Neonatal hemifacial spasm and fourth ventricle mass

Congential hemifacial spasmis a rare condition that is characterized by the occurrence of paroxysmal hemifacial contractions in neonates. We review the clinical, neurophysiological, neuroimaging, and histopathological findings, as well as the differential diagnosis, therapeutic approach, and outcome of all the described cases. Moreover, we report two new cases including the ictal video-electroencephalography recordings. Hemifacial spasm starts early in life, and is characterized by unilateral, involuntary, irregular tonic or clonic contractions ofmuscles innervated by the seventh cranial nerve. Hemifacial spasmis associated with eyelid blinking, and sometimes with breathing irregularities, hyperventilation, and ⁄ or other neurological manifestations (dystonicmovements, nystagmus). Interictal and ictal video-electroencephalography did not reveal epileptiform abnormalities. In all cases, brain magnetic resonance imaging showed amass involving the cerebellar peduncle, the cerebellar hemisphere, or the floor of the fourth ventricle. The semiology of the paroxysmal attacks is probably due to the activation of cranial nerve nuclei through intralesional hypersynchronous discharges, as shown by the intraoperative recordings and functional brain imaging described in the literature.We point out the importance of identifying such seizures in order tomake an early diagnosis of the underlying cerebral lesion

Risk of seizures during intraoperative electrocortical stimulation of brain motor areas: a retrospective study on 50 patients

Tumours close to cerebral cortices involved in motor and language functions represent a major challenge for neurosurgeons. Intraoperative neurophysiologic monitoring is useful to gain insight into the anatomy of and the relationship between pathological and normal tissues. In this study we report on the experience of electrocortical stimulation in the surgery of tumours adjacent to the motor cortex in 50 patients under general anaesthesia (26 under propofol, 24 under sevoflurane), and on EMG responses from contralateral muscles. In 18 patients stimulation evoked seizures, which were controlled only with antiepileptic drugs (36%). No difference was found in the incidence of intra-operative seizures between the patients with (10 out of 27) or without (8 out of 23) pre-operative epilepsy (p = 0.8685). The majority of the patients (13 out of 18) with intraoperative seizures were under sevoflurane (p = 0.01) and there was a statistically significant difference in the mean electrical intensity used between the two groups, sevoflurane and propofol, respectively 5.3 \ub1 1.3 mA and 3.6 \ub1 2 mA (p = 0.03). Regarding pre-operative anti-epileptic drugs, the use of levitiracetam was associated with a high incidence of intraoperative seizure (5 out of 6 patients). 4 patients developed new, unwanted, permanent neurological deficits, of which 2 had intraoperative seizures controlled only with antiepileptic drugs. Electrocortical stimulation is a powerful tool to understand the functional organization of patients' eloquent areas. Intraoperative epileptic seizures may represent an unwanted complication preventing further stimulation and possibly worsening neurological results. The choice of anaesthetics according to the patients' characteristics, pre-op symptoms and medical therapy is pivotal

Health technology assessment report on vagus nerve stimulation in drug-resistant epilepsy

Background: Vagus nerve stimulation (VNS) is a palliative treatment for medical intractable epileptic syndromes not eligible for resective surgery. Health technology assessment (HTA) represents a modern approach to the analysis of technologies used for healthcare. The purpose of this study is to assess the clinical, organizational, financial, and economic impact of VNS therapy in drug-resistant epilepsies and to establish the congruity between costs incurred and health service reimbursement. Methods: The present study used an HTA approach. It is based on an extensive detailed bibliographic search on databases (Medline, Pubmed, Embase and Cochrane, sites of scientific societies and institutional sites). The HTA study includes the following issues: (a) social impact and costs of the disease; (b) VNS eligibility and clinical results; (c) quality of life (QoL) after VNS therapy; (d) economic impact and productivity regained after VNS; and (e) costs of VNS. Results: Literature data indicate VNS as an effective treatment with a potential positive impact on social aspects and on quality of life. The diagnosis-related group (DRG) financing, both on national and regional levels, does not cover the cost of the medical device. There was an evident insufficient coverage of the DRG compared to the full cost of implanting the device. Conclusions: VNS is a palliative treatment for reducing seizure frequency and intensity. Despite its economic cost, VNS should improve patients\u2019 quality of life and reduce care needs

