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1.
Eur Phys J C Part Fields ; 78(12): 1006, 2018.
Artigo em Inglês | MEDLINE | ID: mdl-30872956

RESUMO

One of the main objectives of the ANTARES telescope is the search for point-like neutrino sources. Both the pointing accuracy and the angular resolution of the detector are important in this context and a reliable way to evaluate this performance is needed. In order to measure the pointing accuracy of the detector, one possibility is to study the shadow of the Moon, i.e. the deficit of the atmospheric muon flux from the direction of the Moon induced by the absorption of cosmic rays. Analysing the data taken between 2007 and 2016, the Moon shadow is observed with 3.5 σ statistical significance. The detector angular resolution for downward-going muons is 0 . 73 ∘ ± 0 . 14 ∘ . The resulting pointing performance is consistent with the expectations. An independent check of the telescope pointing accuracy is realised with the data collected by a shower array detector onboard of a ship temporarily moving around the ANTARES location.

2.
Eur Phys J C Part Fields ; 77(6): 419, 2017.
Artigo em Inglês | MEDLINE | ID: mdl-28775667

RESUMO

A novel algorithm to reconstruct neutrino-induced particle showers within the ANTARES neutrino telescope is presented. The method achieves a median angular resolution of [Formula: see text] for shower energies below 100 TeV. Applying this algorithm to 6 years of data taken with the ANTARES detector, 8 events with reconstructed shower energies above 10 TeV are observed. This is consistent with the expectation of about 5 events from atmospheric backgrounds, but also compatible with diffuse astrophysical flux measurements by the IceCube collaboration, from which 2-4 additional events are expected. A [Formula: see text] C.L. upper limit on the diffuse astrophysical neutrino flux with a value per neutrino flavour of [Formula: see text] is set, applicable to the energy range from 23 TeV to 7.8 PeV, assuming an unbroken [Formula: see text] spectrum and neutrino flavour equipartition at Earth.

3.
Sci Rep ; 7: 45517, 2017 04 12.
Artigo em Inglês | MEDLINE | ID: mdl-28401960

RESUMO

Despite dedicated research has been carried out to adequately map the distribution of the sperm whale in the Mediterranean Sea, unlike other regions of the world, the species population status is still presently uncertain. The analysis of two years of continuous acoustic data provided by the ANTARES neutrino telescope revealed the year-round presence of sperm whales in the Ligurian Sea, probably associated with the availability of cephalopods in the region. The presence of the Ligurian Sea sperm whales was demonstrated through the real-time analysis of audio data streamed from a cabled-to-shore deep-sea observatory that allowed the hourly tracking of their long-range echolocation behaviour on the Internet. Interestingly, the same acoustic analysis indicated that the occurrence of surface shipping noise would apparently not condition the foraging behaviour of the sperm whale in the area, since shipping noise was almost always present when sperm whales were acoustically detected. The continuous presence of the sperm whale in the region confirms the ecological value of the Ligurian sea and the importance of ANTARES to help monitoring its ecosystems.

4.
Appl Radiat Isot ; 123: 54-59, 2017 May.
Artigo em Inglês | MEDLINE | ID: mdl-28242294

RESUMO

The BiPo-3 detector is a low radioactive detector dedicated to measuring ultra-low natural contaminations of 208Tl and 214Bi in thin materials, initially developed to measure the radiopurity of the double ß decay source foils of the SuperNEMO experiment at the µBq/kg level. The BiPo-3 technique consists in installing the foil of interest between two thin ultra-radiopure scintillators coupled to low radioactive photomultipliers. The design and performances of the detector are presented. In this paper, the final results of the 208Tl and 214Bi activity measurements of the first enriched 82Se foils are reported for the first time, showing the capability of the detector to reach sensitivities in the range of some µBq/kg.

5.
Phys Rev Lett ; 119(4): 041801, 2017 Jul 28.
Artigo em Inglês | MEDLINE | ID: mdl-29341770

RESUMO

We report the results of a first experimental search for lepton number violation by four units in the neutrinoless quadruple-ß decay of ^{150}Nd using a total exposure of 0.19 kg yr recorded with the NEMO-3 detector at the Modane Underground Laboratory. We find no evidence of this decay and set lower limits on the half-life in the range T_{1/2}>(1.1-3.2)×10^{21} yr at the 90% C.L., depending on the model used for the kinematic distributions of the emitted electrons.

