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Background: Patellar dislocation accounts for 2–3% of all knee lesions and is the second most common cause of traumatic hemarthrosis of the knee. Proper treatment is essential to minimize long term sequelae, such as recurrent dislocation, painful subluxation, and osteoarthritis. Aim: The purposes of this perspective randomized study were: 1) to evaluate the mid-term clinical and radiological results of the Elmslie–Trillat procedure for the treatment of traumatic patellar re-dislocation; and 2) to compare the results, especially radiological osteoarthritis development, with those of non-operative management for traumatic patellar re-dislocation. Methods: From June 2013 to February 2015, 65 patients were admitted to our Emergency Department for a first patellar dislocation episode. All patients were treated non-operatively. When a second episode occurred within two years, patients were randomly assigned in two groups: Group A, patients treated surgically with Elmslie-Trillat procedure, and Group B, patients were further treated non-operatively. The Knee Society Score (KSS), Lysholm scale and Kujala questionnaire were administered immediately after the re-dislocation and at the final follow-up. Tangential patella radiographs were evaluated for patellofemoral osteoarthritis and graded according to Sperner’s classification. Results: The mean follow-up was 6.4±1.7 years (range 5-8) in Group A and 6.3±2.1 years (range 5-9) in Group B. In Group A, the KSS and Lysholm scale showed significant differences between pre- and post-operatively values (p

Neonatal and infantile cholestasis (NIC) can represent the onset of a surgically correctable disease and of a genetic or metabolic disorder worthy of medical treatment. Timely recognition of NIC and identification of the underlying etiology are paramount to improve outcomes. Upon invitation by the Italian National Institute of Health (ISS), an expert working grouped was formed to formulate evidence-based positions on current knowledge about the diagnosis of NIC. A systematic literature search was conducted to collect evidence about epidemiology, etiology, clinical aspects and accuracy of available diagnostic tests in NIC. Evidence was scored using the GRADE system. All recommendations were approved by a panel of experts upon agreement of at least 75% of the members. The final document was approved by all the panel components. This position document summarizes the collected statements and defines the best-evidence diagnostic approach to cholestasis in the first year of life.

Objectives: It is assumed that autoimmune limbic encephalitis (ALE) demonstrates distinct neuropsychological manifestations with differential responses to immunotherapy according to which associated autoantibody (AAB), if any, is identified. Towards investigating whether this is the case, this study aims to summarize respective findings from the primary literature on ALE with AABs binding to cell surface neural antigens and ALE with AABs against intracellular neural antigens. Methods: We chose ALE with AABs against leucine-rich, glioma inactivated protein 1 (LGI1) and contactin-associated protein-like 2 (CASPR2) as the most frequent cell surface membrane antigens, and ALE with AABs to Embryonic Lethal, Abnormal Vision, Like 1 (ELAVL) proteins (anti-Hu) and glutamic acid decarboxylase 65 (GAD65) as the most frequent intracellular neural antigens. The PubMed and Scopus databases were searched on March 1st, 2021 for neuropsychological test and -screening data from patients with ALE of these AAB-types. Findings were reviewed according to AAB-type and immunotherapy status and are presented in a review section and are further statistically evaluated and presented in a meta-analysis section in this publication. Results: Of the 1304 initial hits, 32 studies on ALE with AABs against LGI1, CASPR2, and GAD65 reporting cognitive screening data could be included in a review. In ALE with AABs against LGI1, CASPR2 and GAD65, memory deficits are the most frequently reported deficits. However, deficits in attention and executive functions including working memory, fluency, and psychological function have also been reported. This review shows that ALE patients with AABs against both LGI1 and CASPR2 show higher percentages of neuropsychological deficits compared to ALE patients with AABs against GAD65 before and after initiation of immunotherapy. However, the methodologies used in these studies were heterogenous, and longitudinal studies were not comparable. Moreover, 21 studies including ALE patients with AABs against LGI1 and GAD65 were also suitable for meta-analysis. No suitable study on ALE with AABs against ELAVL proteins could be identified. Meta-Analyses could be executed for cognitive screening data and only partially, due to the small number of studies. However, in statistical analysis no consistent effect of AAB or immunotherapy on performance in cognitive screening tests could be found. Conclusion: Currently, there is no definite evidence supporting the notion that different AAB-types of ALE exhibit distinct neuropsychological manifestations and respond differently to immunotherapy. Overall, we could not identify evidence for any effect of immunotherapy on cognition in ALE. More systematic, in-depth and longitudinal neuropsychological assessments of patients with different AAB-types of ALE are required in the future to investigate these aspects.

