesthesia are regarded as signs of nociception. In this sense, modifications of intraoperative administration of opiates are made on clinical bases. However, recording of nociceptive flexion reflex (NFR), has been proposed as an objective tool in order to assess nociception/antinociception balance. To test the correlation between intraoperative NFR, hemodynamics modifications and range of opiates administration in patients undergoing spinal spinal/spinal cord surgery. The NFR was intraoperatively assessed in 15 patients during TIVA/TCI (10 subjects) or alogenates (5 pts.) anesthesia for spine surgery. In order to elicits NFR, the sural nerve was stimulated at the ankle (train of 5 stimuli, ISI 4 ms, intensity up to 100 mA): responses were recorded from the ipsilateral biceps femoris. In the alogenates group NFR was abolished. NFR threshold correlated with opiates administration changes in 6/10 patients. NFR threshold seems to correlate with nociception/antinociception balance. Further studies are needed in order to test the correlation between intraoperative changes of hemodynamic parameters, the amount of intraoperative opiates administration and postoperative pain.

The laryngeal adductor reflex (LAR) represents the thyroarytenoid muscle response to electrical, mechanical or chemical stimulation of the laryngeal mucosa and is been considered to be a mechanism of laryngeal protection, preventing material from inappropriately entering the upper airway. The intraoperative recording of LAR has been recently proposed in patients undergoing thyroid and cervical spine surgeries. In the last years, intraoperative neurophysiology (IN) has been extensively applied in the surgery of IV ventricle tumors in children. In particular, brainstem mapping (BSM) and corticobulbar tract (CBT) monitoring have demonstrated to be effective in order to reduce the risk of severe post-operative neurologic sequelae. IN techniques (BSM and CBT monitoring) were applied during the removal of a large IV ventricle tumor in a 4 yrs. old child under TIVA/TCI anesthesia. LAR was elicited by electrical stimulation of left or right vocal cord (train of 5 stimuli, 200 μs duration, ISI 2–4 ms, intensity

The optimal management of blood pressure (BP) during acute stroke is controversial. We aimed to investigate whether (1) acute BP has differential impact on clinical outcome of ischemic stroke (IS) and spontaneous intracerebral hemorrhage (ICH), and (2) the magnitude of such an effect varies from the very acute phase to the postacute phase of the two diseases. BP values were automatically recorded at 15-min intervals within the first 48 h in consecutive patients with stroke onset less than 24 h before Stroke Unit admission. Growth mixture models were applied to evaluate the association between binary outcome measures [(1) early neurological deterioration (defined as a ≥4-point increase in 48-h National Institutes of Health Stroke Scale [NIHSS] score), (2) 90-day unfavorable functional status (modified Rankin Scale [mRS] 3-6), and (3) 90-day mortality] and the latent heterogeneity of maximum BP trajectories over time, expressed by two (high/low) BP latent classes within stroke groups. After exclusions, 264 patients (198 IS, 66 ICH) were included. High systolic BP (sBP) class was associated with (1) a direct ~15% increased risk of early neurological deterioration [risk difference (RD), +0.151; 95% confidence interval (CI) +0.039 to +0.263] and ~4% worse 48-h outcome for ICH with respect to IS (RD, +0.038; 95% CI +0.005 to +0.071), (2) a ~28% increased risk of 90-day unfavorable outcome in the group of patients with ICH with respect to IS [IRD = RD(ICH) - RD(IS), +0.289; 95% CI +0.010 to +0.571], and (3) no significant effect on 90-day mortality. The influence of acute BP values on mid-term stroke outcome varies depending on the stroke subtype.