6.
Org Biomol Chem ; 14(4): 1473-84, 2016 Jan 28.
Artigo em Inglês | MEDLINE | ID: mdl-26690983

RESUMO

A series of conformationally locked C-glycosides based on the 3-aminopyrano[3,2-b]pyrrol-2(1H)-one (APP) scaffold has been synthesized. The key step involved a totally stereocontrolled C-Michael addition of a serine-equivalent C-nucleophile to tri-O-benzyl-2-nitro-D-galactal, previously published by the authors. Stereoselective transformations of the Michael adduct allowed us the synthesis of compounds with mono- or diantennated aglycone moieties and different topologies. In vitro screening showed highly selective inhibition of bovine liver ß-glucosidase/ß-galactosidase and specific inhibition of human ß-glucocerebrosidase among lysosomal glycosidases for compounds bearing palmitoyl chains in the aglycone, with a marked dependence of the inhibition potency upon their number and location. Molecular dynamics simulations highlighted the paramount importance of an optimal orientation of the hydrophobic substituent to warrant efficient non-glycone interactions, which are critical for the binding affinity. The results provide a rationale for the strong decrease of the inhibition potency of APP compounds on going from neutral to acidic pH. The best candidate was found to behave as pharmacological chaperone in Gaucher fibroblasts with homozygous N370S and F213I mutations, with enzyme activity enhancements similar to those encountered for the reference compound Ambroxol.


Assuntos
Inibidores Enzimáticos/farmacologia , Fibroblastos/efeitos dos fármacos , Doença de Gaucher/patologia , Chaperonas Moleculares/farmacologia , Monossacarídeos/farmacologia , Animais , Bovinos , Relação Dose-Resposta a Droga , Inibidores Enzimáticos/síntese química , Inibidores Enzimáticos/química , Glucosilceramidase/antagonistas & inibidores , Glicosídeos , Humanos , Fígado/enzimologia , Modelos Moleculares , Chaperonas Moleculares/síntese química , Chaperonas Moleculares/química , Conformação Molecular , Simulação de Dinâmica Molecular , Monossacarídeos/síntese química , Monossacarídeos/química , Relação Estrutura-Atividade , beta-Galactosidase/antagonistas & inibidores , beta-Glucosidase/antagonistas & inibidores
7.
Homo ; 66(2): 158-64, 2015 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-25659892

RESUMO

The study of surnames in a territory over time is an opportunity to obtain knowledge of the evolution of allelic frequencies. Geographic and cultural factors influence the renovation of surnames and reflect accelerations or delays in the gene flow. Political borders may also condition the genetic structure of a population. Using isonymy, this paper studies the evolution (from 1750 to 2006) of the frequencies of surnames and the components of inbreeding in Olivenza, a border town whose sovereignty was transferred from Portugal to Spain in 1801. After the change in dominion the number of Portuguese surnames fell sharply and the expected values for a population so close to Portugal recovered only after a long period of time. The results indicate that although the border has made population movement more difficult, and has therefore had an impact on the rate of gene exchange, a certain gene flow with Portugal persisted.


Assuntos
Nomes , Consanguinidade , Emigração e Imigração/história , Emigração e Imigração/tendências , Fluxo Gênico , Frequência do Gene , Genética Populacional , História do Século XVIII , História do Século XIX , História do Século XX , História do Século XXI , Humanos , Dinâmica Populacional/história , Dinâmica Populacional/tendências , Portugal , Espanha
8.
Neurologia ; 30(2): 90-6, 2015 Mar.
Artigo em Inglês, Espanhol | MEDLINE | ID: mdl-25440067