Background and ObjectivesWe sought to identify early factors associated with relapse and outcome in paediatric-onset myelin oligodendrocyte glycoprotein antibody-associated disorders (MOGAD).MethodsIn a multicenter retrospective cohort of pediatric MOGAD (≤18 years), onset features and treatment were compared in patients with monophasic vs relapsing disease (including cases with follow-up ≥12 months after onset or relapse at any time) and in patients with final Expanded Disability Status Scale (EDSS) 0 vs ≥1 at last follow-up (including cases with follow-up >3 months after last event or EDSS0 at any time). Multivariable logistic regression models were used to evaluate factors associated with relapsing disease course and EDSS ≥ 1 at final follow-up.ResultsSeventy-five children were included (median onset age 7 years; median 30 months of follow-up). Presentation with acute disseminated encephalomyelitis was more frequent in children aged 8 years or younger (66.7%, 28/42) than in older patients (30.3%, 10/33) (p= 0.002), whereas presentation with optic neuritis was more common in children older than 8 years (57.6%, 19/33) than in younger patients (21.4%, 9/42) (p= 0.001). 40.0% (26/65) of patients relapsed. Time to first relapse was longer in children aged 8 years or younger than in older patients (median 18 vs 4 months) (p= 0.013). Factors at first event independently associated with lower risk of relapsing disease course were immunotherapy p= 0.009), corticosteroid treatment for ≥5 weeks (6.7-fold reduced odds of relapse, OR 0.15, 95% CI 0.03–0.80,p= 0.026), and abnormal optic nerves on onset MRI (12.5-fold reduced odds of relapse, OR 0.08, 95% CI 0.01–0.50,p= 0.007). 21.1% (15/71) had EDSS ≥ 1 at final follow-up. Patients with a relapsing course had a higher proportion of final EDSS ≥ 1 (37.5%, 9/24) than children with monophasic disease (12.8%, 5/39) (p= 0.022, univariate analysis). Each 1-point increment in worst EDSS at onset was independently associated with 6.7-fold increased odds of final EDSS ≥ 1 (OR 6.65, 95% CI 1.33–33.26,p= 0.021).DiscussionAt first attack of pediatric MOGAD, early immunotherapy, longer duration of corticosteroid treatment, and abnormal optic nerves on MRI seem associated with lower risk of relapse, whereas higher disease severity is associated with greater risk of final disability (EDSS ≥ 1).

Myositis ossificans traumatica (MOT) is a self-limiting and self-resolving pathology. In most cases, conservative treatment is chosen as the first step. Surgical treatment is reserved for cases of failure of conservative treatment with persistence of pain and mass. The case presented concerns an 18-year-old soccer player suffering from bilateral adductor longus (AL) MOT results following two different sports injuries. The patient reports the appearance of swelling and palpable mass at the proximal and medial region of the thigh, about 2 cm from the pubic symphysis, along the course of the adductor magnus. The radiological investigation showed the presence of a calcification along the course of the right and left AL muscles. Surgical treatment was considered for the right thigh injury, being symptomatic 1 year after the onset and refractory to other treatments. At 3 months of follow-up, the patient was asymptomatic and completed the rehabilitation program for the recovery of muscle strength and range of motion (ROM). In cases where MOT manifests with resistant pain, ROM restriction and daily activity reduction, surgical excision is the preferred option.

BackgroundIgG antibodies against myelin oligodendrocyte glycoprotein (MOG-IgG) define a subset of associated disorders (myelin oligodendrocyte glycoprotein associated disorders (MOGAD)) that can have a relapsing course. However, information on relapse predictors is scarce. The utility of retesting MOG-IgG over time and measuring their titres is uncertain. We aimed to evaluate the clinical relevance of longitudinal MOG-IgG titre measurement to predict relapses in patients with MOGAD.MethodsIn this retrospective multicentre Italian cohort study, we recruited patients with MOGAD and available longitudinal samples (at least one >3 months after disease onset) and tested them with a live cell-based assay with endpoint titration (1:160 cut-off). Samples were classified as ‘attack’ (within 30 days since a disease attack (n=59, 17%)) and ‘remission’ (≥31 days after attack (n=295, 83%)).ResultsWe included 102 patients with MOGAD (57% adult and 43% paediatric) with a total of 354 samples (83% from remission and 17% from attack). Median titres were higher during attacks (1:1280 vs 1:640, p=0.001). Median onset titres did not correlate with attack-related disability, age or relapses. Remission titres were higher in relapsing patients (p=0.02). When considering the first remission sample available for each patient, titres >1:2560 were predictors of relapsing course in survival (log rank, pConclusionsPersistent MOG-IgG positivity and high remission titres are associated with an increased relapse risk. Longitudinal MOG-IgG titres could be useful to stratify patients to be treated with long term immunosuppression.

