Michel Fardeau's Books

Pediatric Neurology Part III

Chapter 136. Diagnostic workup for neuromuscular diseases

By: Michel Fardeau,Isabelle Desguerre

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Pediatric Neurology Part III

Chapter 136. Diagnostic workup for neuromuscular diseases

By: Michel Fardeau,Isabelle Desguerre

Clinical symptoms of neuromuscular diseases vary according to age and type of primary involvement (spinal motor neuron, nerve, neuromuscular junction or muscle). Tools at our disposal for diagnostic purposes are graduated based on the age of the patient and diagnostic suspicions generated by the clinical workup. Seven clinical presentations can be identified that all require technical facilities specifically dedicated to pediatric neuromuscular diseases: congenital hypomobility and arthrogryposis, paralytic hypotonia in infancy, motor delay and chronic walking difficulties after the age of 18 months, progressive walking difficulties after the age of 3 years, effort intolerance and acute rhabdomyolysis, acute motor symptoms or fatigability, and variability of symptoms. Electrophysiological investigation, particularly electromyography, is a valuable tool where neurogenic involvement or neuromuscular block is suspected. However, the technique is difficult to perform in children. Muscle biopsy is generally the key investigation and can be performed at any age. Molecular biology helps to improve diagnostic strategy. Muscle MRI, in combination with clinical evaluation, assists the selection of appropriate genetic tests and more generally the identification of genetically distinct forms of neuromuscular disorder. None of these are by any means routine investigations, and only a specialized multidisciplinary clinical approach can permit correct diagnosis and proper follow-up.

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Elsevier Inc. Chapters

Published Date

ISBN 10

0128084022

ISBN 13

9780128084021

Pediatric Neurology Part III

Chapter 140. Myofibrillar myopathies

By: Kristl G. Claeys,Michel Fardeau

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Pediatric Neurology Part III

Chapter 140. Myofibrillar myopathies

By: Kristl G. Claeys,Michel Fardeau

Myofibrillar myopathies (MFMs) are rare, inherited or sporadic, progressive neuromuscular disorders with considerable clinical and genetic heterogeneity. MFMs are defined morphologically by foci of myofibril dissolution that begins at the Z-disk, accumulation of myofibrillar degradation products, and ectopic expression of a large number of proteins including desmin. To date, mutations in six genes are known to cause MFMs, accounting for approximately half of the MFM patients identified. The causative genes encode mainly sarcomeric Z-disk(-related) proteins: desmin, αB-crystallin, myotilin, Z-band alternatively spliced PDZ motif containing protein (ZASP), filamin C and the antiapoptotic BCL2-associated athanogene 3 (Bag3). Although in most MFM patients the disease presents in adulthood and evolves slowly, some patients with desminopathy, αB-crystallinopathy or Bag3opathies have an infantile or juvenile disease onset. Cardiac involvement is very common in desminopathies and can sometimes be the initial or only symptom of the disease. Respiratory symptoms are noted during childhood in αB-crystallinopathies. Early severe cardiac and respiratory involvement is seen in Bag3opathies. Optical microscopic and immunohistochemical features are similar in MFMs; however, ultrastructural findings can be useful to differentiate between the distinct MFM subtypes. No curative treatment for MFMs is currently available. Careful follow-up, especially of cardiac and respiratory function, is important.

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Elsevier Inc. Chapters

Published Date

ISBN 10

0128084065

ISBN 13

9780128084069

Pictures, Passions and Eye

A Life at Sotheby's

By: Michel Strauss

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Pictures, Passions and Eye

A Life at Sotheby's

By: Michel Strauss

Michel Strauss embarked on an enduring love affair at the age of six when he saw for the first time paintings by Manet, Monet and Degas: the passion aroused by these artists never left him. This passion, this 'eye' as he calls it led to his becoming Head of the Impressionist Department at Sotheby's where he remained for forty years. He describes the personalities he met along the way: the collectors, the dealers, the colleagues and even the forgers, as well as the clients who shared his passion. There were times of boom and times of recession, there were very difficult times -in particular the anti-trust era -and there were times that brought great delight and a sense of achievement, in particular the British Rail Pension Fund sale which Michel had helped set up and which exceeded all expectations. An authoritative and highly respected figure in the art world, Michel Strauss has handled the greatest of all Impressionist works, some of which it was thought had been lost forever.

