Database : MEDLINE
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[PMID]: 27085208
[Au] Autor:Peters TL; Beard JD; Umbach DM; Allen K; Keller J; Mariosa D; Sandler DP; Schmidt S; Fang F; Ye W; Kamel F
[Ad] Address:Epidemiology Branch, National Institute of Environmental Health Sciences, NIH, DHHS, Research Triangle Park, NC, USA; Department of Medical Epidemiology and Biostatistics, Karolinska Institutet, Stockholm, Sweden....
[Ti] Title:Blood levels of trace metals and amyotrophic lateral sclerosis.
[So] Source:Neurotoxicology;54:119-26, 2016 May.
[Is] ISSN:1872-9711
[Cp] Country of publication:Netherlands
[La] Language:eng
[Ab] Abstract:Some trace metals may increase risk of amyotrophic lateral sclerosis (ALS), whereas others may be beneficial. Our goal was to examine associations of ALS with blood levels of selenium (Se), zinc (Zn), copper (Cu), and manganese (Mn). We conducted a case-control study of 163 neurologist confirmed patients from the National Registry of Veterans with ALS and 229 frequency-matched veteran controls. We measured metal levels in blood using inductively coupled plasma mass spectrometry and estimated odds ratios (ORs) and 95% confidence intervals (CIs) for associations between ALS and a doubling of metal levels using unconditional logistic regression, adjusting for age, gender, and race/ethnicity. ALS was inversely associated with both Se (OR=0.4, 95% CI: 0.2-0.8) and Zn (OR=0.4, 95% CI: 0.2-0.8). Inverse associations with Se were stronger in patients with bulbar compared to spinal onset, worse function, longer diagnostic delay, and longer collection delay; inverse associations with Zn were stronger for those with worse function and longer collection delay. In contrast, ALS was positively associated with Cu (OR=3.4, 95% CI: 1.5-7.9). For Mn, no linear trend was evident (OR=0.9, 95% CI: 0.6-1.3, Ptrend=0.51). Associations of Se, Zn, Cu, and Mn with ALS were independent of one another. Adjustment for lead levels attenuated the positive association of ALS with Cu but did not change associations with Se, Zn, or Mn. In conclusion, Se and Zn were inversely associated with ALS, particularly among those with worse function, suggesting that supplementation with these metals may benefit such patients, while Cu was positively associated with ALS. Deficiencies of Se and Zn and excess Cu may have a role in ALS etiology.
[Pt] Publication type:JOURNAL ARTICLE
[Em] Entry month:1605
[Js] Journal subset:IM
[St] Status:In-Data-Review

  2 / 198803 MEDLINE  
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[PMID]: 27064831
[Au] Autor:Borzì G; Di Gennaro G; Schmitt FC; D'Aniello A; Mumoli L; Zummo L; Daniele O; Russo E; Gambardella A; Labate A
[Ad] Address:Institute of Neurology, University "Magna Graecia", Catanzaro, Italy....
[Ti] Title:Lacosamide in patients with temporal lobe epilepsy: An observational multicentric open-label study.
[So] Source:Epilepsy Behav;58:111-4, 2016 May.
[Is] ISSN:1525-5069
[Cp] Country of publication:United States
[La] Language:eng
[Ab] Abstract:PURPOSE: The aim of this study was to evaluate the efficacy and tolerability of lacosamide (LCM) both as add-on therapy and monotherapy in patients with temporal lobe epilepsy (TLE) based on an observational, prospective, multicenter study. METHODS: We enrolled 100 patients (mean age: 43.4±12.53years, 57 females) with nonlesional TLE and TLE with hippocampal sclerosis (HS) that did not respond to the first drug and who were referred to epilepsy centers of the University of Catanzaro, University of Palermo, IRCSS Neuromed of Pozzilli, and Otto-von-Guericke University of Magdeburg. In this open-label, multicenter trial, patients were initiated on oral LCM as add-on therapy to first AED monotherapy or as a later add-on to two concomitant AEDs. Seizure frequency changes and adverse events were recorded for at least six months after LCM was added. RESULTS: Fourteen patients dropped out because of positive MRI findings other than HS. Patients received LCM at 200-400mg/day. Fifty-eight out of these 86 patients with seizures that were previously drug-resistant had reduced seizure frequency after introduction of LCM. Forty-five out of 86 patients were classified as responders (12 were seizure-free, 33 achieved a reduction >50%). Interestingly, five patients out of 86 achieved seizure freedom for at least one year and progressively switched to monotherapy with LCM, and all five remained seizure-free at follow-up (6-48months). CONCLUSIONS: Our results may suggest that LCM at doses of 200 to 400mg/day reduces seizure frequency in adults with TLE regardless of the presence of HS, and that it may be considered as a first add-on treatment for patients with pharmacoresistant TLE.
[Pt] Publication type:JOURNAL ARTICLE
[Em] Entry month:1605
[Js] Journal subset:IM
[St] Status:In-Data-Review