Background/Aims. HIV and hepatitis C virus (HCV) share common modes of transmission, resulting in about 33% incidence of coinfection among people infected with HIV. The survival benefit from highly effective antiretroviral therapy (HAART) for HIV infection is resulting in an increased incidence of hepatocellular carcinoma (HCC) in this population. There are no reports to date regarding the coadministration of HAART and sorafenib for hepatocellular carcinoma. Methods. We report the case of a 42-year-old male patient coinfected with HIV and HCV who developed advanced HCC not amenable to curative therapy. The patient was treated with sorafenib, an oral multikinase inhibitor shown to lead to a longer median survival time and time to progression in patients with advanced HCC. Antiretroviral therapy was continued during sorafenib therapy. Results. The patient achieved a partial tumor response after 3 months and continued to respond at subsequent assessments. His serum α-fetoprotein normalized from 2,172 IU/ml to 2 IU/ml. He had durable stable disease after 23 months of therapy. Antiretroviral therapy was efficacious (CD4+ lymphocyte count, 377/μl; HIV viremia, Conclusions. This is the first report in which sorafenib has been successfully used to treat HCC in a patient with HIV–HCV coinfection.

Amyloid is a term used to describe protein deposits with circumscript physical characteristics: beta-pleated sheet configuration, apple green birefringence under polarized light after Congo red staining, fibrillary structure and high insolubility. Cerebral amyloid angiopathy (CAA) defines a clinicopathological phenomenon characterized by amyloid deposition in the walls of leptomeningeal and cortical arteries, arterioles, and, less often capillaries and veins of the central nervous system. CAAs are currently classified according to the protein deposited including amyloid beta peptide (Abeta), cystatin C (ACys C), prion protein (PrP(Sc)), ABri/ADan, transthyretin (ATTR), and gelsolin (AGel). Most often amyloid deposition occurs in sporadic forms. In less common hereditary forms, a mutated variant protein or precursor protein is abnormally metabolized by proteolytic pathways in consequence of specific gene mutations, and accumulates as amyloid. The spectrum of clinical phenotypes associated with CAA-related vasculopathic changes includes both ischemic and hemorrhagic presentations, primary intracerebral hemorrhage (PICH) being probably the most well-recognized. However, in spite of accumulating data and recent progress in understanding the pathogenesis of CAA-related hemorrhage, the exact mechanisms leading to vessel rupture in these cases are yet to be established. This represents, at present, a major limitation to the identification of reliable biomarkers and the development of disease-specific treatment options. The present paper summarizes epidemiologic and clinical aspects of CAA, and highlights the presumed pathomechanisms of amyloid deposition in both sporadic and hereditary forms.

There is compelling evidence that treatment with 3-hydroxy-3-methylglutaryl coenzyme A (HMG CoA) reductase inhibitors - "statins" - the most important class of lipid lowering agents, reduces ischemic stroke incidence independent on their effect on serum cholesterol levels. In this review, the non-lipid-mediated - "pleiotropic" - effects of statins as well as their potential implication in developing new treatment strategies for stroke prevention will be discussed.

Frontotemporal dementia (FTD) is characterized by executive dysfunctions, behavioral disturbances, language deficits and extrapyramidal symptoms. Frontotemporal lobar degeneration-modified Clinical Dementia Rating Scale (FTLD modified-CDR) has been proposed to measure disease severity in behavioral variant FTD (bvFTD). No tools of global disease severity are available in the other FTLD phenotypes [primary progressive aphasias (PPAs), progressive supranuclear palsy (PSP), and corticobasal syndrome (CBS)]. This would be strategic as outcome measures in clinical trials. To this aim, we evaluated the association between brain volume (voxel based morphometry) and available clinical scales in FTD. In 176 FTD patients (64 bvFTD, 40 PPAs, 32 PSP, 40 CBS), instrumental activities of daily living (ADLs), FTLD-modified CDR, Mini-Mental State Examination (MMSE), Frontal Behavioral Inventory (FBI), and Neuropsychiatry Inventory (NPI) were administered and MRI performed. Whole-brain linear correlation between each clinical rating scale and brain volume was performed. In bvFTD and PPAs, FTLD-modified CDR was associated with regional brain volume, thereby providing evidence for validity of the FTLD-modified CDR. In PSP, none of the clinical indicators were associated with regional brain volume. In CBS, ADLs and MMSE correlated with frontotemporal lower volume. Considering monogenic disease, FTLD-modified CDR was the best measure. In FTD continuum, different measures able to correlate with brain damage should be considered for the different clinical phenotypes or genetic traits.