PurposeBinding proteins (G-proteins) mediate signalling pathways involved in diverse cellular functions and comprise Gα and Gβγ units. Human diseases have been reported for all five Gβ proteins. A de novo missense variant in GNB2 was recently reported in one individual with developmental delay/intellectual disability (DD/ID) and dysmorphism. We aim to confirm GNB2 as a neurodevelopmental disease gene, and elucidate the GNB2-associated neurodevelopmental phenotype in a patient cohort.MethodsWe discovered a GNB2 variant in the index case via exome sequencing and sought individuals with GNB2 variants via international data-sharing initiatives. In silico modelling of the variants was assessed, along with multiple lines of evidence in keeping with American College of Medical Genetics and Genomics guidelines for interpretation of sequence variants.ResultsWe identified 12 unrelated individuals with five de novo missense variants in GNB2, four of which are recurrent: p.(Ala73Thr), p.(Gly77Arg), p.(Lys89Glu) and p.(Lys89Thr). All individuals have DD/ID with variable dysmorphism and extraneurologic features. The variants are located at the universally conserved shared interface with the Gα subunit, which modelling suggests weaken this interaction.ConclusionMissense variants in GNB2 cause a congenital neurodevelopmental disorder with variable syndromic features, broadening the spectrum of multisystem phenotypes associated with variants in genes encoding G-proteins.

Hepatic hydrothorax represents a rare complication of portal hypertension among adult cirrhotic patients. Here we describe a pediatric case of hepatic hydrothorax, observed in a biliary atresia infant. The child presented with recurrent right side pleural effusion, after a successful Kasai portoenterostomy with restoration of bile flow and without overt signs of hepatic failure. Recurrence of hepatic hydrothorax led the patient to liver transplant despite a low Pediatric End-stage Liver Disease value. Although rare, hepatic hydrothorax can also occur in children and should be suspected in patients with portal hypertension and respiratory distress. HH may be an indication for liver transplantation.

BACKGROUND/OBJECTIVES: Several scoring systems have been specifically developed for risk stratification in COVID-19 patients. DESIGN: We compared, in a cohort of confirmed COVID-19 older patients, three specifically developed scores with a previously established early warning score. Main endpoint was all causes in-hospital death. SETTING: This is a single-center, retrospective observational study, conducted in the Emergency Department (ED) of an urban teaching hospital, referral center for COVID-19. PARTICIPANTS: We reviewed the clinical records of the confirmed COVID-19 patients aged 60 years or more consecutively admitted to our ED over a 6-week period (March 1st to April 15th, 2020). A total of 210 patients, aged between 60 and 98 years were included in the study cohort. MEASUREMENTS: International Severe Acute Respiratory Infection Consortium Clinical Characterization Protocol-Coronavirus Clinical Characterization Consortium (ISARIC-4C) score, COVID-GRAM Critical Illness Risk Score (COVID-GRAM), quick COVID-19 Severity Index (qCSI), National Early Warning Score (NEWS). RESULTS: Median age was 74 (67-82) and 133 (63.3%) were males. Globally, 42 patients (20.0%) deceased. All the score evaluated showed a fairly good predictive value with respect to in-hospital death. The ISARIC-4C score had the highest area under ROC curve (AUROC) 0.799 (0.738-0.851), followed by the COVID-GRAM 0.785 (0.723-0.838), NEWS 0.764 (0.700-0.819), and qCSI 0.749 (0.685-0.806). However, these differences were not statistical significant. CONCLUSION: Among the evaluated scores, the ISARIC-4C and the COVID-GRAM, calculated at ED admission, had the best performance, although the qCSI had similar efficacy by evaluating only three items. However, the NEWS, already widely validated in clinical practice, had a similar performance and could be appropriate for older patients with COVID-19.