  3 / 198803 MEDLINE  
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[PMID]: 27064828
[Au] Autor:Maulisova A; Korman B; Rey G; Bernal B; Duchowny M; Niederlova M; Krsek P; Novak V
[Ad] Address:Charles University, Faculty of Arts, Department of Psychology, Prague, Czech Republic; Motol University Hospital, Prague, Czech Republic....
[Ti] Title:Atypical language representation in children with intractable temporal lobe epilepsy.
[So] Source:Epilepsy Behav;58:91-6, 2016 May.
[Is] ISSN:1525-5069
[Cp] Country of publication:United States
[La] Language:eng
[Ab] Abstract:This study evaluated language organization in children with intractable epilepsy caused by temporal lobe focal cortical dysplasia (FCD) alone or dual pathology (temporal lobe FCD and hippocampal sclerosis, HS). We analyzed clinical, neurological, fMRI, neuropsychological, and histopathologic data in 46 pediatric patients with temporal lobe lesions who underwent excisional epilepsy surgery. The frequency of atypical language representation was similar in both groups, but children with dual pathology were more likely to be left-handed. Atypical receptive language cortex correlated with lower intellectual capacity, verbal abstract conceptualization, receptive language abilities, verbal working memory, and a history of status epilepticus but did not correlate with higher seizure frequency or early seizure onset. Histopathologic substrate had only a minor influence on neuropsychological status. Greater verbal comprehension deficits were noted in children with atypical receptive language representation, a risk factor for cognitive morbidity.
[Pt] Publication type:JOURNAL ARTICLE
[Em] Entry month:1605
[Js] Journal subset:IM
[St] Status:In-Data-Review

  4 / 198803 MEDLINE  
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[PMID]: 26286698
[Au] Autor:Bhargava P; Steele SU; Waubant E; Revirajan NR; Marcus J; Dembele M; Cassard SD; Hollis BW; Crainiceanu C; Mowry EM
[Ad] Address:Department of Neurology, Johns Hopkins University, Baltimore, MD, USA....
[Ti] Title:Multiple sclerosis patients have a diminished serologic response to vitamin D supplementation compared to healthy controls.
[So] Source:Mult Scler;22(6):753-60, 2016 May.
[Is] ISSN:1477-0970
[Cp] Country of publication:England
[La] Language:eng
[Ab] Abstract:BACKGROUND: Vitamin D insufficiency is a risk factor for multiple sclerosis (MS), and patients do not always show the expected response to vitamin D supplementation. OBJECTIVE: We aimed to determine if vitamin D supplementation leads to a similar increase in serum 25-hydroxyvitamin-D (25(OH)D) levels in patients with MS and healthy controls (HCs). METHODS: Participants in this open-label study were female, white, aged 18-60 years, had 25(OH)D levels ⩽ 75 nmol/l at screening, and had relapsing-remitting MS (RRMS) or were HCs. Participants received 5000 IU/day of vitamin D3 for 90 days. Utilizing generalized estimating equations we examined the relationship between the primary outcome (serum 25(OH)D level) and the primary (MS versus HC status) and secondary predictors. RESULTS: For this study 27 MS patients and 30 HCs were enrolled. There was no significant difference in baseline 25(OH)D level or demographics except for higher body mass index (BMI) in the MS group (25.3 vs. 23.6 kg/m(2), p=0.035). In total, 24 MS subjects and 29 HCs completed the study. In a multivariate model accounting for BMI, medication adherence, and oral contraceptive use, MS patients had a 16.7 nmol/l (95%CI: 4.2, 29.2, p=0.008) lower increase in 25(OH)D levels compared with HCs. CONCLUSIONS: Patients with MS had a lower increase in 25(OH)D levels with supplementation, even after accounting for putative confounders.
[Pt] Publication type:JOURNAL ARTICLE
[Em] Entry month:1605
[Cu] Class update date: 160521
[Lr] Last revision date:160521
[Js] Journal subset:IM
[St] Status:In-Data-Review
[do] DOI:10.1177/1352458515600248

