An Italian consensus on the management of Lennox-Gastaut syndrome.

Purpose: Although international guidelines exist, the clinical heterogeneity of Lennox-Gastaut syndrome (LGS) and the increasing availability of new and repurposed drugs (e.g., fenfluramine and cannabidiol) requires a practical guide to patient management in the clinical context.

Young Neurosurgeons and Technology: Survey of Young Neurosurgeons Section of Italian Society of Neurosurgery (Società Italiana di Neurochirurgia, SINch).

Background: Technological advancement in neurosurgery is a continuous process aimed at improving existing devices and implementing innovative ones. Recently, artificial intelligence (AI)-derived technologies (i.e.

Further delineation and long-term evolution of electroclinical phenotype in Mowat Wilson Syndrome. A longitudinal study in 40 individuals.

Background: Epilepsy is a main feature of Mowat Wilson Syndrome (MWS), a congenital malformation syndrome caused by ZEB2 variants. The aim of this study was to investigate the long-term evolution of the electroclinical phenotype of MWS in a large population.

Methods: Forty-individuals with a genetically confirmed diagnosis were enrolled.

Results From an Italian Expanded Access Program on Cannabidiol Treatment in Highly Refractory Dravet Syndrome and Lennox-Gastaut Syndrome.

Purified cannabidiol (CBD) was administered to highly refractory patients with Dravet (DS) or Lennox-Gastaut (LGS) syndromes in an ongoing expanded access program (EAP). Herein, we report interim results on CBD safety and seizure outcomes in patients treated for a 12-month period. Thirty centers were enrolled from December 2018 to December 2019 within the open-label prospective EAP up to a maximum of 25 mg/kg per day.

Italian cohort of Lafora disease: Clinical features, disease evolution, and genotype-phenotype correlations.

Background: Lafora disease (LD) is characterized by progressive myoclonus, refractory epilepsy, and cognitive deterioration. This complex neurodegenerative condition is caused by pathogenic variants in EPM2A/EPM2B genes, encoding two essential glycogen metabolism enzymes known as laforin and malin. Long-term follow-up data are lacking.

Cannabidiol in Pharmacoresistant Epilepsy: Clinical Pharmacokinetic Data From an Expanded Access Program.

Data on the clinical pharmacokinetics of cannabidiol (CBD) are scanty. We explored the effect of demographic and clinical variables on plasma concentrations of purified CBD in patients with Dravet (DS) and Lennox-Gastaut syndrome (LGS). The study design was an open, prospective, multicenter expanded access program (EAP).

Modified Kudo classification can improve accuracy of virtual chromoendoscopy with FICE in endoscopic surveillance of ulcerative colitis.

 Virtual chromoendoscopy with Fuji Intelligent Color Enhancement (FICE) has never been studied in prospective trials of endoscopic surveillance for ulcerative colitis (UC). We compared FICE and white light endoscopy (WLE) in differentiation of visible lesions in UC.  In a prospective parallel study, we compared consecutive outpatients with UC submitted to surveillance colonoscopy with FICE or WLE.

Severe prolonged neutropenia following administration of tocilizumab in a patient affected by COVID-19: a case report and brief review of the literature.

Tocilizumab is one of the newest therapeutic options for the acute respiratory distress syndrome (ARDS) caused by the recently discovered severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) β-coronavirus. Several trials are currently ongoing to assess the efficacy and safety profile of tocilizumab in treating ARDS. In this article, we present the case of a Black patient with acute pneumonia who benefited greatly from tocilizumab, but developed severe prolonged neutropenia.

Aicardi Syndrome: Key Fetal MRI Features and Prenatal Differential Diagnosis.

Objective: This study was aimed to investigate the prenatal findings in Aicardi syndrome (AIC) by intrauterine magnetic resonance imaging (iuMRI) suggesting possible diagnostic criteria and differential diagnosis.

Methods: The iuMRI features of nine AIC confirmed cases were described and then compared with those of postnatal MRI. Furthermore, all iuMRI cases with both corpus callosum (CC) agenesis-dysgenesis and cortical malformation (AIC mimickers) were retrospectively reviewed and compared with iuMRI AIC cases, in order to identify possible neuroradiological predictors of AIC syndrome.

Reorganisation of medical oncology departments during the novel coronavirus disease-19 pandemic: a nationwide Italian survey.

The novel severe acute respiratory syndrome coronavirus-2 pandemic is a global health problem, which started to affect China by the end of 2019. In Europe, Italy has faced this novel disease entity (named novel coronavirus disease [COVID-19]) first and severely. COVID-19 represents a significant hurdle for public health services and a potential harm for patients with cancer.

Positioning issues of spinal surgery during pregnancy.

Background: Albeit rarely, different spinal pathologies may require surgical treatment during pregnancy. The management of such cases poses a series of challenges, starting with adequate body positioning.

Objective: To illustrate limits and indications of the different surgical positioning strategies for pregnant women undergoing spine surgery.

Myocardial edema in paroxysmal permeability disorders: The paradigm of Clarkson's disease.