Background— We sought to compare the benefit of percutaneous closure to that of medical therapy alone for the secondary prevention of embolism in patients with patent foramen ovale (PFO) and otherwise unexplained ischemic stroke, in a propensity scored study. Methods and Results— Between 2000 and 2012, we selected consecutive first-ever ischemic stroke patients aged 18 to 45 years with PFO and no other cause of brain ischemia, as part of the IPSYS registry (Italian Project on Stroke in Young Adults), who underwent either percutaneous PFO closure or medical therapy for comparative analysis. Primary end point was a composite of ischemic stroke, transient ischemic attack, or peripheral embolism. Secondary end point was brain ischemia. Five hundred and twenty-one patients qualified for the analysis. The primary end point occurred in 15 patients treated with percutaneous PFO closure (7.3%) versus 33 patients medically treated (10.5%; hazard ratio, 0.72; 95% confidence interval, 0.39–1.32; P =0.285). The rates of the secondary end point brain ischemia were also similar in the 2 treatment groups (6.3% in the PFO closure group versus 10.2% in the medically treated group; hazard ratio, 0.64; 95% confidence interval, 0.33–1.21; P =0.168). Closure provided a benefit in patients aged 18 to 36 years (hazard ratio, 0.19; 95% confidence interval, 0.04–0.81; P =0.026) and in those with a substantial right-to-left shunt size (hazard ratio, 0.19; 95% confidence interval, 0.05–0.68; P =0.011). Conclusions— PFO closure seems as effective as medical therapy for secondary prevention of cryptogenic ischemic stroke. Whether device treatment might be more effective in selected cases, such as in patients younger than 37 years and in those with a substantial right-to-left shunt size, deserves further investigation.

BACKGROUND AND PURPOSE The impact of body mass index (BMI) on outcome in stroke patients treated with intravenous thrombolysis (IVT) was investigated. METHODS In a multicentre IVT-register-based observational study, BMI with (i) poor 3-month outcome (i.e. modified Rankin Scale scores 3-6), (ii) death and (iii) symptomatic intracranial haemorrhage (sICH) based on criteria of the ECASS II trial was compared. BMI was used as a continuous and categorical variable distinguishing normal weight (reference group 18.5-24.9 kg/m(2) ) from underweight (

Background and Purpose— Although lacunar stroke (LS) and deep intracerebral hemorrhage (dICH) represent acute manifestations of the same pathological process involving cerebral small vessels (small vessel disease), it remains unclear what factors predispose to one phenotype rather than the other at individual level. Methods— Consecutive patients with either acute symptomatic LS or dICH were prospectively enrolled as part of a multicenter Italian study. We compared the risk factor profile of the 2 subgroups using multivariable logistic regression. Results— During a time course of 9.5 years, 1931 subjects (1434 LS and 497 dICH; mean age, 71.3±13.3 years; males, 55.5%) qualified for the analysis. Current smoking was associated with LS (odds ratio [OR], 2.17; P P P =0.001), and more frequently under treatment with warfarin (OR, 2.05; P =0.010) and statins (OR, 3.10; P Conclusions— The risk factor profile of dICH differs from that associated with LS. This might be used for disease risk stratification at individual level.