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294

Publisher

Halban

Published Date

ISBN 10

1905559682

ISBN 13

9781905559688

Unreconciled

Poemes 1991-2013

By: Michel Houellebecq

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Unreconciled

Poemes 1991-2013

By: Michel Houellebecq

Selected poems from the critically acclaimed author of Submission and The Elementary Particles A shimmering selection of poems chosen from four collections of one of France’s most exciting authors, Unreconciled shines a fresh light on Michel Houellebecq and reveals the radical singularity of his work. Drawing on themes that are similar to the ones in his novels, these poems are a journey into the depths of individual experience and universal passions. Divided into five parts, Unreconciled forms a narrative of love, hopelessness, catastrophe, dedication, and—ultimately—redemption. In a world of supermarkets and public transportation, indifferent landscapes and lonely nights, Houellebecq manages to find traces of divine grace even as he exposes our inexorable decline into chaos. Told through forms and rhythms that are both ancient and new, with language steeped in the everyday, Unreconciled stands in the tradition of Baudelaire while making a bold new claim on contemporary verse. It reveals that in addition to his work as an incisive novelist, Houellebecq is one of our most perceptive poets with a vision of our era that brims with tensions that cannot—and will not—be reconciled.

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337

Publisher

Farrar, Straus & Giroux

Published Date

ISBN 10

0374279640

ISBN 13

9780374279646

E Pluribus Unum:Liber Amicorum Georges A.L. Droz - on the Progressive Unification of Private International Law

By: Georges Droz,Alegría Borrás,Andreas Bucher,Michel Verwilghen,Teun Struycken

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E Pluribus Unum:Liber Amicorum Georges A.L. Droz - on the Progressive Unification of Private International Law

By: Georges Droz,Alegría Borrás,Andreas Bucher,Michel Verwilghen,Teun Struycken

The unification of Private International Law is a goal to which all the contributors to this impressive volume have committed themselves, and one which seems increasingly to attract the attention of legal practitioners, researchers, writers and legislators. The essays give a unique overview of the current state of the law with respect to those areas which have been unified, or which are susceptible to unification. Insights are given into national as well as international practice, and theoretical aspects have not been neglected.

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594

Publisher

Kluwer Law International B.V.

Published Date

ISBN 10

9041102825

ISBN 13

9789041102829

A Matter of Blue

Poems

By: Jean-Michel Maulpoix

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A Matter of Blue

Poems

By: Jean-Michel Maulpoix

In A Matter of Blue, we read that blue is what we would like to cultivate, something that clings to bees' feet and the poet's lips, something that can be used as a basis for composition or creation, something that is inherent in the gaze of the dark-eyed women . . ."--Dawn Cornelio A Matter of Blue is the most successful book by Maulpoix, author of over 25 French collections of poetry and the rightful heir to the 150-year tradition of French prose poetry. Jean-Michel Maulpoix (www.maulpoix.net) is director of a quarterly literary journal and professor of poetry at University Paris X-Nanterre. Dawn Cornelio wrote her PhD thesis on translating Maulpoix. She is assistant professor of French studies at University of Guelph, Ontario.

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204

Publisher

BOA Editions, Ltd.

Published Date

ISBN 10

1929918674

ISBN 13

9781929918676

Statues

The Second Book of Foundations

By: Michel Serres

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Statues

The Second Book of Foundations

By: Michel Serres

In this first English translation of one of his most important works, Michel Serres presents the statue as more than a static entity: for Serres it is the basis for knowledge, society, the subject and object, the world and experience. Serres demonstrates how sacrificial art founded and still persists in society and reflects on the centrality of death and the statufied dead body to the human condition. Each section covers a different time period and statuary topic, ranging from four thousand years ago to 1986; from Baal, the paintings of Carpaccio, and the Eiffel Tower, to Rodin's The Gates of Hell, the Challenger disaster and the literature of Maupassant, La Fontaine and Jules Verne. Expository, lyrical, fictionalized and hallucinatory, Statues plays with time and place, history and story in order to provoke us into thinking in entirely new ways. Through mythic and poetic meditations on various kinds of descent into the underworld and new insights into the relation of the subject and object and their foundation in death, Statues contains great treasures and provocations for philosophers, literary critics, art historians and sociologists.