Purpose: Paroxysmal Permeability Disorders (PPDs) comprise a variety of diseases characterized by recurrent and transitory increase of endothelial permeability. Idiopathic Systemic Capillary Leak Syndrome (ISCLS) is a rare PPD that leads to an abrupt massive shift of fluids and proteins from the intravascular to the interstitial compartment. In some cases, tissue edema may involve the myocardium, but its role in the development of shock has not been elucidated so far.

A validation study of the clinical diagnosis of Dup15q syndrome: Which symptoms matter most?

Purpose: Dup15q syndrome is a rare genetic disease with a fairly nonspecific phenotype, clinical heterogeneity, and a wide spectrum of severity. However, no formal characterization has been attempted to select clusters of symptoms, signs and instrumental tests, to be used in the differential diagnosis with other neurodevelopmental disorders. Thus, our purpose was to identify symptoms, signs and instrumental findings, singly or in various combinations, favoring the early diagnosis of the Dup15q syndrome and the indication for genetic testing.

New ophthalmic dual-viscoelastic device in cataract surgery: a comparative study.

Objective: To compare the performance and safety in cataract surgery of two ophthalmic viscoelastic devices (OVDs), each having separate dispersive and cohesive characteristics and different concentrations.

Methods And Analysis: In this prospective, randomised, controlled clinical study, one eye of each patient was injected with OVD1 (Viscopack14) or OVD2 (DuoVisc) during phacoemulsification and intraocular lens implantation. Endothelial cell count, intraocular pressure (IOP), central corneal thickness (CCT), intraocular inflammation and corrected distance visual acuity (CDVA) were compared 3 months postoperatively.

Management of psychogenic non-epileptic seizures: a multidisciplinary approach.

The International League against Epilepsy (ILAE) proposed a diagnostic scheme for psychogenic non-epileptic seizure (PNES). The debate on ethical aspects of the diagnostic procedures is ongoing, the treatment is not standardized and management might differ according to age group. The objective was to reach an expert and stakeholder consensus on PNES management.

Nonbiological therapeutic management of ulcerative colitis.

Introduction: Treatment of ulcerative colitis (UC) is constantly evolving. In the last two decades, new therapeutic strategies have been implemented by addressing specific disease mechanisms: biological agents against tumor necrosis factor-α and integrins are now widely used, and more agents targeting different pathological pathways are being marketed. Despite these novel therapies, nonbiological drugs are still the mainstay of treatment, especially in mild-to-moderate disease, since a proven safety and tolerability profile is observed.

Long-term follow-up in pediatric patients with paroxysmal hypothermia (Shapiro's syndrome).

Introduction: Shapiro syndrome (SS) is characterized by spontaneous recurrent episodes of hypothermia, hyperhidrosis and corpus callosum (CC) agenesis. Less than 60 cases have been reported to date and the pathogenic mechanism as well as the prognosis of this syndrome are still debated. We describe the clinical features and long-term follow-up of a pediatric cohort of SS patients.

Electroclinical findings and long-term outcomes in epileptic patients with inv dup (15).

Objective: To define the electroclinical phenotype and long-term outcomes in a cohort of patients with inv dup (15) syndrome.

Material And Methods: The electroclinical data of 45 patients (25 males) affected by inv dup (15) and seizures were retrospectively analysed, and long-term follow-up of epilepsy was evaluated.

Results: Epilepsy onset was marked by generalized seizures in 53% of patients, epileptic spasms in 51%, focal seizures in 26%, atypical absences in 11% and epileptic falls in 9%.

Symptoms of anxiety and depression and family's quality of life in children and adolescents with epilepsy.

Introduction: We studied children and adolescents with epilepsy (CAWE) and their families to evaluate symptoms of anxiety and depression, quality of life (QoL), and their correlations with epilepsy characteristics.

Material And Methods: The study included 326 (52.5% females) 8 to 18years old CAWE.

Midazolam vs diazepam in prolonged seizures in children: A pharmacoeconomic approach.

Objective: A previous European cost-utility study reported that use of buccal midazolam in the community setting for the treatment of prolonged seizures (ie, seizures lasting ≥5 minutes) in children was associated with an overall €12 507 399 reduction in annual costs charged to the Italian national health service compared with rectal diazepam. We re-evaluated these findings by applying a more conservative approach.

Methods: The Italian Delphi panel reconvened to apply a more conservative assessment of available reports.

Visual performance after bilateral implantation of 2 new presbyopia-correcting intraocular lenses: Trifocal versus extended range of vision.

Purpose: To compare the visual outcomes and quality of vision of 2 new diffractive multifocal intraocular lenses (IOLs) with those of a monofocal IOL.

Setting: Fatebenefratelli e Oftalmico Hospital, Milan, Italy.

Design: Prospective case series.

Sudarshan Kriya Yoga improves cardiac autonomic control in patients with anxiety-depression disorders.

Background: Several studies have demonstrated that adjuvant therapies as exercise and breathing training are effective in improving cardiac autonomic control (CAC) in patients with affective spectrum disorders. However, the effects of Sudarshan Kriya Yoga (SKY) on autonomic function in this population is unknown. Our objective was to test the hypothesis that SKY training improves CAC and cardiorespiratory coupling in patients with anxiety and/or depression disorders.