In 2012, we conducted a retrospective cross-sectional study to assess the number of people living with HIV linked to care and, among these, the number of people on antiretroviral therapy. The health authority in each of the 20 Italian Regions provided the list of Public Infectious Diseases Clinics providing antiretroviral therapy and monitoring people with HIV infection. We asked every Public Infectious Diseases Clinic to report the number of HIV-positive people diagnosed and linked to care and the number of those on antiretroviral therapy during 2012. In 2012, 94,146 people diagnosed with HIV and linked to care were reported. The majority were males (70.1%), Italians (84.4%), and aged between 25 and 49 years (63.4%); the probable route of transmission was heterosexual contact in 37.5% of cases, injecting drug use in 28.1%, and male-to-male contact in 27.9%. Among people in care, 20.1% had less than 350 CD4 cells/μl, 87.6% received antiretroviral therapy, and among these, 62.4% had a CD4 cell count higher than 350 cells/μl. The overall estimated prevalence of individuals diagnosed and linked to care in 2012 in Italy was 0.16 per 100 residents (all ages). Adding the estimated proportion of undiagnosed people, the estimated HIV prevalence would range between 0.19 and 0.26 per 100 residents. In Italy, the majority of people diagnosed and linked to care receive antiretroviral therapy. A higher prevalence of individuals diagnosed and linked to care was observed in Northern Italy and among males. More information for developing the HIV care continuum is necessary to improve the entire engagement in care, focusing on test-and-treat strategies to substantially reduce the proportion of people still undiagnosed or with a detectable viral load.

To date no national guidelines about diagnostic and prognostic evaluation of patients with disorders of consciousness (DoC) are available. As a consequence, diagnostic and prognostic procedures for DoC are extremely heterogeneous across clinical settings both in acute and chronic phases. The aim of this study is to plan a “SINC-SIRN” protocol on diagnostic and prognostic procedures as best practice and gold standard. Special Interest Groups (SIG) of SINC and SIRN, with complementary expertise in clinical and research in neurophysiology and rehabilitation, will cooperate in the following working steps: 1. Revision of scientific literature on diagnostic and prognostic procedures. 2. Elaboration of a “SINC-SIRN” protocol focused on the acute and post-acute/rehabilitative phases of care pathway. 3. Discussion and sharing within SINC and SIRN-SIG members. 4. Approval by SIRN and SINC executive committees. The preliminary diagnostic and prognostic protocol includes: 1. The Italian version of Coma Recovery Scale-Revised as a clinical tool. 2. Standard EEG. 3. Bilateral upper limb somatosensory evoked potentials. 4. Event-related potentials with detection of N100 and P300 cortical components. The integration of clinical and neurophysiological evaluation in the routine care from acute to chronic phase could provide useful diagnostic and prognostic information to guide management of patients with DoC.

The onset of neurological deficit is the most feared complications in surgical correction of spine deformities. Aim of this study is to assess the value of intraoperative neurophysiologic monitoring during scoliosis surgery. 173 scoliosis surgeries in 164 patients were monitored with muscle-motor evoked potentials (m-MEPs) and peripheral and cortical upper and lower limb Somatosensory Evoked Potentials (SEPs). In 45 surgeries, thoracic and lumbar pedicle screws were neurophysiologically tested by triggered-EMG. The overall monitor ability was 98.3%. Significant intraoperative modification occurred in 26 surgeries (15%). In 11 cases (6.4%) changes were consistent with spinal cord damage, in 6 (6.9%) with brachial plexopathy. Finally, three patients (1.7%) presented transient disappearance of four limbs MEPs and SEPs due to a pharmacological effect. 2 patients (1.2%) awaked with a brachial plexopathy: they all completely recovered within 3 months. In one patients, an intraoperative persistent disappearance of unilateral lower limb MEPs and SEPs was associated with a postoperative severe monoparesis: therefore, the rate of new neurological deficit was 0.6%. Intraoperative neurophysiology may provide a valuable biomarker in detection of impending central and/or peripheral nervous system injury at a reversible stage.

