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排序方式: 共有3551条查询结果,搜索用时 15 毫秒
31.
Mura Eleonora Masnada Silvia Antonello Clara Parazzini Cecilia Izzo Giana Garau Jessica Sproviero Daisy Cereda Cristina Orcesi Simona Veggiotti Pierangelo Zuccotti Gianvincenzo Dilillo Dario Penagini Francesca Tonduti Davide 《Metabolic brain disease》2021,36(5):859-863
Metabolic Brain Disease - Aicardi-Goutières Syndrome (AGS) is a monogenic leukodystrophy with pediatric onset, clinically characterized by a variable degree of neurologic impairment. It... 相似文献
32.
Pietro Palmisano MD Matteo Ziacchi MD Ernesto Ammendola MD Antonio D'Onofrio MD Gabriele Dell'Era MD Mattia Laffi MD Mauro Biffi MD Gerardo Nigro MD PhD Walter Bianchi MD Eleonora Prenna MD Andrea Angeletti MD Alessandro Guido MD Giulia Stronati MD Germano Gaggioli MD Antonio Dello Russo MD Michele Accogli MD Federico Guerra MD Italian Association of Arrhythmology Cardiac Pacing 《Journal of cardiovascular electrophysiology》2021,32(6):1712-1723
33.
Krengli Marco Ferrara Eleonora Guaschino Riccardo Puta Erinda Turri Lucia Luciani Ilaria Sacchetti Gian Mauro Franco Pierfrancesco Brambilla Marco 《Annals of nuclear medicine》2022,36(5):450-459
Annals of Nuclear Medicine - [18F] fluorodeoxyglucose positron emission tomography/computed tomography ([18F] FDG-PET/CT) is used for diagnosis, staging, response assessment and prognosis... 相似文献
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Alberto Verrotti Eleonora A. Grasso Marta Cacciatore Sara Matricardi Pasquale Striano 《Acta neurologica Scandinavica》2021,143(1):19-26
Brivaracetam (BRV) is a new antiseizure medication (ASM) that is currently approved for adjunctive treatment in patients with focal onset seizures. Similarly to levetiracetam (LEV), BRV works by binding SV2A vesicles with a high affinity and a linear pharmacokinetic profile. Retrospective studies and randomized clinical trials have already proven the efficacy of BRV, even in patients who failed treatment with LEV. Most studies about the efficacy and tolerability conducted so far were performed in adult cohorts, whereas few studies have been performed in children; however, BRV was proven to be a useful ASM for pediatric focal epilepsies, with fewer studies and conflicting results among patients with generalized epilepsies and epileptic syndromes. Retention rates were high in the cohorts analyzed, and no serious treatment‐emergent adverse events were reported in the majority of patients, with somnolence, drowsiness, irritability, aggression, and decreased appetite being the most frequently reported side effects. Although there are few original papers published on the subject so far, the analysis of the literature data demonstrated the efficacy and safety of BRV in pediatric patients, with more evidence for children aged 4‐16 years with an onset of focal seizures. However, a positive response was also achieved in patients affected by encephalopathic epilepsies (eg, Jeavons' epilepsy, Dravet syndrome, Lennox‐Gastaut syndrome, and juvenile myoclonic epilepsy), and ongoing studies are now testing BRV in order to widen its application to other forms of epilepsy and to test its effectiveness when used in monotherapy. This review aims to provide a comprehensive analysis of the literature surrounding the efficacy and tolerability of BRV for pediatric patients. 相似文献
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De Matteis Eleonora Affaitati Giannapia Frattale Ilaria Caponnetto Valeria Pistoia Francesca Giamberardino Maria Adele Sacco Simona Ornello Raffaele 《Neurological sciences》2021,42(8):3297-3303
Neurological Sciences - Monoclonal antibodies targeting the calcitonin gene-related peptide, including erenumab, are migraine-specific preventive treatments, whose long-term effectiveness has still... 相似文献
39.
Roberta Biancheri Camillo Rosano Laura Denegri Eleonora Lamantea Francesca Pinto Federica Lanza Mariasavina Severino Mirella Filocamo 《European journal of human genetics : EJHG》2013,21(1):34-39
Homozygous or compound heterozygous mutations in the GJC2 gene, encoding the gap
junction protein connexin47 (Cx47), cause the autosomal recessive hypomyelinating
Pelizaeus–Merzbacher-like disease (PMLD1, MIM# 608804). Although clinical and
neuroradiological findings resemble those of the classic Pelizaeus–Merzbacher
disease, PMLD patients usually show a greater level of cognitive and motor functions.
Unpredictably a homozygous missense GJC2 mutation (p.Glu260Lys) was found in a
patient presenting with a very severe clinical picture characterised by congenital
nystagmus and severe neurological impairment. Also magnetic resonance imaging was
unusually severe, showing an abnormal supra- and infratentorial white matter involvement
extending to the spinal cord. The novel p.Glu260Lys (c.778G>A) mutation, occurring in a
highly conserved motif (SRPTEK) of the Cx47 extracellular loop-2 domain, was
predicted, by modelling analysis, to break a ‘salt bridge network'', crucial
for a proper connexin–connexin interaction to form a connexon, thus hampering the
correct formation of the connexon pore. The same structural analysis, extended to the
previously reported missense mutations, predicted that most changes were expected to have
less severe impact on protein functions, correlating with the mild PMLD1 form of the
patients. Our study expands the spectrum of PMLD1 and provides evidence that the extremely
severe clinical and neuroradiological PMLD1 form of our patient likely correlates with the
predicted impairment of gap junction channel assembly resulting from the detrimental
effect of the new p.Glu260Lys mutant allele on Cx47 protein. 相似文献
40.
Laura Kropp Anish Baswanth Chakka Svetlana Yatsenko Eleonora Di Gregorio Daniela Lacerenza Giovanna Vaula Flavia Talarico Paola Mandich Camilo Toro Eleonore Eymard Pierre Pierre Labauge Sabina Capellari Pietro Cortelli Filippo Pinto Vairo Diego Miguel Danielle Stubbolo Lourenco Charles Marques William Gahl Odile Boespflug‐Tanguy Atle Melberg Sharon Hassin‐Baer Oren S. Cohen Rastislav Pjontek Armin Grau Thomas Klopstock Brent Fogel Inge Meijer Guy Rouleau Jean‐Pierre L. Bouchard Madhavi Ganapathiraju Adeline Vanderver Niklas Dahl Grace Hobson Alfredo Brusco Quasar Saleem Padiath 《Human mutation》2013,34(8):1160-1171
Autosomal dominant leukodystrophy (ADLD) is an adult onset demyelinating disorder that is caused by duplications of the lamin B1 (LMNB1) gene. However, as only a few cases have been analyzed in detail, the mechanisms underlying LMNB1 duplications are unclear. We report the detailed molecular analysis of the largest collection of ADLD families studied, to date. We have identified the minimal duplicated region necessary for the disease, defined all the duplication junctions at the nucleotide level and identified the first inverted LMNB1 duplication. We have demonstrated that the duplications are not recurrent; patients with identical duplications share the same haplotype, likely inherited from a common founder and that the duplications originated from intrachromosomal events. The duplication junction sequences indicated that nonhomologous end joining or replication‐based mechanisms such fork stalling and template switching or microhomology‐mediated break induced repair are likely to be involved. LMNB1 expression was increased in patients’ fibroblasts both at mRNA and protein levels and the three LMNB1 alleles in ADLD patients show equal expression, suggesting that regulatory regions are maintained within the rearranged segment. These results have allowed us to elucidate duplication mechanisms and provide insights into allele‐specific LMNB1 expression levels. 相似文献