  5 / 198803 MEDLINE  
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[PMID]: 26238464
[Au] Autor:Miller DM; Bethoux F; Victorson D; Nowinski CJ; Buono S; Lai JS; Wortman K; Burns JL; Moy C; Cella D
[Ad] Address:Mellen Center, Cleveland Clinic, USA millerd@ccf.org....
[Ti] Title:Validating Neuro-QoL short forms and targeted scales with people who have multiple sclerosis.
[So] Source:Mult Scler;22(6):830-41, 2016 May.
[Is] ISSN:1477-0970
[Cp] Country of publication:England
[La] Language:eng
[Ab] Abstract:BACKGROUND: Multiple sclerosis (MS) is a chronic, progressive, and disabling disease of the central nervous system with dramatic variations in the combination and severity of symptoms it can produce. The lack of reliable disease-specific health-related quality of life (HRQL) measures for use in clinical trials prompted the development of the Neurology Quality of Life (Neuro-QOL) instrument, which includes 13 scales that assess physical, emotional, cognitive, and social domains, for use in a variety of neurological illnesses. OBJECTIVE: The objective of this research paper is to conduct an initial assessment of the reliability and validation of the Neuro-QOL short forms (SFs) in MS. METHODS: We assessed reliability, concurrent validity, known groups validity, and responsiveness between cross-sectional and longitudinal data in 161 recruited MS patients. RESULTS: Internal consistency was high for all measures (α = 0.81-0.95) and ICCs were within the acceptable range (0.76-0.91); concurrent and known groups validity were highest with the Global HRQL question. Longitudinal assessment was limited by the lack of disease progression in the group. CONCLUSIONS: The Neuro-QOL SFs demonstrate good internal consistency, test-re-test reliability, and concurrent and known groups validity in this MS population, supporting the validity of Neuro-QOL in adults with MS.
[Pt] Publication type:JOURNAL ARTICLE
[Em] Entry month:1605
[Cu] Class update date: 160521
[Lr] Last revision date:160521
[Js] Journal subset:IM
[St] Status:In-Data-Review
[do] DOI:10.1177/1352458515599450

  6 / 198803 MEDLINE  
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[PMID]: 27195135
[Au] Autor:Le Pimpec-Barthes F; Legras A; Arame A; Pricopi C; Boucherie JC; Badia A; Panzini CM
[Ad] Address:1 Department of Thoracic Surgery and Lung Transplantation, Assistance Publique-Hôpitaux de Paris, Hopital Europeen Georges Pompidou, Paris, France ; 2 Université Paris Descartes, Faculté de Médecine, Paris, France ; 3 Department of Anesthesiology and Intensive Care, Assistance Publique-Hôpitaux de P...
[Ti] Title:Diaphragm pacing: the state of the art.
[So] Source:J Thorac Dis;8(Suppl 4):S376-86, 2016 Apr.
[Is] ISSN:2072-1439
[Cp] Country of publication:China
[La] Language:eng
[Ab] Abstract:Diaphragm pacing (DP) is an orphan surgical procedure that may be proposed in strictly selected ventilator-dependent patients to get an active diaphragm contraction. The goal is to wean from mechanical ventilation (MV) and restore permanent efficient breathing. The two validated indications, despite the lack of randomised control trials, concern patients with high-level spinal cord injuries (SCI) and central hypoventilation syndromes (CHS). To date, two different techniques exist. The first, intrathoracic diaphragm pacing (IT-DP), based on a radiofrequency method, in which the electrodes are directly placed around the phrenic nerve. The second, intraperitoneal diaphragm pacing (IP-DP) uses intradiaphragmatic electrodes implanted through laparoscopy. In both techniques, the phrenic nerves must be intact and diaphragm reconditioning is always required after implantation. No perioperative mortality has been reported and ventilator-weaning rate is about 72% to 96% in both techniques. Improvement of quality of life, by restoring a more physiological breathing, has been almost constant in patients that could be weaned. Failure or delay in recovery of effective diaphragm contractions could be due to irreversible amyotrophy or chest wall damage. Recent works have evaluated the interest of IP-DP in amyotrophic lateral sclerosis (ALS). After some short series were reported in the literature, the only multicentric randomized study including 74 ALS patients was prematurely stopped because of excessive mortality in paced patients. Then, another trial analysed the place of IP-DP in peripheral diaphragm dysfunction but, given the multiple biases, the published results cannot validate that indication. Reviewing all available literature as in our experience, shows that DP is an effective method to wean selected patients dependent on ventilator and improve their daily life. Other potential indications will have to be evaluated by randomised control trials.
[Pt] Publication type:JOURNAL ARTICLE; REVIEW
[Em] Entry month:1605
[Cu] Class update date: 160521
[Lr] Last revision date:160521
[Da] Date of entry for processing:160519
[St] Status:PubMed-not-MEDLINE
[do] DOI:10.21037/jtd.2016.03.97