Erratum in: Familial occurrence and heritable connective tissue disorders in cervical artery dissection. [Neurology. 2016]; International audience; In a large series of patients with cervical artery dissection (CeAD), a major cause of ischemic stroke in young and middle-aged adults, we aimed to examine frequencies and correlates of family history of CeAD and of inherited connective tissue disorders. We combined data from 2 large international multicenter cohorts of consecutive patients with CeAD in 23 neurologic departments participating in the CADISP-plus consortium, following a standardized protocol. Frequency of reported family history of CeAD and of inherited connective tissue disorders was assessed. Putative risk factors, baseline features, and 3-month outcome were compared between groups. Among 1,934 consecutive patients with CeAD, 20 patients (1.0%, 95% confidence interval: 0.6%-1.5%) from 17 families (0.9%, 0.5%-1.3%) had a family history of CeAD. Family history of CeAD was significantly more frequent in patients with carotid location of the dissection and elevated cholesterol levels. Two patients without a family history of CeAD had vascular Ehlers-Danlos syndrome with a mutation in COL3A1. This diagnosis was suspected in 2 additional patients, but COL3A1 sequencing was negative. Two patients were diagnosed with classic and hypermobile Ehlers-Danlos syndrome, one patient with Marfan syndrome, and one with osteogenesis imperfecta, based on clinical criteria only. In this largest series of patients with CeAD to date, family history of symptomatic CeAD was rare and inherited connective tissue disorders seemed exceptional. This finding supports the notion that CeAD is a multifactorial disease in the vast majority of cases.

The poly(3-hexanoyloxyethyl-2,5-thienylene) was studied by means of mono-and bidimensional nuclear magnetic resonance spectroscopy. The regiochemistry of the polymer is assigned on the basis of the 13C chemical shifts, which are easily obtained through inverse detection experiments, utilizing a triad-based model. © 1997 Elsevier Science Ltd. All rights reserved.

The purpose of this work is the estimation of the Glasgow outcome scale (GOS) from a single continuous electroencephalogram (c-EEG) routinely recorded to monitor comatose patients in the neurosurgical intensive care unit. c-EEG was recorded from 13 patients in the acute phase: five with GOS = 5, four with GOS = 3 and four with GOS = 1. Different indexes were extracted from epochs of c-EEG (classical: amplitude and spectral estimators; non classical: from recurrence quantification analysis—RQA—and approximate entropy). Descriptors of different indexes (temporal variation and mean, standard deviation, skewness of the distribution across epochs) were used to train support vector machines to identify the correct GOS. We found classifiers allowing correct classification of the patients. Spectral indexes allowed to get optimal performances in classifying GOS 1 and 3. Nonlinear indexes (especially determinism from RQA) were optimal for identifying GOS = 5. Thus, the integration of information from classical/linear and nonlinear c-EEG descriptors in a multi-index classifier is important for GOS estimation.

Background: Seizures are common neurological consequences of stroke. Although a number of factors including stroke severity on admission, cortical involvement, and stroke subtype have been consistently associated with post-stroke seizures, the effect that medical and neurological complications of stroke, occurring in the very acute phase, might have on such a risk has never been adequately explored. In the present study we aimed at determining the extent to which complications within the first week of stroke influence the risk of early seizures (ES). Methods: Data of consecutive patients with first-ever acute stroke included in the Brescia Stroke Registry were analyzed. ES (≤7 days) were recorded and correlated with demographic data, disease characteristics, risk factors, and prespecified medical and neurological stroke complications in a multivariate path analysis model. Results: 516 patients with first-ever acute stroke were eligible for inclusion in the present study. Of them, 436 patients had ischemic stroke (IS) [64 (14.6%) with hemorrhagic transformation (HT)] and 80 had intracerebral hemorrhage (ICH). Twenty patients (3.9%) developed ES. Patients with ES had a higher burden of complications compared with those without (30 vs. 4.2%, for patients with >6 complications). Lesion type, stroke complications, and lesion site were directly related to the risk of seizure occurrence (OR, 0.24; 95% CI, 0.07-0.80 for IS vs. ICH; OR, 1.57; 95% CI, 1.21-2.01 for any increase of 1 in the number of complications; OR, 0.15; 95% CI, 0.04-0.56 for subcortical lesions vs. cortical lesions). Complications appeared also to mediate the indirect effect of lesion type on the occurrence of ES (OR, 0.75; 95% CI, 0.60-0.94). No significant difference on the risk of ES was observed when HT and ICH were compared. The total effect of lesion type was 0.25 × 0.75 = 0.18, corresponding to (1-0.18) = 82% lower risk of ES for IS as compared to ICH. Conclusion: Although major determinants of ES are nonmodifiable, preventable and treatable medical and neurologic complications within the first week of stroke increase the risk of ES and mediate the effect of established predictors on the propensity to post-stroke epilepsy. Future epidemiologic studies aimed at investigating post-stroke seizures should include precise information on these complications.