RESUMO

INTRODUCTION: Corpora amylacea (CoA) are present in about 60% of atrophic hippocampi resected from patients with drug resistant temporal lobe epilepsy (DRTLE). They have also been described in the lateral temporal neocortex, although less frequently. OBJECTIVE: The objective is to measure the presence, distribution and density of CoA in the lateral temporal lobes of patients with DRTLE and focal cortical dysplasia (FCD), also examining how CoA density may be linked to demographic and clinical traits. METHODS: Resected tissue from 35 patients was analysed. CoA density was assessed with a semi-quantitative scale according to the criteria established by Cherian et al. RESULTS: Presence of CoA in the neocortex of 9 patients was associated with hippocampal sclerosis (FCD type iiia, 7 cases), disembryoplastic neuroepithelial tumour (FCD type iiib, 1 case), and cavernous angioma (FCD type iiic, 1 case). The meningeal surface (MS) was involved in all cases, and 8 cases displayed CoA in the cerebral parenchyma (white matter) and around blood vessels. CoA density on the MS showed a negative correlation with age at seizure onset (r = -0.828, P<.05) and a positive correlation with disease duration (r = 0.678, P<.05) but not with postoperative clinical outcome. CONCLUSIONS: Patients with DRTLE and a primary lesion (hippocampal sclerosis, tumour, vascular malformation) associated with mild FCD were shown to have CoA deposits in the neocortex. No association was found between presence of CoA and clinical outcome one year after surgery.


Assuntos
Epilepsia do Lobo Temporal/patologia , Malformações do Desenvolvimento Cortical/patologia , Neocórtex/patologia , Adulto , Epilepsia Resistente a Medicamentos , Feminino , Hipocampo/patologia , Humanos , Masculino , Pessoa de Meia-Idade , Estudos Retrospectivos , Lobo Temporal/patologia , Adulto Jovem
9.
Hum Biol ; 87(2): 122-31, 2015 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-26829295

RESUMO

The existing relationship between human populations is a function of their migratory and genetic exchange, which will be inversely proportional to the distance separating them. The effect of geographic distance on population structure may be estimated by means of isonymic methods that use information on the surnames present in a territory as an approximation to the distribution of allele frequencies. The objective of this study was to analyze whether the 1801 modification of the political border in an area surrounding the town of Olivenza, which experienced a change of sovereignty from Portugal to Spain, has had a noticeable influence on the migration pattern and isolation by distance in that region. For this purpose, data from marriage records of Olivenza and the neighboring Portuguese municipalities of Alandroal, Juromenha, Elvas, Vila Boim (and Terrugem), Terena, Monsaraz, and Vila Viçosa were analyzed. Rates of diversity and inbreeding coefficients were determined to analyze the population structure before (1775-1801) and after (1802-1825) the change of domain. The results show that after the border modification the migration matrices changed differently according to sex, therefore altering the relationship between the various localities of the territory. In Olivenza inbreeding declined slightly and surnames became more heterogeneous. Moreover, after the change of domain the isolation-by-distance models illustrate a temporal reduction in the relative weight of geographical distance on interpopulation kinship. The political border acted as a factor in population differentiation in the Iberian Peninsula (Spain and Portugal).


Assuntos
Frequência do Gene , Migração Humana/história , Política , Dinâmica Populacional/história , Feminino , Genética Populacional , Geografia , História do Século XVIII , História do Século XIX , Humanos , Masculino , Casamento , Nomes , Portugal , Registros , Espanha
10.
J Biosoc Sci ; 47(1): 90-104, 2015 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-24524355

RESUMO

The geographic and demographic dimensions of Spain, in terms of surface and number of inhabitants, and its heterogeneous socioeconomic development offer an adequate opportunity to study the provincial differences in birth weight from 1996 to 2010, focusing on possible factors determining the relative frequency of low birth weight. The study analysed geographic differences with regard to biological, demographic and socioeconomic factors that interfere with the female reproductive pattern. The variables considered here were: birth order, proportion of premature deliveries, mother's age, multiparity, mother's country of origin and professional qualifications. Two periods (1996-2000 and 2006-2010) were compared by means of principal components analysis. An increase in the relative frequency of deliveries weighing less than 2500 g occurred in most of the 52 geographic units studied, differences being significant in 42. Only in five cases was there a non-significant reduction in the proportion of low weight births. The first component after principal component analysis indicated that low birth weight was positively related to maternal age and to multiple deliveries, and negatively to the mother's low professional qualification. The second component related positively to the incidence of premature deliveries and to non-Spanish status and negatively in the case of primiparous mothers. The progressive increase in low birth weight incidence observed in Spain from 1996 onwards has occurred with considerable variation in each province. In part, this diversity can be attributed to the unequal reproductive patterns of immigrant mothers.