  7 / 198803 MEDLINE  
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[PMID]: 27195035
[Au] Autor:Kumar S; Aga P; Gupta A; Kohli N
[Ad] Address:Department of Radiodiagnosis, King George's Medical University, Lucknow, Uttar Pradesh, India....
[Ti] Title:Juvenile amyotrophic lateral sclerosis: Classical wine glass sign on magnetic resonance imaging.
[So] Source:J Pediatr Neurosci;11(1):56-7, 2016 Jan-Mar.
[Is] ISSN:1817-1745
[Cp] Country of publication:India
[La] Language:eng
[Ab] Abstract:Amyotrophic lateral sclerosis (ALS), also known as Lou Gehrig disease, is a chronic degenerative neurologic disease and is characterized by the selective involvement of the motor system. Usually, patients present with upper motor neuron (UMN) and lower motor neuron compromise. Degeneration of the UMN in the cerebral cortex is one of the main pathologic changes in ALS. These changes usually affect corticospinal tracts leading to degeneration of the fibers which show characteristic hyperintensities along the tracts leading to the "wine glass sign." Patients with ALS usually present in the sixth decade of life; presentation in pediatric age in the form of juvenile ALS being rare.
[Pt] Publication type:JOURNAL ARTICLE
[Em] Entry month:1605
[Cu] Class update date: 160521
[Lr] Last revision date:160521
[Da] Date of entry for processing:160519
[St] Status:PubMed-not-MEDLINE
[do] DOI:10.4103/1817-1745.181251

  8 / 198803 MEDLINE  
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[PMID]: 27195190
[Au] Autor:Rousseau MC; Baumstarck K; Billette de Villemeur T; Auquier P
[Ad] Address:Hôpital San Salvadour (Assistance Publique Hôpitaux de Paris), Paris, France....
[Ti] Title:Evaluation of quality of life in individuals with severe chronic motor disability: A major challenge.
[So] Source:Intractable Rare Dis Res;5(2):83-9, 2016 May.
[Is] ISSN:2186-3644
[Cp] Country of publication:Japan
[La] Language:eng
[Ab] Abstract:Diverse conditions causing a very heavy and chronic motor disability, such as an advanced amyotrophic lateral, advanced form of multiple sclerosis, high spinal cord injury or a locked-in syndrom, are now getting better medical care and benefit of life support technology with consequent prolonged survival. Quality of life (QoL) assessment is being considered increasingly important to globally apprehend their general well-being. However, the motor disability that affects them appears as a substantial limitation for the assessment of their QoL and consequently a major challenge for all the community that carries an interest for them. This review discussed several avenues to provide to patients and caregivers, clinicians and researchers, and health decision making authority: i) elements to determine the most appropriate QoL measure with regard to the interest of patient's point of view, the QoL instruments suitable for this category of patients and their acceptability, ii) some arguments of the clinical relevance and accuracy of QoL assessment: interpretations of the questionnaires, QoL determinants, particularity of QoL evaluation for individuals with cognitive impairment and the caregivers perceptions of patients QoL. In conclusion, evaluation of QoL in patients with severe chronic motor handicap is a challenge of major interest, with major ethical issues. It needs to use adapted QoL scales and longitudinal following because of adaptive phenomena to the degree of handicap.
[Pt] Publication type:JOURNAL ARTICLE; REVIEW
[Em] Entry month:1605
[Cu] Class update date: 160521
[Lr] Last revision date:160521
[Da] Date of entry for processing:160519
[St] Status:PubMed-not-MEDLINE
[do] DOI:10.5582/irdr.2016.01017

