988 resultados para La Revellière-Lépeaux, Louis-Marie de, 1753-1824.
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Law.--La Chine.--L'Empire russe depuis le Congrès de Vienne.--Tallemant des Réaux.--La société française pendant la révolution.--L'Empire chinois.--Le Sahara algérien et le Grand désert.--Les Kœnigsmark.--L'ancien régime et la révolution.--Le christianisme en Chine, en Tartarie et au Thibet.--L'insurrection normande en 1793.--Les Philippiques de La Grange-Chancel.--Louis XVI et sa cour.--Gabrielle d'Estrées et Henry IV.--Mathilde de Toscane.--Joseph de Maistre.--Royalistes et républicains.--Les civilisations.--La diplomatie au XVIIe siècle.--Deux diplomates [Le comte Raczynski et Donoso Cortès]--Saint-Simon.--Le marquis de Grignan.--Philippe II.
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Contains "Résumé de l'entretien familier du président en chef de l'Institut polytechnique du Canada à la séance hebdomadaire du 30 avril 1857, l'an II de sa fondation": p. 43-55. "Introduction à l'étude de la géologie": p. 57-68. "Le code Napoléon": p. 69-70.
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CONTENTS CONTINUED: --3. Lhoillier, T. Notes sur quelques artists musiciens dans la Brie.--Carlez, J. La musique à Caen de 1066 à 1848.--Carlez, J. La puy de musique de Caen (1671-1685)--Coutagne, H. G. Duiffoproucart et les luthiers lyonnais du XVIe siècle.--Collette et Bourbon. Notice historique sur les orgues et les organistes de la Cathédrale de Rouen.--Tricou, G. Les deux Layolle et les organistes lyonnais du XVIe siècle. Tricou, G. Documents sur l'art musical en Touraine, 1508.
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Background: Mutations of GDAP1 gene cause autosomal dominant and autosomal recessive Charcot-Marie-Tooth disease and more than 40 different mutations have been reported. The recessive Q163X mutation has been described in patients of Spanish ancestry, and a founder mutation in South American patients, originating in Spain has been demonstrated. Objective: We describe physical and histological features, and the molecular impact of mutation Q163X in a Colombian family. Methods: We report two female patients, daughters of consanguineous parents, with onset of symptoms within the first two years of life, developing severe functional impairment, without evidence of dysmorphic features, hoarseness or diaphragmatic paralysis. Electrophysiology tests showed a sensory and motor neuropathy with axonal pattern. Sequencing of GDAP1 gene was requested and the study identified a homozygous point mutation (c.487 C>T) in exon 4, resulting in a premature stop codon (p.Q163X). This result confirms the diagnosis of Charcot-Marie-Tooth disease, type 4A. Results: The patients were referred to Physical Medicine and Rehabilitation service, in order to be evaluated for ambulation assistance. They have been followed by Pulmonology service, for pulmonary function assessment and diaphragmatic paralysis evaluation. Genetic counseling was offered. The study of the genealogy of the patient, phenotypic features, and electrophysiological findings must be included as valuable tools in the clinical approach of the patient with Charcot-Marie-Tooth disease, in order to define a causative mutation. In patients of South American origin, the presence of GDAP1 gene mutations should be considered, especially the Q163X mutation, as the cause of CMT4A disease.
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Background: Mutations of GDAP1 gene cause autosomal dominant and autosomal recessive Charcot-Marie-Tooth disease and more than 40 different mutations have been reported. The recessive Q163X mutation has been described in patients of Spanish ancestry, and a founder mutation in South American patients, originating in Spain has been demonstrated. Objective: we describe physical and histological features, and the molecular impact of mutation Q163X in a Colombian family. Methods: We report two female patients, daughters of consanguineous parents, with onset of symptoms within the first two years of life, developing severe functional impairment, without evidence of dysmorphic features, hoarseness or diaphragmatic paralysis. Electrophysiology tests showed a sensory and motor neuropathy with axonal pattern. Sequencing of GDAP1 gene was requested and the study identified a homozygous point mutation (c.487 C>T) in exon 4, resulting in a premature stop codon (p.Q163X). This result confirms the diagnosis of Charcot-Marie-Tooth disease, type 4A. Results: The patients were referred to Physical Medicine and Rehabilitation service, in order to be evaluated for ambulation assistance. They have been followed by Pulmonology service, for pulmonary function assessment and diaphragmatic paralysis evaluation. Genetic counseling was offered. The study of the genealogy of the patient, phenotypic features, and electrophysiological findings must be included as valuable tools in the clinical approach of the patient with Charcot-Marie-Tooth disease, in order to define a causative mutation. In patients of South American origin, the presence of GDAP1 gene mutations should be considered, especially the Q163X mutation, as the cause of CMT4A disease.
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Nota: Homenaje a Octavio Nicolás Derisi en sus veinticinco años de docencia filosófica.
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A scalable large vocabulary, speaker independent speech recognition system is being developed using Hidden Markov Models (HMMs) for acoustic modeling and a Weighted Finite State Transducer (WFST) to compile sentence, word, and phoneme models. The system comprises a software backend search and an FPGA-based Gaussian calculation which are covered here. In this paper, we present an efficient pipelined design implemented both as an embedded peripheral and as a scalable, parallel hardware accelerator. Both architectures have been implemented on an Alpha Data XRC-5T1, reconfigurable computer housing a Virtex 5 SX95T FPGA. The core has been tested and is capable of calculating a full set of Gaussian results from 3825 acoustic models in 9.03 ms which coupled with a backend search of 5000 words has provided an accuracy of over 80%. Parallel implementations have been designed with up to 32 cores and have been successfully implemented with a clock frequency of 133?MHz.