Assuntos
Peso ao Nascer , Recém-Nascido de Baixo Peso , Fatores Socioeconômicos , Adulto , Ordem de Nascimento , Parto Obstétrico , Demografia , Emigrantes e Imigrantes/estatística & dados numéricos , Feminino , Idade Gestacional , Humanos , Recém-Nascido , Idade Materna , Paridade , Gravidez , Nascimento Prematuro , Análise de Componente Principal , Espanha
11.
Ann Hum Biol ; 40(3): 266-75, 2013 May.
Artigo em Inglês | MEDLINE | ID: mdl-23388000

RESUMO

BACKGROUND: Low weight and premature deliveries arouse clinical interest concerning the survival of newborns. The determinants of birth weight among Spanish natives and immigrants may differ. Research which considers maternal origin and associated factors such as age and parity is important. AIM: This study analyses and models the influence of the rapid and intense arrival of immigrants in Spain on birth weight variation. METHOD: Data on deliveries from the Spanish National Institute for Statistics (n = 9 443 882) are analysed regarding low birth weight, premature births and other variables. The inter-relation among these variables was interpreted by means of logistic regression models. RESULTS AND CONCLUSIONS: The birth weight has decreased since 1980 in Spain, but has slightly recovered in recent years. Meanwhile the percentage of foreign maternities increased to 17.3% in 2010. Logistic regression models assess the different influence of variables known to determine low birth weight (weeks of gestation, sex, etc.) and other maternal characteristics (age at delivery, professional activity, etc.). The progressively greater contribution of foreign women to total births in Spain and their differential numerical input to the various risk groups have slowed the pattern of reduction in the mean weight of newborns in this country.


Assuntos
Peso ao Nascer , Emigrantes e Imigrantes , Recém-Nascido de Baixo Peso , Nascimento Prematuro/epidemiologia , Adulto , Feminino , Idade Gestacional , Inquéritos Epidemiológicos , Humanos , Recém-Nascido , Modelos Logísticos , Masculino , Modelos Biológicos , Fatores de Risco , Fatores Socioeconômicos , Espanha/epidemiologia , Fatores de Tempo , Adulto Jovem
12.
J Biosoc Sci ; 45(1): 79-93, 2013 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-22874007

RESUMO

The present analysis compares the distribution of surnames by means of spatial autocorrelation analysis in the Spain-Portugal border region. The Spanish National Institute of Statistics provides a database of surnames of residents in the western Spanish provinces of Zamora, Salamanca, Cáceres, Badajoz and Huelva. The Spanish and Portuguese patterns of surname distribution were established according to various geographic axes. The results obtained show a low diversity of surnames in this region - especially in the centre - which can be explained by the absence of any major geographic barriers, with the exception of the mountain ranges between hydrographic basins, and by the presence of traditional roads that have existed since Roman times. The latter have resulted in a constant migratory flow over short-median distances, which, as can be deduced from the surnames, fits two north/south territorial axes running parallel to the border between Spain and Portugal. The distribution patterns of Portuguese and Spanish surnames differ with regard to their frequencies in the five provinces studied, which can be attributed to their respective historical, economic and social conditions. It is concluded that the border delimiting these two countries has affected the migratory flow, thereby conditioning the demographic and genetic structure of the western Spanish regions.


Assuntos
Genética Populacional , Nomes , Dinâmica Populacional , Feminino , Humanos , Masculino , Portugal , Espanha
13.
Anthropol Anz ; 69(2): 243-53, 2012.
Artigo em Inglês | MEDLINE | ID: mdl-22606917

RESUMO

As a part of a wider analysis of population and genetic exchange between Spain and Portugal, the long-term pattern of Portuguese immigration to the Canary Islands was studied by means of the frequency of Portuguese surnames. A database of 1,995,833 individuals was obtained from the Spanish National Statistics Institute (2006). Among the 826 most frequent surnames to appear, 79 surnames of Portuguese origin were selected. The distribution of these surnames by municipalities and islands, the Fisher index of diversity, and the Lasker inter-population relationship coefficients R(ij) were considered. These coefficients were inter-correlated and correlated with other variables that could have influenced the distribution of surnames. From the observed distribution of the frequency of surnames, a non-random migration pattern conditioned by economic factors was found. The greatest diversity of surnames existed in cane cultivating areas after the first arrival of Portuguese immigrants. A later dispersion of surnames among islands was correlated with the inter-island geographic distances. In some islands the arrival of new immigrants continued due to their strategic location within the Canary archipelago. The Canary Islands reveal a high frequency and diversity of Portuguese surnames. The results also prove a heterogeneous distribution of these surnames throughout the archipelago. In contrast to the Portuguese archipelagos, some Canary localities have received immigrants continuously because of their economic importance in sugar cane cultivation and strategic geographic location on the maritime routes to Africa and America.