ObjectiveTo evaluate whether eculizumab helps patients with anti–acetylcholine receptor–positive (AChR+) refractory generalized myasthenia gravis (gMG) achieve the Myasthenia Gravis Foundation of America (MGFA) post-intervention status of minimal manifestations (MM), we assessed patients' status throughout REGAIN (Safety and Efficacy of Eculizumab in AChR+ Refractory Generalized Myasthenia Gravis) and its open-label extension.MethodsPatients who completed the REGAIN randomized controlled trial and continued into the open-label extension were included in this tertiary endpoint analysis. Patients were assessed for the MGFA post-intervention status of improved, unchanged, worse, MM, and pharmacologic remission at defined time points during REGAIN and through week 130 of the open-label study.ResultsA total of 117 patients completed REGAIN and continued into the open-label study (eculizumab/eculizumab: 56; placebo/eculizumab: 61). At week 26 of REGAIN, more eculizumab-treated patients than placebo-treated patients achieved a status of improved (60.7% vs 41.7%) or MM (25.0% vs 13.3%; common OR: 2.3; 95% CI: 1.1–4.5). After 130 weeks of eculizumab treatment, 88.0% of patients achieved improved status and 57.3% of patients achieved MM status. The safety profile of eculizumab was consistent with its known profile and no new safety signals were detected.ConclusionEculizumab led to rapid and sustained achievement of MM in patients with AChR+ refractory gMG. These findings support the use of eculizumab in this previously difficult-to-treat patient population.ClinicalTrials.gov IdentifierREGAIN, NCT01997229; REGAIN open-label extension, NCT02301624.Classification of EvidenceThis study provides Class II evidence that, after 26 weeks of eculizumab treatment, 25.0% of adults with AChR+ refractory gMG achieved MM, compared with 13.3% who received placebo.

ObjectiveTo clinically characterize patients with anti-metabotropic glutamate receptor (mGluR) 1 encephalitis, to identify prognostic factors, and to study the immunoglobulin G (IgG) subclasses and effects of antibodies on neuronal mGluR1 clusters.MethodsClinical information on new and previously reported patients was reviewed. Antibodies to mGluR1 and IgG subclasses were determined with brain immunohistochemistry and cell-based assays, and their effects on mGluR1 clusters were studied on rat hippocampal neurons.ResultsEleven new patients were identified (10 adults, 1 child);4 were female. In these and 19 previously reported cases (n = 30, median age 55 years), the main clinical manifestation was a subacute cerebellar syndrome that in 25 (86%) patients was associated with behavioral/cognitive changes or other neurologic symptoms. A tumor was found in 3 of 26 (11%). Brain MRI was abnormal in 7 of 19 (37%) at onset and showed cerebellar atrophy in 10 of 12 (83%) at follow-up. Twenty-five of 30 (83%) patients received immunotherapy. Follow-up was available for 25: 13 (52%) had clinical stabilization; 10 (40%) showed significant improvement; and 2 died. At the peak of the disease, patients with bad outcome at 2 years (modified Rankin Scale score > 2, n = 7) were more likely to have higher degree of initial disability, as reflected by a worse Scale for Assessment and Rating of Ataxia score, and more frequent need of assistance to walk. Antibodies to mGluR1 were mainly IgG1 and caused a significant decrease of mGluR1 clusters in cultured neurons.ConclusionsAnti-mGluR1 encephalitis manifests as a severe cerebellar syndrome, often resulting in long-term disability and cerebellar atrophy. The antibodies are pathogenic and cause significant decrease of mGluR1 clusters in cultured neurons.

Alagille syndrome (ALGS) is a multisystemic disease caused by mutations in genes of Notch pathway, which regulates embryonic cell differentiation and angiogenesis. Clinically, ALGS is characterized by cholestasis, cardiac defects, characteristic facial features, skeletal and ophthalmologic abnormalities. The aim of this review is to illustrate neuroradiological findings in ALGS, which are less well-known and prevalent, including cerebrovascular anomalies (such as aneurysms, dolichoectasia, Moyamoya syndrome and venous peculiarities), Chiari 1 malformation, craniosynostosis, intracranial hypertension, and vertebral anomalies (namely butterfly vertebra, hemivertebra, and craniocervical junction anomalies). Rarer cerebral midline malformations and temporal bone anomalies have also been described.