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There is considerable interest in creating embedded, speech recognition hardware using the weighted finite state transducer (WFST) technique but there are performance and memory usage challenges. Two system optimization techniques are presented to address this; one approach improves token propagation by removing the WFST epsilon input arcs; another one-pass, adaptive pruning algorithm gives a dramatic reduction in active nodes to be computed. Results for memory and bandwidth are given for a 5,000 word vocabulary giving a better practical performance than conventional WFST; this is then exploited in an adaptive pruning algorithm that reduces the active nodes from 30,000 down to 4,000 with only a 2 percent sacrifice in speech recognition accuracy; these optimizations lead to a more simplified design with deterministic performance.
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F. 1-12v. Calendrier en français, à l’encre rouge et noire, où l’on remarque des formes dialectales picardes et plusieurs saints du Nord : « sainte Audegonde » (30 janvier) ; « le Candelier » (2 févr.) ; « ste Gertrude » (17 mars) ; « saint Quentin » (2 mai) ; « saint Ernoulx » [ep. Suessonensis] (16 août) ; « saint Bertin » (5 septembre) ; « saint Lambert » (17 septembre) ; « saint Franchois » (4 oct.) ; « saint Gillain » [mart. in Hannonia] (16 oct., Ghislain); « le jour st Estievene » (26 déc., rouge). Une deuxième main très cursive ajouté d’autres saints, dont « s. Amand » (6 févr.) ; « s. Waleri » (1er avril) ; « s. Fuscien » [mart. Ambianensis] (27 juin et 10 déc.) ; « s. Firmin » [mart. Ambianensis] (25 septembre) ; « s. Bavon » (1er oct.). F. 13v-17v. « ... hore sancte Crucis». F. 18v-22v. « ... hore de sancto Spiritu ».F. 23v-85. « ... hore beate marie virginis secundum usum romanum ». F. 86v-94v. Office de la Vierge, selon les temps de l’année. « ... officum beate Marie virginis quod dicitur per totum adventum ad vesperas ».F.95-115. « ... septem psalmi penitentiales cum suis litaniis et precibus ». A noter « ... sancte Lamberte... sancte Rumolde [ep. Mechlinensis]... sancte Fursee [ab. Latiniacensis] ... sancte Amande... sancte Vedaste, sancte Bavo... sancte Philiberte... santa Ursula... sancta Brigida... sancta Aldegondis, sancta Gertrudis... ».F.116v-152v. « ... vigilie mortuorum » office des morts à l’usage de Sarum-Normandie-Metz ; cf. K. Ottosen, Responsories..., p. 242 (116v-151). — « Octo versus beati Bernardi ». « Illumina oculos meos ne umquam obdormiam... » ; cf. Leroquais, Livres d’heures, I, XXX-XXXI (151-152v).F.153-158v. « Initium sancti evvangelii secundum Johannem... secundum Lucam... secundum Matheum... secundum Marcum ».F. 158v-166. Suffrages aux saints : « Memoria de santo Johanne Baptista... ; devota oratio... » ; — « ... de sancto Adriano » ; — « ... de sancto Sebastiano » ; — « ... de sancto Anthonio » ; — « ... de sancto Quintino » ; — « ... de sancto Nicholae » ; — « ... de sancto Andrea » ; — « ... de sancto Fiacro ».F.166v-174v. Prières à la Vierge. « ... devotissima oratio ad beatem virginem Mariam ». « O intemerata... O Johannes... » (éd. Wilmart, Auteurs spirituels, 488-490) (166v-169v). — « Alia oratio ad beatam virginem Mariam ». « Obsecro te.... » (éd. Leroquais, Livres d’heures, II, 346-347) (169v-174). Les deux prières sont rédigées à la forme masculine. — « Laus Marie virginis ». « Salve regina... » (Analecta Hymnica, L, 318-319, n° 245) (174-174v). F. 175-184v. Suffrages aux saintes : « ... de santa Barbara » ; — « ... de sancta Margareta » ; — « ... de sancta Katerina » ; — « ... de sancta Maria Magdelena » ; — « ... de sancta Appolonia » ; — « ... de sancta Venicie [Venice, forme dérivée de Véronique] » « Veni sponsa Christi... Versus. Diffusa est gratia... Oratio. Exaudi nos Deus ut sicut de beate Venicie festivitate gaudemus, ita pie devotionis erudiamur. Per.... » ; sur le culte de sainte Véronique/Venice, en particulier à Paris, voir Gr. A. RUNNALS, Le mystère de sainte Venice, Exeter, 1980 (Textes littéraires) (175-180v). — Prières diverses : « ... quinque gaudia de beata virgine Maria » « Gaude Virgo mater Christi... » (éd. Leroquais, Livres d’heures, I, XXVI-XXVII) (180v-182). — « Oratio devota ad Jesum Christum ». « Ave domine Jhesu Christe verbum patris » (éd. Wilmart, Auteurs spirituels, 412 [III]) (182-183). — « Oratio devota que dicitur ad elevationem Domini ». « Ave verum corpus Domini nostri... » (éd. Szövérffy, Die Annalen , II, 298-299) (183-184). — « Memore de saint Miquiel », en latin (184-184v).
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Collection : Bibliothèque des mémoires relatifs à l'histoire de France pendant le 18e siècle ; 19-20
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Collection : Bibliothèque des mémoires relatifs à l'histoire de France pendant le 18e siècle ; 19-20