Assuntos
Emigração e Imigração/estatística & dados numéricos , Modelos Estatísticos , Nomes , Agricultura , Antropologia Cultural , Humanos , Portugal , Saccharum , Espanha
14.
Seizure ; 18(8): 593-600, 2009 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-19679496

RESUMO

BACKGROUND: Associations between electrophysiological and histological findings might provide an insight into the epileptogenicity of mild focal cortical dysplasia (FCD) in patients with temporal lobe epilepsy (TLE) and a dual pathology. SUBJECTS AND METHODS: A total of 22 patients with pharmacoresistant TLE were included in the study, 16 of them with histologically confirmed hippocampal sclerosis (HS) associated with neocortical temporal mild Palmini Type-I FCD subtypes and 6 with HS. Intraoperative electrocorticography (ECoG) recordings were analysed for epileptiform discharge frequency and morphology. Associations between histological, and electrocorticography pattern findings in these patients were analysed. Electroclinical outcomes in these patients were also evaluated. RESULTS: Neocortical areas with mild Palmini Type-I FCD showed a significantly higher spike frequency (SF) recorded in the inferior temporal gyrus than those neocortical areas in patients with HS. There was a tendency to higher spike frequency and lower amplitude in neocortical areas with histopathologic subtype IB FCD in relation with IA during intraoperative ECoG. Post-SF excision and amplitude were significantly lower during neocortical post-excision intraoperative ECoG than during neocortical pre-excision recording. There was no difference found in the clinical outcome between patients with and without FCD. CONCLUSIONS: Intraoperative electrocorticographic interictal spike frequency recorded in the neocortical inferior temporal gyrus may help to characterize the histopathologic subtypes of mild Palmini Type-I FCD in patients with temporal lobe epilepsy (TLE) and a dual pathology. Our data support the epileptogenicity of neocortical mild FCD in TLE and assessments of ECoG patterns are relevant to determine the extent of the resection in these patients which can influence the electroclinical outcome.


Assuntos
Eletroencefalografia/métodos , Epilepsia do Lobo Temporal/patologia , Epilepsia do Lobo Temporal/fisiopatologia , Malformações do Desenvolvimento Cortical/patologia , Neocórtex/patologia , Adolescente , Adulto , Lobectomia Temporal Anterior/métodos , Epilepsia do Lobo Temporal/cirurgia , Feminino , Seguimentos , Hipocampo/patologia , Humanos , Masculino , Malformações do Desenvolvimento Cortical/fisiopatologia , Malformações do Desenvolvimento Cortical/cirurgia , Pessoa de Meia-Idade , Neocórtex/fisiopatologia , Estudos Retrospectivos , Resultado do Tratamento , Adulto Jovem
15.
Neurología (Barc., Ed. impr.) ; 23(9): 555-565, nov. 2008. tab, graf, ilus
Artigo em Espanhol | IBECS | ID: ibc-76051

RESUMO

Introducción. La participación de mecanismos de muerteapoptótica en la epilepsia del lóbulo temporal resistente afármacos (ELTRF) es un aspecto muy discutido en la actualidad.Investigamos si existe pérdida neuronal y la inmunodeteccióna diferentes marcadores de muerte en tejido neocorticalen ocho pacientes con ELTRF y como tejido controlse evaluaron cinco neocortezas de sujetos fallecidos porcausas no neurológicas, pareados en edad y sexo.Métodos. La evaluación de la pérdida neuronal se realizópor medio de un estudio estereológico y por técnica inmunohistoquímicacon el marcador sinaptofisina. Se evaluóla inmunopositividad a diferentes marcadores apoptóticos(anexina V, caspasa 3 y 8, bcl-2 y p53), así como la detecciónde fragmentación del ácido desoxirribonucleico (ADN) (TUNEL),y se realizó en todos los casos un doble marcaje con sinaptofisina.Los resultados fueron evaluados por microscopiaconfocal y analizados por el programa Zeiss LSM 5 ImageBrowser, 2.80.1113 (Alemania).Resultados. Se observó una disminución estadísticamentesignificativa del número total de células (p<0,05), asícomo de las células sinaptofisina+ (p<0,01) en la neocorteza(capa IV) de los pacientes con ELTRF al ser comparadoscon el tejido control. No mostraron diferencias significativaslos marcadores apoptóticos bcl-2, p53, caspasa 3 y 8 paraninguna de las capas de neocorteza, mientras que sí resultóestadísticamente aumentado el número de células TUNEL+(p<0,05) y anexina V+ (p<0,05) en la capa IV neocortical delos pacientes.Conclusiones. Este grupo de evidencias hablan a favorde la existencia en la capa IV de neocorteza de una afectaciónen el número neuronal que se puede asociar a un procesode muerte apoptótica por una vía no dependiente de caspasas,sin que pueda ser descartada la muerte por necrosis (AU)