Introduction Cerebral venous thrombosis (CVT) is a rare cerebrovascular condition accounting for Methods and analysis To better understand the genetic basis of CVT, we have established an international biobank of CVT cases, Biorepository to Establish the Aetiology of Sinovenous Thrombosis (BEAST) which aims to recruit highly phenotyped cases initially of European descent and later from other populations. To date we have recruited 745 CVT cases from 12 research centres. As an initial step, the consortium plans to undertake a genome-wide association analysis of CVT using the Illumina Infinium HumanCoreExome BeadChip to assess the association and impact of common and low-frequency genetic variants on CVT risk by using a case–control study design. Replication will be performed to confirm putative findings. Furthermore, we aim to identify interactions of genetic variants with several environmental and comorbidity factors which will likely contribute to improve the understanding of the biological mechanisms underlying this complex disease. Ethics and dissemination BEAST meets all ethical standards set by local institutional review boards for each of the participating sites. The research outcomes will be published in international peer-reviewed open-access journals with high impact and visibility. The results will be presented at national and international meetings to highlight the contributions into improving the understanding of the mechanisms underlying this uncommon but important disease. This international DNA repository will become an important resource for investigators in the field of haematological and vascular disorders.

Change in somato-sensory evoked potentials (SSEP) or motor evoked potentials (MEPs) are variably associated with clinical modifications. This study aimed to investigate if neurophysiological changes during IOM are related with clinical modifications in patients underwent spinal surgery. 71 patients that presented neurophysiological changes in somato-sensory evoked potentials (SEPs) or motor evoked potentials (MEPs) during spinal surgery were considered. The pathologies included in the study were: cervical/thoracic myelopathy (27 pts, 38%), spinal cord tumours (31 pts, 44%), scoliosis correction (3 pts, 4%), trauma (7 pts, 10%) and spinal vascular malformations (3 pts, 4%). Clinical outcome, estimated with McCormick scale, was evaluated in the immediate postoperative period and at one month follow-up. Neurophysiological changes were associated to clinical modifications at one month follow-up in 36 patients (51%). When a clinical improvement was observed (19 pts), the percentage of SEPs or MEPs reappearance from basal absence was 42% (8 pts). Contrariwise, evoked potentials disappeared in 65% (11 pts) of observed clinical worsening (17 pts). Neurophysiological modifications in SEPs or MEPS seem to be associated clinical changes in patients submitted spinal surgery.

The prediction of neurologic outcome in comatose patients after cardiac arrest (CA) has been investigated and the findings were summarized in a practice parameter of the American Academy of Neurology (1). These guidelines did not include the EEG as a reliable outcome indicator but recent studies on continuous EEG recorded during TH have re-evaluated the role of EEG (2–4). These studies underline the utility of a simplified classification system of EEG patterns and point to which pattern attains reliable predictive value both for good and bad outcomes. These reports are of high clinical relevance because, until today, the possibility of identifying early prognostic indicators of good outcome in patients surviving to CA has not been investigated. At the best of our knowledge, only few multicentre prospective studies (4–6) analyzed the prognostic value of EEG and SEP recorded in the same patient after CA as single predictor but none analyzed the diagnostic accuracy of the association of these two tests. We present the study protocol of Italian Multicentre Study about prognostic value of EEG and SEP in comatose patients after CA (ProNeCA) proposed by study group of “Intraoperative and Intensive Care Clinical Neurophysiology” of the Italian Society of Clinical Neurophysiology.

