Foundation Neurological Institute C Mondino

Pavia, Italy

Foundation Neurological Institute C Mondino

Pavia, Italy
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D'Aguanno S.,Cervello | D'Aguanno S.,University of Rome Tor Vergata | Franciotta D.,Foundation Neurological Institute C Mondino | Lupisella S.,Cervello | And 15 more authors.
Neuroscience Letters | Year: 2010

Protein profiling of cerebrospinal fluid in Guillain-Barrè syndrome (GBS), an acute and immune-mediated disease affecting the peripheral nervous system, was performed by two-dimensional electrophoresis. Significant modulated spots in GBS patients vs. control groups (a group of multiple sclerosis patients and one of healthy donors) underwent MALDI-TOF/TOF investigation. Inflammation-related proteins, such as vitamin D-binding protein, beta-2 glycoprotein I (ApoH), and a complement component C3 isoform were up-regulated in GBS, whereas transthyretin (the monomer and the dimer forms), apolipoprotein E, albumin and five of its fragments were down-regulated. Then, we used an isoelectric-focusing-dinitrophenylhydrazine-based technique to analyse the extent of carbonylation and, as a result, of oxidative damage of GBS CSF proteome. We observed a major sensitivity to carbonylation for albumin and alpha-glycoprotein in inflammation and a selective increase of reactivity for a glycosylated Fab from an IgM globulin in GBS CSF. Our results add new proteins to candidate CSF features of GBS, and suggest that oxidative stress could contribute to the immunopathological mechanisms in this syndrome. © 2010 Elsevier Ireland Ltd.


Cova E.,Foundation Neurological Institute C Mondino | Bongioanni P.,Neurorehabilitation Unit | Cereda C.,Foundation Neurological Institute C Mondino | Metelli M.R.,University of Pisa | And 5 more authors.
Neurochemistry International | Year: 2010

Oxidative stress markers have been found in nervous and peripheral tissues of familial and sporadic amyotrophic lateral sclerosis patients. Here, we evaluated the activity of some antioxidant enzymes glutathione peroxidase, glutathione reductase and Cu-Zn superoxide dismutase in erythrocyte, the marker of non-enzymatic antioxidant response (total antioxidant status), as well as plasma reactive oxygen species, at the enrolment and during disease progression in 88 patients affected by the sporadic form of amyotrophic lateral sclerosis. Our study has been performed along 72 months by grouping the patients according to the ALS functional rating score or rate of disease progression. Our results showed a significant impairment of erythrocytes glutathione peroxidase activity in all groups of patients that remained low during disease time course. SOD1 activity significantly decreased along disease course in subjects with a more impaired functional status. A decreasing activity of all assayed enzymes was found in patients who have a faster disease progression rate. By this work we have the evidence that different ALS phenotypes present with different profile of enzymatic and non-enzymatic antioxidant response. © 2010 Elsevier Ltd.


Ragona F.,Irccs Foundation Neurological Institute C Besta | Granata T.,Irccs Foundation Neurological Institute C Besta | Bernardina B.D.,University of Verona | Offredi F.,University of Verona | And 17 more authors.
Epilepsia | Year: 2011

Purpose: To clarify the role of epilepsy and genetic background in determining the cognitive outcome of patients with Dravet syndrome. Methods: In this retrospective study, we reviewed the clinical history and cognitive development of 26 patients who had been followed with standardized evaluations since seizure onset. The cognitive outcome was quantified as differential general quotient (dGQ) between ages 12 and 60 months. Statistical analysis correlated the dGQ with genotype and epilepsy course. Key Findings: Epilepsy started at the mean age of 5.6 months. All patients experienced prolonged convulsive seizures, whereas absences and myoclonus were reported in 17. Cognitive outcome was poor in almost all patients; the mean dGQ was 33 points, varying from 6-77 points. The analysis of individual cognitive profiles identified seven patients in whom the dGQ was <20 points; the main clinical characteristic in this subset of patients was lack of early absences and myoclonus. The statistical analysis of the whole series failed to reveal significant differences in cognitive outcome with regard to the presence of SCN1A mutations and their type. In particular, mutation-carrier patients with the best cognitive outcome harbored either missense or truncating mutations. Significance: Dravet syndrome encompasses different epileptic and cognitive phenotypes that probably result from both genetic and epigenetic factors. In this series, early appearance of myoclonus and absences was associated with the worst cognitive outcome. © 2011 International League Against Epilepsy.


