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In the wake of the severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) pandemic, an increasing number of patients with neurological disorders, including Guillain-Barré syndrome (GBS), have been reported following this infection. It remains unclear, however, if these cases are coincidental or not, as most publications were case reports or small regional retrospective cohort studies. The International GBS Outcome Study is an ongoing prospective observational cohort study enrolling patients with GBS within 2 weeks from onset of weakness. Data from patients included in this study, between 30 January 2020 and 30 May 2020, were used to investigate clinical and laboratory signs of a preceding or concurrent SARS-CoV-2 infection and to describe the associated clinical phenotype and disease course. Patients were classified according to the SARS-CoV-2 case definitions of the European Centre for Disease Prevention and Control and laboratory recommendations of the World Health Organization. Forty-nine patients with GBS were included, of whom eight (16%) had a confirmed and three (6%) a probable SARS-CoV-2 infection. Nine of these 11 patients had no serological evidence of other recent preceding infections associated with GBS, whereas two had serological evidence of a recent Campylobacter jejuni infection. Patients with a confirmed or probable SARS-CoV-2 infection frequently had a sensorimotor variant 8/11 (73%) and facial palsy 7/11 (64%). The eight patients who underwent electrophysiological examination all had a demyelinating subtype, which was more prevalent than the other patients included in the same time window [14/30 (47%), P = 0.012] as well as historical region and age-matched control subjects included in the International GBS Outcome Study before the pandemic [23/44 (52%), P = 0.016]. The median time from the onset of infection to neurological symptoms was 16 days (interquartile range 12-22). Patients with SARS-CoV-2 infection shared uniform neurological feat

The power production of the Lillgrund wind farm has been investigated by means of SCADA data covering almost 10 years of operation, wake models and numerical simulation tools, such as WindSim, OpenFOAM and ORFEUS. The analysis of the SCADA data provided the quantification of wake losses and of blockage effects, results that enable a benchmark to assess the uncertainty of other numerical methods of industrial use. The results have been analysed in terms of global array efficiency for different wind directions, wind velocity and stratification conditions. A comparison of the present results with the Jensen and Ainsle models indicated that the two used wake models are affected by an error of about 8–10% in the estimation of the array efficiency, while the error does not vary significantly when using numerical tools such as ORFEUS, WindSim and OpenFOAM. By comparing wind statistics available before the park construction, an assessment of the blockage of the Lillgrund farm was performed with contrasting results from the other numerical tools, highlighting how challenging is to assess farm blockage with current industrial settings., QC 20210319

Hereditary neuropathies may be misdiagnosed with chronic inflammatory demyelinating polyradiculoneuropathy (CIDP). A correct diagnosis is crucial for avoiding unnecessary therapies and access genetic counseling. We report on nine patients (seven men, mean age 49.2 ± 16.1) diagnosed with and treated as CIDP, in whom mutations or variants of unknown significance (VUS) in genes associated with hereditary neuropathies were reported. All underwent neurological and neurophysiological examination, eight also cerebrospinal fluid (CSF) analysis. In 4/9, nerve ultrasound and/or MR-neurography were performed. All the patients complained of progressive upper or lower limbs sensory-motor symptoms, with heterogeneous disease duration (1-34 years, mean 8.6 ± 10.8). Neurophysiology showed demyelinating signs in seven patients, mixed findings with predominant axonal damage in two patients. Neuroimaging disclosed diffuse abnormalities at proximal and distal segments. Molecular screening showed PMP22 duplication in two patients, mutations in the MPZ, EGR2, and GJB1 genes were reported in each of the remaining patients. The two patients with mixed neurophysiological findings had p.Val30Met mutation in the transthyretin gene. Two patients had VUS in the MARS and HSPB1 genes. Four patients had partial response to immunomodulant therapies, and CSF and neurophysiological features suggesting an inflammatory condition concomitant with the hereditary neuropathy. Hereditary neuropathy may be misdiagnosed with CIDP. The most common pitfalls are CSF (high protein levels and oligoclonal bands), incorrect interpretation of neurophysiology, and transient benefit from therapies. Neuroimaging may be helpful in cases with atypical presentations or when severe axonal damage complicate the neurophysiological interpretation.