Introduction. Participation of apoptotic death mechanisms in drug resistant temporal lobe epilepsy (DRTLE) is currently under great debate. We have investigated if there is neuronal loss and the immunodetection to differentmarkers in neocortical tissue death in eigth patients with DRTLE. The neocortexes of five patients deceased due to non-neurological causes, paired in age and gender were evaluated as control tissue. Methods. The evaluation of neuronal loss was made by means of a stereological study and with immunohisto chemical techniques with the synaptophysin marker. Immunopositivity to different apoptotic markers (annexin V, caspase 3 and 8, bcl-2 and p53) and detection of deoxyribonucleic acid (DNA) fragmentation (TUNEL) wereanalyzed and double labeling with synaptophysin was performed in every case. The results were evaluated with confocal microscope and analyzed with the Zeiss LSM 5 Image Browser Program, 2.80.1113 (Germany). Results. A statistically significant decrease in the total number of cells (p < 0.05) and the synaptophysin cells+ (p<0.01) in the neocortex (layer IV) of the patients with DRTLE when compared with the control tissue was found. No significant differences were found in the apoptotic markers bcl-2, p53, caspase 3 and 8 for any of the neocortex layers while there was a statistically significantincrease in the number of TUNEL cells+ (p<0.05) and annexin V+ (p<0.05) in the neocortical layer IV of the patients. Conclusions. This group of evidence speaks in favor of the existence of an effect on the neuronal number in the neocortex layer IV that may be associated with non caspase dependent apoptotic death process, without beingable to rule out death by necrosis


Assuntos
Humanos , Masculino , Feminino , Adulto , Epilepsia do Lobo Temporal/etiologia , Apoptose , Morte Celular , Epilepsia do Lobo Temporal/tratamento farmacológico , Epilepsia do Lobo Temporal/diagnóstico , Neocórtex/citologia , Neocórtex , Anticonvulsivantes/farmacologia
16.
Rev Neurol ; 46(4): 203-9, 2008.
Artigo em Espanhol | MEDLINE | ID: mdl-18327741

RESUMO

INTRODUCTION: The dual pathology consisting of hippocampal sclerosis plus focal cortical dysplasia (FCD) is often reported in patients with medication-resistant medial temporal lobe epilepsy (MTLE). AIMS: To determine the histopathological changes that take place in the neocortex of patients with medication-resistant MTLE submitted to surgery and to evaluate the relation between the histopathological changes, pathological background and the clinical course of patients who had received surgical treatment. MATERIALS AND METHODS: Tissue obtained by en bloc resection from the neocortex of 18 patients with MTLE refractory to medical treatment was processed histologically and a tailored temporal lobectomy was performed with electrocorticography. RESULTS: Dual pathology was diagnosed in 13 patients (72.2%). Imaging studies confirmed the existence of mesial sclerosis of the temporal in 100% of cases and there was no evidence of neocortical lesions. Histologically, 46.15% and 38.46% of the patients were diagnosed as belonging to FCD type 1a and FCD type 1b, respectively. Only one patient presented FCD type 2a. A statistically significant relation was found between the presence of dual pathology and the existence of an early precipitating injury (p = 0.04). One year after surgery, 72.7% (8/11) patients with dual pathology were classified as belonging to Engel class I. CONCLUSIONS: In patients with MTLE there are microscopic FCD-type alterations in the neocortex. There is an association between these alterations and the existence of an initial precipitating injury. Complete resection of the epileptogenic area, which is guaranteed by the lobectomy tailored by electrocorticography, allows patients to enjoy a favourable post-surgical progression one year after surgery.