Cianchetti C.,Clinic of Child and Adolescent Neuropsychiatry | Messina P.,IRCSS Instituto for Pharmacological Research Mario Negri | Pupillo E.,IRCSS Instituto for Pharmacological Research Mario Negri | Crichiutti G.,University of Santa María in Ecuador | And 8 more authors.
Seizure | Year: 2015

Purpose: The assessment of the quality of life (QoL) is relevant for a comprehensive treatment of patients with epilepsy. In children and adolescents, an impact of epilepsy on the QoL of the entire family is expected. Methods: We asked 293 parents of children and adolescents with epilepsy, included in an observational study on treatment satisfaction, to evaluate the impact of the disease on several aspects of the QoL of the whole family using a specifically organized questionnaire (IEQoL). Results: The degree of parents' concerns about epilepsy and the severity of the disease correlated with a deterioration of QoL in both the children and the family. This involved all aspects of QoL (conflicts within the family, job, leisure activities, peer relationship, economy) although to a different degree. Parents frequently admitted increased apprehensiveness, even when not justified by the low severity of the disease. There was general agreement between parents and their adolescent children, although in a few cases adolescents overrated their school and daily performance in respect to the parents, suggesting a tendency to overlook their problems. Conclusion: Epilepsy impairs all aspects of QoL, although at different degree, both in children/ adolescents and in their families. Parental apprehensiveness appears to have a role on this, and it may not reflect the severity of the disease. © 2014 Published by Elsevier Ltd on behalf of British Epilepsy Association.


PubMed | G Salesi Pediatric Hospital, University of Genoa, University of Bari, IRCSS Instituto for Pharmacological Research Mario Negri and 5 more.
Type: | Journal: Seizure | Year: 2015

The assessment of the quality of life (QoL) is relevant for a comprehensive treatment of patients with epilepsy. In children and adolescents, an impact of epilepsy on the QoL of the entire family is expected.We asked 293 parents of children and adolescents with epilepsy, included in an observational study on treatment satisfaction, to evaluate the impact of the disease on several aspects of the QoL of the whole family using a specifically organized questionnaire (IEQoL).The degree of parents concerns about epilepsy and the severity of the disease correlated with a deterioration of QoL in both the children and the family. This involved all aspects of QoL (conflicts within the family, job, leisure activities, peer relationship, economy) although to a different degree. Parents frequently admitted increased apprehensiveness, even when not justified by the low severity of the disease. There was general agreement between parents and their adolescent children, although in a few cases adolescents overrated their school and daily performance in respect to the parents, suggesting a tendency to overlook their problems.Epilepsy impairs all aspects of QoL, although at different degree, both in children/adolescents and in their families. Parental apprehensiveness appears to have a role on this, and it may not reflect the severity of the disease.


Beghi E.,Mario Negri Institute for Pharmacological Research | Messina P.,Mario Negri Institute for Pharmacological Research | Pupillo E.,Mario Negri Institute for Pharmacological Research | Crichiutti G.,University of Santa María in Ecuador | And 6 more authors.
Epilepsy Research | Year: 2012

Purpose: To assess incidence, indicators and outcome of satisfaction with antiepileptic drugs in children. Methods: Multicenter, observational, open, prospective survey of children and adolescents with epilepsy with three-month follow-up. Included were patients aged 3-17 years with newly diagnosed (" new diagnosis") or chronic epilepsy (" old diagnosis") requiring treatment start or change. Satisfaction was assessed with the Hedonic Visual Scale or direct questions, depending on patient's age. Quality of life of adolescents (QOLIE-48) and of caregivers (SF-36) and predictors of (dis)satisfaction were also assessed. Results: 293 patients completed the study. Most had generalized idiopathic epilepsy, and a disease lasting <12 months. Newly diagnosed patients were 60.8%. Patients declaring satisfaction were 70.6% at one month and 75.8% at three months. Compared to old diagnosis, new diagnosis carried a higher satisfaction rate and improved satisfaction at end of follow-up. Independent predictors of dissatisfaction were an old diagnosis, adverse events and SF-36 score. The latter remained the only independent predictor of persisting dissatisfaction when adjusting for the presence of and the interaction with adverse events. Conclusions: About one-fourth of children and adolescents with epilepsy are dissatisfied with treatment. Chronic epilepsy, adverse events, and parents/caregivers with poor quality of life predict dissatisfaction. © 2012 Elsevier B.V.

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