BACKGROUND AND PURPOSE This study was undertaken to assess the long-term outcome of patients with paraneoplastic and non paraneoplastic autoimmune cerebellar ataxia (ACA) using the Scale for the Assessment and Rating of Ataxia (SARA). METHODS Patients with subacute cerebellar ataxia admitted to our institution between September 2012 and April 2020 were prospectively recruited. Serum and/or cerebrospinal fluid was tested for neural autoantibodies by indirect immunofluorescence on mouse brain, cell-based assays, and radioimmunoassay. SARA and modified Rankin Scale (mRS) score were employed to assess patients' outcome. RESULTS Fifty-five patients were recruited, of whom 23 (42%) met the criteria for cerebellar ataxia of autoimmune etiology. Neural autoantibodies were detected in 22 of 23 patients (Yo-immunoglobulin G [IgG], n = 6; glutamic acid decarboxylase 65-IgG, n = 3; metabotropic glutamate receptor 1-IgG, n = 2; voltage-gated calcium channel P/Q type-IgG, n = 2; Hu-IgG, n = 1; glial fibrillary acidic protein-IgG, n = 1; IgG-binding unclassified antigens, n = 7). Thirteen patients were diagnosed with paraneoplastic cerebellar syndrome (PCS) and 10 with idiopathic ACA. All patients received immunotherapy. Median SARA score was higher in the PCS group at all time points (p = 0.0002), while it decreased significantly within the ACA group (p = 0.049) after immunotherapy. Patients with good outcome (mRS ≤ 2) had less neurological disability (SARA

Metallic cerclage cables are reliable and cost-effective internal fixation devices, which are largely used in surgical practice for the treatment of periprosthetic fractures. Nevertheless, complications connected with their use have been described in the literature, including the following: third-body generation, failure and consequent migration, fraying, allergies, and injury to the surgical team. The development of new materials offers alternatives to traditional metallic cables. This study compares the outcomes between two groups of patients affected by periprosthetic hip fractures, treated with titanium cables or with ultra-high-molecular-weight polyethylene (UHMWPe) iso-elastic cables. Our retrospective study aims to compare the clinical and radiological outcomes of titanium cables and UHMWPe iso-elastic cables, isolated or associated with dedicated plates, for the surgical treatment of periprosthetic fractures with stable implants. Two groups of 30 (group A—metallic cables) and 24 (group B—UHMWPe iso-elastic cables) patients have been surgically treated in our institution for hip periprosthetic fractures, between September 2017 and June 2020. The mean age of the patients was 81 years in group A and 80 years in group B. In our study, we included fractures classified as B1 or C, according to the Vancouver postoperative fractures classification; the patients were evaluated retrospectively at 1 year postoperatively, regarding the following: surgery time, blood loss, partial weight-bearing time, radiographical healing time, Harris hip score, and postoperative complications. Comparable outcomes were observed in patients from both groups. Group A showed a higher complication rate compared to group B, at 1 year postoperatively. Non-metallic nylon fiber and ultra-high-molecular-weight polyethylene (UHMWPe) cerclage cables could represent a reliable fixation device, ensuring comparable healing and complication rates with traditional titanium cerclage cables.

Background and ObjectivesTo report a case of anti-NMDAR encephalitis presenting with isolated memory dysfunction.MethodsA 29-year-old woman was admitted to the Neurology Department referring memory impairment with a subacute onset. The initial assessment included EEG, neuropsychological tests, and brain MRI. Serum and CSF samples were collected for immunologic studies. The diagnostic evaluation was completed with a total body PET scan.ResultsPatient's neurologic examination was unremarkable apart from an episodic memory deficit, confirmed by neuropsychological examination. The EEG revealed epileptiform discharges in the temporal lobes, whereas brain MRI showed bilateral temporal lobes hyperintense lesions on fluid-attenuated inversion recovery images and T2-weighted images. NMDAR-IgG was detected in the patient's serum and CSF by cell-based assay confirming the diagnosis of definite anti-NMDAR encephalitis. The total body PET showed only a slight hypometabolism in the right temporal cortex and in the cerebellar hemispheres. After a course of IV immunoglobulin and corticosteroid therapy, a marked improvement of the memory deficit was observed.DiscussionThis case shows that anti-NMDAR encephalitis can present with isolated memory loss. Neural antibody testing in these patients could play a pivotal role in early diagnosis and prompt treatment.