Assuntos
Epilepsia do Lobo Temporal/patologia , Epilepsia do Lobo Temporal/cirurgia , Neocórtex/patologia , Adulto , Resistência a Medicamentos , Epilepsia do Lobo Temporal/tratamento farmacológico , Feminino , Humanos , Masculino , Pessoa de Meia-Idade
17.
Neurologia ; 23(9): 555-65, 2008 Nov.
Artigo em Espanhol | MEDLINE | ID: mdl-18247188

RESUMO

Introduction. Participation of apoptotic death mechanisms in drug resistant temporal lobe epilepsy (DRTLE) is currently under great debate. We have investigated if there is neuronal loss and the immunodetection to different markers in neocortical tissue death in eigth patients with DRTLE. The neocortexes of five patients deceased due to non-neurological causes, paired in age and gender were evaluated as control tissue. Methods. The evaluation of neuronal loss was made by means of a stereological study and with immunohistochemical techniques with the synaptophysin marker. Immunopositivity to different apoptotic markers (annexin V, caspase 3 and 8, bcl-2 and p53) and detection of deoxyribonucleic acid (DNA) fragmentation (TUNEL) were analyzed and double labeling with synaptophysin was performed in every case. The results were evaluated with confocal microscope and analyzed with the Zeiss LSM 5 Image Browser Program, 2.80.1113 (Germany). Results. A statistically significant decrease in the total number of cells (p < 0.05) and the synaptophysin cells+ (p<0.01) in the neocortex (layer IV) of the patients with DRTLE when compared with the control tissue was found. No significant differences were found in the apoptotic markers bcl-2, p53, caspase 3 and 8 for any of the neocortex layers while there was a statistically significant increase in the number of TUNEL cells+ (p<0.05) and annexin V+ (p<0.05) in the neocortical layer IV of the patients. Conclusions. This group of evidence speaks in favor of the existence of an effect on the neuronal number in the neocortex layer IV that may be associated with noncaspase dependent apoptotic death process, without being able to rule out death by necrosis. Key words: Drug resistant temporal lobe epilepsy. Apoptosis. Necrosis. Neuronal loss. Neurología 2008;23(9):555-565.


Assuntos
Morte Celular , Epilepsia do Lobo Temporal/tratamento farmacológico , Epilepsia do Lobo Temporal/patologia , Neocórtex/patologia , Neurônios/patologia , Adulto , Anticonvulsivantes/uso terapêutico , Biomarcadores/metabolismo , Resistência a Medicamentos , Epilepsia do Lobo Temporal/fisiopatologia , Feminino , Humanos , Marcação In Situ das Extremidades Cortadas , Masculino , Pessoa de Meia-Idade , Neocórtex/fisiopatologia
18.
Rev. neurol. (Ed. impr.) ; 46(4): 203-209, 27 feb., 2008. ilus, tab
Artigo em Es | IBECS | ID: ibc-65418

RESUMO

La patología dual compuesta por esclerosis hipocampal y displasia cortical focal (DCF) se describecon frecuencia en pacientes con epilepsia del lóbulo temporal medial (ELTM) farmacorresistente. Objetivos. Determinar los cambios histopatológicos en la neocorteza de pacientes con ELTM farmacorresistente sometidos a cirugía y evaluar la relación entre los cambios histopatológicos, los antecedentes patológicos y la evolución clínica en los pacientes operados. Materialesy métodos. Se procesó histológicamente el tejido resecado en bloque (neocorteza) de 18 pacientes con ELTM refractaria a tratamiento médico, y se les realizó lobectomía temporal ajustada por electrocorticografía. Resultados. Se diagnóstico patología dual en 13 pacientes (72,2%). Los estudios imagenológicos confirmaron en el 100% de los casos la esclerosis mesialdel temporal y no existieron evidencias de lesión neocortical. Histológicamente, el 46,15% y el 38,46% de los pacientes fueron diagnosticados como DCF tipo 1a y DCF tipo 1b, respectivamente. Sólo un paciente presentó DCF tipo 2a. Se demostró una relación estadísticamente significativa entre la presencia de patología dual y la existencia de una daño precipitante inicial (p = 0,04). El 72,7% (8/11) de los pacientes con patología dual un año después de la cirugía se clasificó en la clase Ide Engel. Conclusiones. En los pacientes con ELTM existen alteraciones microscópicas en la neocorteza del tipo DCF. Estas alteraciones se asocian a la existencia de un daño precipitante inicial. La resección completa de la zona epileptogénica, garantizadapor la lobectomía ajustada por electrocorticografía, permite una buena evolución posquirúrgica un año después de la cirugía