Targeting the brain : considerations in 332 consecutive patients treated by deep brain stimulation (DBS) for severe neurological diseases

Deep Brain Stimulation (DBS) extends the treatment of some severe neurological diseases beyond pharmacological and conservative therapy. Our experience extends the field of DBS beyond the treatment of Parkinson disease and dystonia, including several other diseases such as cluster headache and disruptive behavior. Since 1993, at the Istituto Nazionale Neurologico "C. Besta" in Milan 580 deep brain electrodes were implanted in 332 patients. The DBS targetnacc, IC, PPN, include Stn, GPi, Voa, Vop, Vim, CM-pf, pHyp, cZi, and Broadmann areas 24 and 25s. Three hundred patients are still avaiable for follow-up and therapeutic considerations. DBS gave a new therapeutic chance to these patients affected by severe neurological diseases and in some cases controlled life-threatening pathological conditions, which would otherwise result in the death of the patient such as in status dystonicus, status epilepticus and post-stroke hemiballism. The balance of DBS in severe neurological disease is strongly positive even if further investigations and studies are needed to search for new applications and refine the selection criteria for the actual indication

Vagus nerve stimulation in refractory epilepsy : New indications and outcome assessment

Although vagus nerve stimulation (VNS) is an effective alternative option for patients with refractory epilepsy unsuitable for conventional resective surgery, predictors of a better control of seizure frequency and severity are still unavailable. This prospective study reports on 39 patients, including 4 children affected by epilepsia partialis continua (EPC), who underwent VNS for refractory epilepsy. The overall seizure frequency outcome was classified into three groups according to reduction rate: 6575%, 6550%, and <50%. Engel and McHugh classifications were also used. The median follow-up period was 36months. A seizure reduction rate 6550% or EPC improvement was observed in 74% of the patients. Twenty-one out of 35 cases (60%) resulted in Engel classes II and III. Outcome, as defined by the McHugh scale, showed a responder rate of 71%. These results suggest that younger patient age and focal or multifocal epilepsy are related to a better seizure control and cognitive outcome. Vagus nerve stimulation could also be considered as an effective procedure in severe conditions, such as drug-refractory EPC

Health technology assessment report on the presurgical evaluation and surgical treatment of drug-resistant epilepsy

PURPOSE: Epilepsy is a neurologic disorder with major social impact. Surgery is a valuable option in patients who are not responding to antiepileptic drugs. The literature reports demonstrate that a proportion ranging from 40 to 100% of patients with epilepsy achieve seizure remission after surgery. A presurgical evaluation (clinical and instrumental) must be performed in all patients with drug-resistant epilepsy to assess their suitability for surgical intervention. Health Technology Assessment (HTA) represents a modern approach to the analysis of technologies used for health care. HTA could be considered a bridge between science that produces evidence and the decisions that can be taken on the basis of that evidence at different levels of the health care system. The aim of this study is the HTA of epilepsy surgery including clinical, ethical, social, and economic features. METHODS: The present study includes an analysis of the diagnostic and surgical workup performed at the Italian centers for the diagnosis and treatment of drug-resistant epilepsy (DRE). The study includes the following issues: (1) social, ethical impact, and costs of the disease; (2) clinical results, efficacy, and safety of surgery; (3) ethics and quality of life after surgery; and (4) economic impact and productivity regained after surgery. The cost of managing a patient with DRE included in the presurgical study was estimated by the bottom-up microcosting technique that starts from a detailed collection of data on consumption of resources and full costing. The phases analyzed were (1) noninvasive diagnostic workup; (2) neurosurgical intervention; and (3) follow-up. KEY FINDINGS: The literature reports indicate epilepsy surgery as an effective treatment both on clinical results and on ethical, social, and quality of life aspects. The workup including the noninvasive presurgical study followed by surgery has a total cost of \u20ac20,827. Management of short-term follow-up increases the overhead to \u20ac22,291 at the first year, and then to \u20ac23,571 after 5 years. According to the estimates made in this survey, funding based on diagnosis-related group (DRG) tariff for the noninvasive diagnostic stage involving hospital admission is not remunerative in Italy either at regional or national levels. Effectively the difference between full cost and DRG has a delta of \u20ac3,402 and \u20ac2,537 respectively. The total cost of the presurgical, surgical, and follow-up evaluation is not remunerative for \u20ac10,554 (national data). SIGNIFICANCE: Economic surveys in Italy have shown that surgery for DRE is an advantageous treatment from the standpoint of third-party payers and is cost-effective for society. DRE presurgical evaluation and surgery are not remunerative either at regional or national levels