  9 / 198803 MEDLINE  
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[PMID]: 27192568
[Au] Autor:Zhang Q; Vignali DA
[Ad] Address:Department of Immunology, University of Pittsburgh School of Medicine, Pittsburgh, PA 15261, USA.
[Ti] Title:Co-stimulatory and Co-inhibitory Pathways in Autoimmunity.
[So] Source:Immunity;44(5):1034-51, 2016 May 17.
[Is] ISSN:1097-4180
[Cp] Country of publication:United States
[La] Language:eng
[Ab] Abstract:The immune system is guided by a series of checks and balances, a major component of which is a large array of co-stimulatory and co-inhibitory pathways that modulate the host response. Although co-stimulation is essential for boosting and shaping the initial response following signaling through the antigen receptor, inhibitory pathways are also critical for modulating the immune response. Excessive co-stimulation and/or insufficient co-inhibition can lead to a breakdown of self-tolerance and thus to autoimmunity. In this review, we will focus on the role of co-stimulatory and co-inhibitory pathways in two systemic (systemic lupus erythematosus and rheumatoid arthritis) and two organ-specific (multiple sclerosis and type 1 diabetes) emblematic autoimmune diseases. We will also discuss how mechanistic analysis of these pathways has led to the identification of potential therapeutic targets and initiation of clinical trials for autoimmune diseases, as well as outline some of the challenges that lie ahead.
[Pt] Publication type:JOURNAL ARTICLE; REVIEW
[Em] Entry month:1605
[Cu] Class update date: 160521
[Lr] Last revision date:160521
[Js] Journal subset:IM
[St] Status:In-Data-Review

  10 / 198803 MEDLINE  
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[PMID]: 27195055
[Au] Autor:Grant WB
[Ad] Address:Sunlight, Nutrition, and Health Research Center , San Francisco, CA, USA.
[Ti] Title:The role of geographical ecological studies in identifying diseases linked to UVB exposure and/or vitamin D.
[So] Source:Dermatoendocrinol;8(1):e1137400, 2016 Jan-Dec.
[Is] ISSN:1938-1972
[Cp] Country of publication:United States
[La] Language:eng
[Ab] Abstract:Using a variety of approaches, researchers have studied the health effects of solar ultraviolet (UV) radiation exposure and vitamin D. This review compares the contributions from geographical ecological studies with those of observational studies and clinical trials. Health outcomes discussed were based on the author's knowledge and include anaphylaxis/food allergy, atopic dermatitis and eczema, attention deficit hyperactivity disorder, autism, back pain, cancer, dental caries, diabetes mellitus type 1, hypertension, inflammatory bowel disease, lupus, mononucleosis, multiple sclerosis, Parkinson disease, pneumonia, rheumatoid arthritis, and sepsis. Important interactions have taken place between study types; sometimes ecological studies were the first to report an inverse correlation between solar UVB doses and health outcomes such as for cancer, leading to both observational studies and clinical trials. In other cases, ecological studies added to the knowledge base. Many ecological studies include other important risk-modifying factors, thereby minimizing the chance of reporting the wrong link. Laboratory studies of mechanisms generally support the role of vitamin D in the outcomes discussed. Indications exist that for some outcomes, UVB effects may be independent of vitamin D. This paper discusses the concept of the ecological fallacy, noting that it applies to all epidemiological studies.
[Pt] Publication type:JOURNAL ARTICLE; REVIEW
[Em] Entry month:1605
[Cu] Class update date: 160521
[Lr] Last revision date:160521
[Da] Date of entry for processing:160519
[St] Status:PubMed-not-MEDLINE
[do] DOI:10.1080/19381980.2015.1137400


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