Post-operative sensory-motor outcome is related to intraoperative surgical or vascular complications. Intraoperative Neurophysiologic Monitoring (IONM) identifies changes to allow quick correction of the causes before neurological damages. The aim of the study is to assess the significance of IONM during scoliosis surgery, in particular for evaluating the relevance of motor evoked potential. 145 patients in TIVA/TCI anaesthesia were monitored through motor evoked potentials derived by upper and lower limbs’ muscles (mMEPs) and Somatosensory Evoked Potentials derived from Erb-point and scalp by Median and Posterior Tibial nerve stimulation (SEPs). 94, 5% of patients were recordable, while 5,5% presented no basal mMEPs and/or SEPs for previous pathologies and were non-elected. 2% had abortion of mMEPs for anaesthetic plane. 5,5% presented fleeting alteration of peripheral responses from Erb-point during surgery. 6% suffered of transitory modification of neurophysiological parameters: 100% of the alterations hit the mMEPs and just 22,2% the SEPs but anyone had only SEPs modifications in preserved mMEPs. Intraoperative combined SEPs and mMEPs monitoring is a safe, reliable and sensitive method to detect and reduce intraoperative injury to the spinal cord during scoliosis surgery, in particular, mMEPs are elective for early intraoperative detection of anterior medullar ischemia.

The role of intraoperative neurophysiologic monitoring (IOM) in detection and prevention of an jatrogenic injury has been well documented. Only few studies, however, has been focused on recovery of evoked potentials. Aim of this study is to describe the rate of reappearance of basally unexcitable muscle-motor evoked potentials (m-MEPs) and somatosensory evoked potentials (SEPs) in a large cohort of spinal surgeries. Data on 720 spinal surgeries are shown. Transcranially elicited lower limbs m-MEPs and tibial nerve SEPs were intraoperative recorded according to standard protocols. Reasons for surgey included cervical/thoracic myelopathy (300 pts, 41.7%), spinal cord tumors (190, 26.4%), lumbar stabilization (109 pts, 15.1%), scoliosis correction (83, 11.5%), trauma (32, 4.4%) and spinal vascular malformations (6 pts, 0.8%). Overall no intraoperative changes were observed in 648 surgeries (90%), 54 (7.5%) transient or persistent deterioration of evoked potentiantials and 18 (2.5%) recovery of basally unexcitable SEPs or MEPs (9 myelopathies, 4 spinal cord tumors, 2 metastasis, 3 trauma). The large majority of these “positive” modifications (17/18, 94.4%) consisted in reappearance of MEPs. Surgical decompression of spinal cord may lead to an acute improving of spinal motoneuron functionality. IOM can provide useful information even in patient with basally absent m-MEPs.

Cerebral vein thrombosis (CVT) is a rare complication of spontaneous intracranial hypotension (SIH). When to suspect a thrombotic disorder during the course of intracranial hypotension is not fully elucidated. A 48-year-old woman was admitted because of SIH with no signs of CVT on neuroimaging. The occurrence of diplopia and blurred vision 12 days later led to the performance of further investigations, which revealed thrombosis of the left lateral sinus, in the absence of variations in the headache characteristics. Among the other 4 cases of SIH clearly preceding the occurrence of CVT reported so far, only one had a change in the headache pattern related to CVT development. Although a change in the characteristics of headache is considered a marker of CVT in patients with SIH, this is not invariably part of the clinical scenario. Any new neurologic finding on exam in the disease course should raise a suspicion of venous thrombosis, thus prompting further specific investigations.