Technical design report for the upgrade of the ALICE inner tracking system

ALICE (A Large Ion Collider Experiment) is studying the physics of strongly interacting matter, and in particular the properties of the Quark-Gluon Plasma (QGP), using proton-proton, proton-nucleus and nucleus-nucleus collisions at the CERN LHC (Large Hadron Collider). The ALICE Collaboration is preparing a major upgrade of the experimental apparatus, planned for installation in the second long LHC shutdown in the years 2018-2019. A key element of the ALICE upgrade is the construction of a new, ultra-light, high-resolution Inner Tracking System (ITS) based on monolithic CMOS pixel detectors. The primary focus of the ITS upgrade is on improving the performance for detection of heavy-flavour hadrons, and of thermal photons and low-mass di-electrons emitted by the QGP. With respect to the current detector, the new Inner Tracking System will significantly enhance the determination of the distance of closest approach to the primary vertex, the tracking efficiency at low transverse momenta, and the read-out rate capabilities. This will be obtained by seven concentric detector layers based on a 50 \uce\ubcm thick CMOS pixel sensor with a pixel pitch of about 30\uc3\u9730 \uce\ubcm2. This document, submitted to the LHCC (LHC experiments Committee) in September 2013, presents the design goals, a summary of the R&D activities, with focus on the technical implementation of the main detector components, and the projected detector and physics performance. \uc2\ua9 2014 CERN on behalf of The ALICE Collaboration

Author Correction: The FLUXNET2015 dataset and the ONEFlux processing pipeline for eddy covariance data (Scientific Data, (2020), 7, 1, (225), 10.1038/s41597-020-0534-3)

The following authors were omitted from the original version of this Data Descriptor: Markus Reichstein and Nicolas Vuichard. Both contributed to the code development and N. Vuichard contributed to the processing of the ERA-Interim data downscaling. Furthermore, the contribution of the co-author Frank Tiedemann was re-evaluated relative to the colleague Corinna Rebmann, both working at the same sites, and based on this re-evaluation a substitution in the co-author list is implemented (with Rebmann replacing Tiedemann). Finally, two affiliations were listed incorrectly and are corrected here (entries 190 and 193). The author list and affiliations have been amended to address these omissions in both the HTML and PDF versions. © 2021, This is a U.S. government work and not under copyright protection in the U.S.; foreign copyright protection may apply

The FLUXNET2015 dataset and the ONEFlux processing pipeline for eddy covariance data

The FLUXNET2015 dataset provides ecosystem-scale data on CO2, water, and energy exchange between the biosphere and the atmosphere, and other meteorological and biological measurements, from 212 sites around the globe (over 1500 site-years, up to and including year 2014). These sites, independently managed and operated, voluntarily contributed their data to create global datasets. Data were quality controlled and processed using uniform methods, to improve consistency and intercomparability across sites. The dataset is already being used in a number of applications, including ecophysiology studies, remote sensing studies, and development of ecosystem and Earth system models. FLUXNET2015 includes derived-data products, such as gap-filled time series, ecosystem respiration and photosynthetic uptake estimates, estimation of uncertainties, and metadata about the measurements, presented for the first time in this paper. In addition, 206 of these sites are for the first time distributed under a Creative Commons (CC-BY 4.0) license. This paper details this enhanced dataset and the processing methods, now made available as open-source codes, making the dataset more accessible, transparent, and reproducible