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Bucello  Sebastiano  Annovazzi  Pietro  Ragonese  Paolo  Altieri  Marta  Barcella  Valeria  Bergamaschi  Roberto  Bianchi  Alessia  Borriello  Giovanna  Buscarinu  Maria Chiara  Callari  Graziella  Capobianco  Marco  Capone  Fioravante  Cavalla  Paola  Cavarretta  Rosella  Cortese  Antonio  De Luca  Giovanna  Di Filippo  Massimiliano  Dattola  Vincenzo  Fantozzi  Roberta  Ferraro  Elisabetta  Filippi  Maria Maddalena  Gasperini  Claudio  Grimaldi  Luigi Maria Edoardo  Landi  Doriana  Re  Marianna Lo  Mallucci  Giulia  Manganotti  Paolo  Marfia  Girolama Alessandra  Mirabella  Massimiliano  Perini  Paola  Pisa  Marco  Realmuto  Sabrina  Russo  Margherita  Tomassini  Valentina  Torri-Clerici  Valentina Liliana Adriana  Zaffaroni  Mauro  Zuliani  Cristina  Zywicki  Sofia  Filippi  Massimo  Prosperini  Luca 《Journal of neurology》2021,268(8):2922-2932
Journal of Neurology - To identify baseline factors associated with disease activity in patients with relapsing–remitting multiple sclerosis (RRMS) under teriflunomide treatment. This was an...  相似文献   
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Aims To review the literature on mortality among dependent or regular users of opioids across regions, according to specific causes, and related to a number of demographic and clinical variables. Methods Multiple search strategies included searches of Medline, EMBASE and PsycINFO, consistent with the methodology recommended by the Meta‐analysis of Observational Studies in Epidemiology (MOOSE) group; grey literature searches; and contact of experts for any additional unpublished data from studies meeting inclusion criteria. Random‐effects meta‐analyses were conducted for crude mortality rates (CMRs) and standardized mortality ratios (SMRs), with stratified analyses where possible. Meta‐regressions examined potentially important sources of heterogeneity across studies. Results Fifty‐eight prospective studies reported mortality rates from opioid‐dependent samples. Very high heterogeneity across studies was observed; pooled all‐cause CMR was 2.09 per 100 person‐years (PY; 95% CI; 1.93, 2.26), and the pooled SMR was 14.66 (95% CI: 12.82, 16.50). Males had higher CMRs and lower SMRs than females. Out‐of‐treatment periods had higher mortality risk than in‐treatment periods (pooled RR 2.38 (CI: 1.79, 3.17)). Causes of death varied across studies, but overdose was the most common cause. Multivariable regressions found the following predictors of mortality rates: country of origin; the proportion of sample injecting; the extent to which populations were recruited from an entire country (versus subnational); and year of publication. Conclusions Mortality among opioid‐dependent users varies across countries and populations. Treatment is clearly protective against mortality even in non‐randomized observational studies. Study characteristics predict mortality levels; these should be taken into account in future studies.  相似文献   
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Among postural abnormalities in Parkinson’s disease (PD), striatal hand (SH) is a particularly underexplored phenomenon. It leads to extreme abnormalities of hand posture, causing altered dexterity, pain and disfigurement. In our study, three blinded investigators examined several pictures of the hands of individuals with PD (N = 40) and controls (N = 15). The investigators quantified postural alterations using the Striatal Hand Score. Demographic and clinical data were also collected. As no differences were detected among investigators agreement, a final Hand Score (HS, range 0–4) was obtained for each hand. The Striatal Hand Score in both the left and right hand was significantly different in PD compared to controls (p < 0.001 for both left and right hand). Striatal hand was significantly worse on the side of PD onset, and on the side with greater PD symptomatology. The finding of a striatal hand was 100 % specific for a diagnosis of PD. Nine PD subjects were evaluated both on and off medication, and dopaminergic treatment did not significantly change the Striatal Hand Score. Our findings suggest that in patients without any explanation for hand deformities other than PD, striatal hand occurs very often, and is highly specific for the side of worst PD involvement. We recommend including an evaluation for SH as part of routine practice. This study emphasizes the importance of a careful observation of the patient in order to improve diagnostic accuracy.  相似文献   
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A sixth month phase II multicenter-pilot trial with a low dose of the opiate antagonist Naltrexone (LDN) has been carried out in 40 patients with primary progressive multiple sclerosis (PPMS). The primary end points were safety and tolerability. Secondary outcomes were efficacy on spasticity, pain, fatigue, depression, and quality of life. Clinical and biochemical evaluations were serially performed. Protein concentration of beta-endorphins (BE) and mRNA levels and allelic variants of the mu-opiod receptor gene (OPRM1) were analyzed. Five dropouts and two major adverse events occurred. The remaining adverse events did not interfere with daily living. Neurological disability progressed in only one patient. A significant reduction of spasticity was measured at the end of the trial. BE concentration increased during the trial, but no association was found between OPRM1 variants and improvement of spasticity. Our data clearly indicate that LDN is safe and well tolerated in patients with PPMS.  相似文献   
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Sangalli  F.  Moiola  L.  Bucello  S.  Annovazzi  P.  Rizzo  A.  Radaelli  M.  Vitello  G.  Grimaldi  L. M. E.  Ghezzi  A.  Martinelli  V.  Comi  G. 《Neurological sciences》2010,31(3):299-306
The X-linked genetic Fabry disease causes multiorgan lesions due to intracellular storage of the substrate globotriaosylceramide. Neurological involvement ranges from painful, small fiber neuropathy to cerebrovascular disorders to multifocal aggressive forms. Disease identification through proper differential diagnosis and timely assessment of organ damage should guide a careful treatment planning. Mainstay treatment, include enzyme replacement and support therapy. Neurologists have a pivotal role in early instrumental and clinical detection of organ damage. A panel of experts has developed a set of consensus recommendations to guide the approach of neurologists to Fabry disease.  相似文献   
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