Background: The impact of anterior cruciate ligament reconstruction (ACLR) on the performance and career of professional soccer players has not been extensively investigated. Purpose: To evaluate in professional European soccer players (1) the ACL injury incidence, (2) the return-to-play (RTP) rate and time after ACLR, (3) career survival and athlete performance in the first 3 postoperative seasons after RTP, (4) factors likely related to different outcomes after ACLR, and (5) any related differences between the top 8 European soccer leagues. Study Design: Descriptive epidemiology study. Methods: Included were professional soccer players in the top 8 European Soccer leagues (Serie A [Italy], Premier League [England], Ligue 1 [France], LaLiga [Spain], Bundesliga [Germany], Jupiler Pro League [Belgium], Liga NOS [Portugal], and Premier Liga [Russia]) who sustained an ACL injury during seasons 2014 to 2015, 2015 to 2016, and 2016 to 2017. Data were retrieved from publicly available online sources. Outcomes were evaluated based on player age (30 years), position (goalkeeper, defender, midfielder, and forward), affected side (dominant vs nondominant), and league. Results: Overall, 195 players sustained an ACL injury, for a mean annual ACL injury incidence of 1.42%. The RTP rate was 95%, with a mean RTP time of 248 ± 136 days. Within the third postoperative season, 66 players (36%) competed in a lower level national league, and 25 (13.6%) ended their careers; a significant reduction in the mean minutes played per season was found in all 3 postoperative seasons. Player age correlated significantly with reduction in performance or recovery from an ACL injury. No significant correlation was found between postoperative player performance and affected side, position, league, or time to RTP. Conclusion: A substantial ACL injury incidence was found in top European elite soccer players; however, a high RTP rate in a reasonable time was seen after ACLR. Nevertheless, professional soccer players experienced a short-term decline in their performance.

Autoantibodies against the extracellular domain of the N-methyl-d-aspartate receptor (NMDAR) NR1 subunit cause a severe and common form of encephalitis. To better understand their generation, we aimed to characterize and identify human germinal centres actively participating in NMDAR-specific autoimmunization by sampling patient blood, CSF, ovarian teratoma tissue and, directly from the putative site of human CNS lymphatic drainage, cervical lymph nodes. From serum, both NR1-IgA and NR1-IgM were detected more frequently in NMDAR-antibody encephalitis patients versus controls (both P

Autoimmune encephalitis associated with antibodies against neuronal surface targets (NSAE) are rare but still underrecognized conditions that affect adult and pediatric patients. Clinical guidelines have recently been published with the aim of providing diagnostic clues regardless of antibody status. These syndromes are potentially treatable but the choice of treatment and its timing, as well as differential diagnoses, long-term management, and clinical and paraclinical follow-up, remain major challenges. In the absence of evidence-based guidelines, management of these conditions is commonly based on single-center expertise. Taking into account different published expert recommendations in addition to the multicenter experience of the Italian Working Group on Autoimmune Encephalitis, both widely accepted and critical aspects of diagnosis, management and particularly of immunotherapy for NSAE have been reviewed and are discussed. Finally, we provide consensus-based practical advice for managing hospitalization and follow-up of patients with NSAE.

Ultrasound scan is a painless and radiation-free imaging modality and, therefore, it is widely considered the first-choice diagnostic tool in the setting of hepatopathies in paediatric patients. This article focuses on the normal ultrasound anatomy of the liver in neonatal and paediatric age and reviews the ultrasound appearance of the most common diffuse and focal liver affections.

In this retrospective multicenter study, we evaluated the safety of SARS-CoV-2 vaccination in patients harboring autoantibodies targeting neuronal surface and/or synaptic antigens.From eight Italian Neurology Units, we included patients with: a) serum and/or CSF positivity for specific neuronal autoantibodies; b) a compatible neurological syndrome; and c) available follow-up ≥6 weeks after vaccination with any of the approved SARS-CoV-2 vaccines. Demographics, clinical data, and information regarding previous SARS-CoV-2 infection and vaccination were collected. Disease relapses were considered "post-infectious" or "post-vaccination" when occurring within 6 weeks from infection/vaccination.We included 66 patients; 7/66 (11%) had a previous history of SARS-CoV-2 infection and 1/7 (14%) had post-infection relapses. BNT162b2-Pfizer-BioNTec was administered in 55 cases (83.3%) and mRNA-1273-Moderna in 11 (16.7%). The median number of doses administered per patient was 2 (1-3) and50% of patients did not experience side effects. Five patients (8%) had post-vaccination relapses (seizure 3/5); 4/5 improved after immunotherapy, while one did not receive immunotherapy and worsened. Patients with post-vaccination relapses had higher disability scores at vaccination (p = 0.025), a trend favoring Leucine-rich glioma-inactivated protein 1 LGI1 glutamic acid decarboxylase 65 (GAD65) antibodies (p = 0.054) and shorter time from last relapse (p = 0.057).Our data support the safety of SARS-CoV-2 vaccines in patients with neurological disorders associated with antibodies to neuronal and synaptic antigens.

