1,722,126 research outputs found
Leukoencephalopathy, calcifications, and cysts: Labrune syndrome
No full textLabrune syndrome is an extremely rare disorder characterized by a radiological triad of leukoencephalopathy, cerebral calcifications, and cysts. The condition is the result of an autosomal mutation in the SNORD118 gene, a non-protein encoding gene that mediates rRNA synthesis. The mutation results selectively in cerebral microangiopathy through an unknown mechanism. Radiological imaging is central to diagnosing the condition, but, because the condition is so rare, there is no standard treatment paradigm. We describe the longitudinal progression of a case of Labrune syndrome, including the radiological diagnosis and imaging and surgical management
Síndrome de Labrune – gene SNORD118 : relato de caso
No full textA Síndrome de Labrune é uma desordem rara, que tem como característica o desenvolvimento de uma microangiopatia nos vasos cerebrais que leva à seguinte tríade de alterações radiológicas no parênquima cerebral: leucoencefalopatia com presença de calcificações cerebrais e cistos (LABRUNE P, et al. 1996). Recentemente, foi descoberto o gene responsável pela microangiopatia, gene SNORD118 (JENKINSON EM, et al. 2016) porém o mecanismo envolvido na fisiopatologia ainda é incerto. Clinicamente a síndrome pode apresentar convulsões, sintomas piramidais, extrapiramidais e cerebelares, que variam de acordo com a localização das lesões (LABRUNE P, et al. 1996). O presente estudo pretende realizar um relato sobre um caso diagnosticado ainda na infância e discorrer sobre as manifestações observadas durante o acompanhamento do paciente.Labrune Syndrome is a rare disorder, characterized by the development of a microangiopathy in the cerebral vessels that leads to the following triad of radiological changes in the brain parenchyma: leukoencephalopathy with the presence of cysts and calcifications (LABRUNE P, et al. 1996). Recently, the gene responsible for the microangiopatic alterations was discovered, the SNORD118 gene (JENKINSON EM, et al. 2016), but the mechanism involved in the pathophysiology is still uncertain. Clinically, the syndrome may present seizures, pyramidal, extrapyramidal and cerebellar symptoms, which may vary according to the location of the lesions (LABRUNE P, et al. 1996). The present study intends to report on a case diagnosed in the childhood period and expatiate on the manifestations observed during the patient's follow-up
Pista colini Labrune, Lavesque, Bonifacio & Hutchings 2019
No full textPista colini Labrune, Lavesque, Bonifácio & Hutchings, 2019 Pista colini Labrune et al. 2019: 71–83, figs 2–4. Diagnosis ( for a detailed description, see Labrune et al. 2019). Live specimens with ventral shields divided in two regions, anterior part pinkish, posterior part blood red, dark pigmentation on dorsal side of the upper lip. One pair of unequal-sized pompom-like branchiae inserted on SG II, branchial filaments arranged in spiral around the central axis with dichotomous filaments. Two pairs of lateral lobes on SG II–III; well developed on SG II, with rounded anterior margins, merging with ventral shields; half width of segment, asymmetrical and slightly displaced dorsally on SG III, connected across ventrum; absent on SG IV. Neurochaetae as long-handled avicular uncini on SG V–VI, short-handled thereafter. Type locality. France, Western Mediterranean Sea, Gulf of Lion, Banyuls-sur-Mer harbour. Type material. Holotype MNHN-IA-TYPE 1850, four paratypes MNHN-IATYPE 1851 to 1855. Two other paratypes: AM W.50625, AM W.50626. Distribution. Mediterranean Sea, Gulf of Lion (Labrune et al. 2019); Aquitanian coast, Bay of Biscay (this study); English Channel, Bay of Seine (this study). Habitat. Shallow water (depth 3 