The dual pathology consisting of hippocampal sclerosis plus focal cortical dysplasia (FCD) is oftenreported in patients with medication-resistant medial temporal lobe epilepsy (MTLE). Aims. To determine the histopathological changes that take place in the neocortex of patients with medication-resistant MTLE submitted to surgery and to evaluate the relation between the histopathological changes, pathological background and the clinical course of patients whohad received surgical treatment. Materials and methods. Tissue obtained by en bloc resection from the neocortex of 18 patients with MTLE refractory to medical treatment was processed histologically and a tailored temporal lobectomy was performed with electrocorticography. Results. Dual pathology was diagnosed in 13 patients (72.2%). Imaging studies confirmed the existenceof mesial sclerosis of the temporal in 100% of cases and there was no evidence of neocortical lesions. Histologically, 46.15% and 38.46% of the patients were diagnosed as belonging to FCD type 1a and FCD type 1b, respectively. Only one patient presented FCD type 2a. A statistically significant relation was found between the presence of dual pathology and the existenceof an early precipitating injury (p = 0.04). One year after surgery, 72.7% (8/11) patients with dual pathology were classified as belonging to Engel class I. Conclusions. In patients with MTLE there are microscopic FCD-type alterations in the neocortex.There is an association between these alterations and the existence of an initial precipitating injury. Complete resection of the epileptogenic area, which is guaranteed by the lobectomy tailored by electrocorticography, allows patients to enjoy a favourable post-surgical progression one year after surgery


Assuntos
Humanos , Epilepsia do Lobo Temporal/cirurgia , Neocórtex/patologia , Epilepsia do Lobo Temporal/complicações , Esclerose/patologia , Hipocampo/patologia , Lobectomia Temporal Anterior , Neoplasias Encefálicas/patologia
19.
Heredity (Edinb) ; 99(4): 406-13, 2007 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-17551521

RESUMO

Major histocompatibility complex class II locus DRB variation was investigated by single-strand conformation polymorphism analysis and sequence analysis in the two subspecies of Pyrenean chamois (Rupicapra pyrenaica) endemic to the Iberian Peninsula. Low levels of genetic variation were detected in both subspecies, with seven different alleles in R. p. pyrenaica and only three in the R. p. parva. After applying the rarefaction method to cope with the differences in sample size, the low allele number of parva was highlighted. The low allelic repertoire of the R. p. parva subspecies is most likely the result of bottlenecks caused by hunting pressure and recent parasitic infections by sarcoptic mange. A phylogenetic analysis of both Pyrenean chamois and DRB alleles from 10 different caprinid species revealed that the chamois alleles form two monophyletic groups. In comparison with other Caprinae DRB sequences, the Rupicapra alleles displayed a species-specific clustering that reflects a large temporal divergence of the chamois from other caprinids, as well as a possible difference in the selective environment for these species.


Assuntos
Antígenos HLA-DR/genética , Rupicapra/genética , Sequência de Aminoácidos , Animais , Evolução Molecular , Frequência do Gene , Variação Genética , Genética Populacional , Geografia , Cadeias HLA-DRB1 , Dados de Sequência Molecular , Filogenia , Polimorfismo Genético , Polimorfismo Conformacional de Fita Simples , Análise de Sequência de DNA , Espanha
20.
Org Lett ; 9(7): 1235-8, 2007 Mar 29.
Artigo em Inglês | MEDLINE | ID: mdl-17346053

RESUMO

[structure: see text]. In this paper we describe a highly regioselective ring-opening metathesis-cross metathesis (ROM-CM) process between methyl N-Boc-7-azabicyclo[2.2.1]hept-2-en-1-carboxylate, a bridgehead-substituted 7-azanorbornene system, and electron-poor olefins. The reaction opens the way to the synthesis of interesting alpha-amino diacids and pyrrolizinone derivatives that incorporate quaternary stereocenters.

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