Introduction: Up to 30% of patients with spinal cord injury and to 20% of patients with traumatic brain injury develop neurogenic heterotopic ossification (NHO). Patients develop restriction in joint range of motion (ROM) and impairment in activities of daily life. When neurological recovery occurs, joints dysfunction represents the cause for patients’ autonomy loss. Case Report: We present the case of a 39-year-old Caucasian male involved in a car accident and experienced 14 days of post-traumatic coma. After rehabilitation, no residual motor or sensory neurological deficit was present, but bilateral NHOs surrounding hip joints developed. Seventeen months after trauma, the patient was admitted to our institute. He was confined to bed, absolutely unable to walk, stand or sit. Radiological evaluation consisted in Antero-Posterior X-ray view only, due to the inability to open up his hips for lateral views, and 3D computed tomography scan. “Functional resection” of the ossifications was performed and rehabilitation started from day 1 after surgery. At the final follow-up 24 months from the second operation high grade of clinical satisfaction was reported. The patients were able to walk independently, to put on his socks, and to drive a car and bike. Painless right and left hip ROM was, respectively, 100° and 90° for flexion, 10° for extension, 35° and 30° for abduction, and near normal internal and external rotation. The patient referred that further improvement in mobility and fluency was still present day by day. Conclusion: The rarity of the condition frequently bring to a delay in treatment and the absence of specific guidelines made treatment still dependent on surgeon experiences. A multidisciplinary approach is essential for success. Surgeons should be aware that it is important to refer patients to specialized center, because early resection could provide excellent results, preserving hip, and restoring function and patient independency. Keywords: hip, ossifica

Background and Aims: Disease progression in children with primary sclerosing cholangitis (PSC) is variable. Prognostic and risk-stratification tools exist for adult-onset PSC, but not for children. We aimed to create a tool that accounts for the biochemical and phenotypic features and early disease stage of pediatric PSC. Approach and Results: We used retrospective data from the Pediatric PSC Consortium. The training cohort contained 1,012 patients from 40 centers. We generated a multivariate risk index (Sclerosing Cholangitis Outcomes in Pediatrics [SCOPE] index) that contained total bilirubin, albumin, platelet count, gamma glutamyltransferase, and cholangiography to predict a primary outcome of liver transplantation or death (TD) and a broader secondary outcome that included portal hypertensive, biliary, and cancer complications termed hepatobiliary complications (HBCs). The model stratified patients as low, medium, or high risk based on progression to TD at rates of

Kasai portoenterostomy (KP) represents the first-line treatment for biliary atresia (BA). The purpose was to compare the accuracy of quantitative parameters extracted from laboratory tests, US imaging, and MR imaging studies using machine learning (ML) algorithms to predict the long-term medical outcome in native liver survivor BA patients after KP. Twenty-four patients were evaluated according to clinical and laboratory data at initial evaluation (median follow-up = 9.7 years) after KP as having ideal (n = 15) or non-ideal (n = 9) medical outcomes. Patients were re-evaluated after an additional 4 years and classified in group 1 (n = 12) as stable and group 2 (n = 12) as non-stable in the disease course. Laboratory and quantitative imaging parameters were merged to test ML algorithms. Total and direct bilirubin (TB and DB), as laboratory parameters, and US stiffness, as an imaging parameter, were the only statistically significant parameters between the groups. The best algorithm in terms of accuracy, sensitivity, specificity, and AUCROC was naive Bayes algorithm, selecting only laboratory parameters (TB and DB). This preliminary ML analysis confirms the fundamental role of TB and DB values in predicting the long-term medical outcome for BA patients after KP, even though their values may be within the normal range. Physicians should be alert when TB and DB values change slightly.