m), on gravelly sands (Labrune et al. 2019), coastal waters from depths of 15–50 m, sandy bottoms (this study). Remarks. Examination of fresh specimens allowed us to add pigmentation patterns to the diagnosis, such as the dark pigmentation on the dorsal side of the upper lip of these animals.Published as part of Lavesque, Nicolas, Daffe, Guillemine, Londoño-Mesa, Mario H. & Hutchings, Pat, 2021, Revision of the French Terebellidae sensu stricto (Annelida, Terebelliformia), with descriptions of nine new species, pp. 1-63 in Zootaxa 5038 (1) on page 40, DOI: 10.11646/zootaxa.5038.1.1, http://zenodo.org/record/550286
Les «indéfinis» en coréen et en japonais
No full textChang In-Bong, Labrune Laurence. Les «indéfinis» en coréen et en japonais. In: Faits de langues, n°4, Septembre 1994. L'indéfini. pp. 73-80
Lebens-Beschreibung deß weyland Durchleuchtigsten und Weltberühmtesten Heldens Carl des V. Hertzogen zu Lothringen und Bar/ auch Generalissimi über die Käyserliche Armeen : worinnen Dessen jugendliche Erziehung/ verrichtete Heldenthaten/ und endliches Abscheiden auß dieser Zeitlichkeit außführlich enthalten ist. Bestehend in V. Büchern
No full text[Jean de Labrune]Nicht identisch mit 39:122536S (4. Schlüssels. und Fingerprint abweichend)Kupfert.: LebensBeschreibung des Herzogs von LotharingenVorlageform des Erscheinungsvermerks: Franckfurt am Mayn/ Gedruckt und Verlegt bey Philipp Fievet/ Buchdrucker und Buchhändler/ Anno 1691.Aus dem Franz. übers
Céline Labrune-Badiane (2022) - Le pari de l’école : une histoire de l’institution scolaire en Casamance 1860-1960
Get PDFCritical review: Céline Labrune-Badiane, Le pari de l’école : une histoire de l’institution scolaire en Casamance 1860-1960, Paris, Hémisphères Éditions, 2022, 374 p.Recensé : Céline Labrune-Badiane, Le pari de l’école : une histoire de l’institution scolaire en Casamance 1860-1960, Paris, Hémisphères Éditions, 2022, 374 p
Japanese /r/ is not feature-less: A rejoinder to Labrune (2014)
No full textLabrune (2014) argues that Japanese /r/ is structurally empty. This reply points out that the phonological
characteristics of /r/ which are discussed by Labrune (2014) come with many systematic exceptions,
and hence they are better modeled by violable constraints. To illustrate how violable constraints accommodate
such systematic exceptions, alternative analyses based onOptimality Theory (Prince&Smolensky, 2004)
are presented. This reply also points out that the three constraints on /r/ that are discussed by Labrune (2014)
are in fact not specific to /r/, and therefore, motivating structural emptiness based on these patterns would
face a problem of not being able to distinguish /r/ from other segments. Based on these considerations, this
reply concludes that /r/ (and other segments) in Japanese should have segmental contents
Jeanne Labrune, C'est le bouquet ! : fantaisie n° 2 sur la société contemporaine
No full textGapp Estelle. Jeanne Labrune, C'est le bouquet ! : fantaisie n° 2 sur la société contemporaine. In: Raison présente, n°144, 4e trimestre 2002. De la psychiatrie française en 2003. pp. 133-134
Séance du 28 janvier: Pierre Labrune, “Swift et les machines : entre rhétorique et mécanique, théologie et scatologie”
No full textLors de cette séance, Pierre Labrune a pris pour point de départ l'ambivalence du positionnement de Jonathan Swift à propos de la modernité et des machines, à la fois sources de fascination et de répulsion. Pierre Labrune est doctorant en Études Anglophones et ATER à l'UFR de Littérature Française et Comparée de Sorbonne Université. Il prépare actuellement une thèse intitulée « Le siècle des jargons : querelles et discours politiques, religieux et esthétiques autour du ‘cant’ dans l'Angleterr..
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