Background: Diagnostic delay of hereditary transthyretin amyloidosis (ATTRv, v for variant) prevents timely treatment and, therefore, concurs to the mortality of the disease. The aim of the present study was to explore with nerve ultrasound (US) possible red flags for early diagnosis in ATTRv patients with carpal tunnel syndrome (CTS) and/or polyneuropathy and in pre-symptomatic carriers. Methods: Patients and pre-symptomatic carriers with a TTR gene mutation were enrolled from seven Italian centers. Severity of CTS was assessed with neurophysiology and clinical evaluation. Median nerve cross-section area (CSA) was measured with US in ATTRv carriers with CTS (TTR-CTS). One thousand one hundred ninety-six idiopathic CTS were used as controls. Nerve US was also performed in several nerve trunks (median, ulnar, radial, brachial plexi, tibial, peroneal, sciatic, sural) in ATTRv patients with polyneuropathy and in pre-symptomatic carriers. Results: Sixty-two subjects (34 men, 28 women, mean age 59.8 years ± 12) with TTR gene mutation were recruited. With regard to CTS, while in idiopathic CTS there was a direct correlation between CTS severity and median nerve CSA (r = 0.55, p < 0.01), in the subgroup of TTR-CTS subjects (16 subjects, 5 with bilateral CTS) CSA did not significantly correlate with CTS severity (r = − 0.473). ATTRv patients with polyneuropathy showed larger CSA than pre-symptomatic carriers in several nerve sites, more pronounced at brachial plexi (p < 0.001). Conclusions: The present study identifies nerve morphological US patterns that may help in the early diagnosis (morpho-functional dissociation of median nerve in CTS) and monitoring of pre-symptomatic TTR carriers (larger nerve CSA at proximal nerve sites, especially at brachial plexi).

Gli interventi di Adolfo de Carolis (1870-1928) e di Lodovico Pogliaghi (1857-1950) nella decorazione della Basilica del Santo nei primi decenni del Novecento costituiscono un interessante caso di studio per indagare la ricezione delle novità stilistiche dell’arte europea in Italia tra fine Ottocento – periodo al quale i due artisti appartengono come formazione di un loro stile maturo – l’epoca delle avanguardie e gli anni Venti di Novecento e del “ritorno all’ordine”. La decorazione della Cappella di san Francesco ad opera del pittore marchigiano si colloca infatti tra il 1925 e il 1928, anno della sua scomparsa, mentre l’esecuzione della cappella del Sacramento dello scultore lombardo Lodovico Pogliaghi ha una lunghissima gestazione tra il 1906 e il 1936. Entrambi gli artisti, pur con un diverso approccio stilistico, sono stati protagonisti nel periodo fin de siècle del rinnovamento dello stile dell’arte murale e della ricerca di un linguaggio artistico nazionale fondato sulla riscoperta della tradizione dell’arte italiana, in particolare medievale e rinascimentale, filtrata attraverso le coeve ricerche dell’arte decorativa sviluppatesi in Inghilterra, prima e in Francia poi. Tutti questi riferimenti storici e stilistici vengono dai due autori filtrati dalla personale attitudine artistica al fine di individuare uno stile nuovo e italiano da applicare all’arte decorativa e all’arte murale in particolare. Accomunati dall’interesse anche per le tecniche antiche, dall’affresco alle arti decorative, si trovano però a lavorare nella Basilica di Padova in un’epoca in cui il loro stie appare attardato e non aggiornato sulle nuove istanze dell’arte contemporanea, esemplarmente rappresentata, proprio negli stessi spazi della basilica dal pittore novecentista Ubaldo Oppi, chiamato a completare gli affreschi iniziati da de Carolis. Pertanto la loro presenza nella decorazione della basilica costituisce un ponte, senza soluzione di continuità, oltre le avanguardie, tra le i, The activities of Adolfo de Carolis (1870-1928) and Lodovico Pogliaghi (1857-1950) to decorate the Basilica del Santo, in the first decades of the twentieth century, constitute an interesting case study, to investigate the reception of the stylistic novelties of European art in Italy, between the late nineteenth century - a period in which the two artists belonged to their own mature style - the era of the avant-gardes and the “Novecento” of the twentieth century and the "return to order". The decoration of the Chapel of St. Francis by the painter from the Marche, an Italian region, was operated between 1925 and 1928, the year of his death, while the execution of the Chapel of the Sacrament by the Lombard sculptor Lodovico Pogliaghi had a very long gestation between 1906 and 1936. Both artists, although with a different stylistic approach, were protagonists, in the fin de siècle period, of the renewal of the style of mural art and of the search for a national artistic language based on the rediscovery of the Italian artistic tradition, particularly Medieval and Renaissance, filtered through the contemporary researches of decorative art developed in England, first and then in France. All these historical and stylistic references come from the two authors filtered by the personal artistic attitude in order to identify a new and Italian style to be applied to decorative and mural art. United by the interest also for the ancient techniques, from the fresco to the decorative arts, they find themselves working in the Basilica of Padua in an age in which their style appears to be delayed and not updated on the new instances of contemporary art, exemplarily represented , right in the same spaces of the basilica by the twentieth century painter Ubaldo Oppi, called to complete the frescoes started by de Carolis. Therefore, their presence in the decoration of the basilica constitutes a bridge, without solution of continuity, beyond the avant-gardes, between the instances of t