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223

Publisher

Bloomsbury Publishing

Published Date

ISBN 10

1472522060

ISBN 13

9781472522061

La Renovation du Shi'isme Ismaelien En Inde Et Au Pakistan

D'apres les Ecrits et les Discours de Sultan Muhammad Shah Aga Khan

By: Michel Boivin

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La Renovation du Shi'isme Ismaelien En Inde Et Au Pakistan

D'apres les Ecrits et les Discours de Sultan Muhammad Shah Aga Khan

By: Michel Boivin

This French-language book is the first to propose a scientific approach to the Aga Khan's religious thought, placing it in its proper perspective by revealing how the Aga Khan responded to contemporary challenges. It will be of interest to both students and scholars of history, orientalism and Islamic thought and cultures, and to anyone interested in South Asia or in the fundamental issues of religion and modernity.

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504

Publisher

Routledge

Published Date

ISBN 10

1136850198

ISBN 13

9781136850196

Haunted Childhoods

By: Pauline Michel

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Haunted Childhoods

By: Pauline Michel

Children travel across generations and across time: yesterday to the present. Childhoods determine the fantasms of existence. Wounds slow to heal: abandonment, adoption, feelings of rejection and uselessness, of blame, of uncontrolled initiation to sexuality, extreme loneliness when one retreats from society, obsessive searching for the absolute, desire to rebuild the world again by giving birth, confrontation of death. All these themes appear in the violent yet tender stories in this collection, rather like tunnelling through to a rebirth.

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188

Publisher

Dundurn

Published Date

ISBN 10

1459703170

ISBN 13

9781459703179

Impersonals and other Agent Defocusing Constructions in French

By: Michel Achard

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Impersonals and other Agent Defocusing Constructions in French

By: Michel Achard

This book investigates French impersonals as a functional category. Any structure whose agent is defocused and whose predicate describes a situation stable enough to be generally available should be considered impersonal. In addition to il impersonals, the category also includes demonstrative (ce/ça), middle (se), and indefinite (on) structures. These different forms belong to the same functional category because they systematically code general and predictable events that cannot be imputed to a specific cause. Because generality and predictability are gradual notions, impersonals can only be identified within the context of specific constructional islands which therefore constitute the organizing principle of the French impersonal category. Conducted in Cognitive Grammar, the analysis follows the functional tradition in expanding the scope of French impersonals beyond il constructions, but also proposes a way of precisely delineating the category. This book will be of interest to anyone interested in impersonal constructions and French linguistics.

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384

Publisher

John Benjamins Publishing Company

Published Date

ISBN 10

9027269076

ISBN 13

9789027269072

Voyage secret

By: Michel Giliberti

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Voyage secret

By: Michel Giliberti

Ce livre ressemble l'œuvre peint de Giliberti, inspiré par ses voyages en Tunisie. Il nous fait pénétrer dans le secret des lumières de son pays natal et nous invite à boire aux sources d'un présent chargé des souvenirs de son enfance. Une immense sérénité, des couleurs, des impressions et une paix retrouvée se dégagent de ces scènes attachantes aux portes de l'intime. Une peinture à laquelle cet artiste tourmenté ne nous a pas habitués. (Jean-Charles Fischhoff)

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193

Publisher

Editions BONOBO

Published Date

ISBN 10

2952040540

ISBN 13

9782952040549

Le christianisme bogomile cathare

By: Michel Mounié

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Le christianisme bogomile cathare

By: Michel Mounié

Ce livre révèle la vérité concernant cette hérésie qui fut un tournant de l'histoire de l'Europe. L'auteur y fait des analyses approfondies détaillées et trouve des liens entre les idées bogomiles et plusieurs contes de différents peuples du continent.

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Page Count

618

Publisher

Lulu.com

Published Date

ISBN 10

1471685683

ISBN 13

9781471685682

Book's by Michel Fardeau

Pediatric Neurology Part III

Pediatric Neurology Part III

Pediatric Neurology Part III

Pediatric Neurology Part III

Pictures, Passions and Eye

Pictures, Passions and Eye

Unreconciled

Unreconciled

E Pluribus Unum:Liber Amicorum Georges A.L. Droz - on the Progressive Unification of Private International Law

E Pluribus Unum:Liber Amicorum Georges A.L. Droz - on the Progressive Unification of Private International Law

A Matter of Blue

A Matter of Blue

Statues

Statues

La Renovation du Shi'isme Ismaelien En Inde Et Au Pakistan

La Renovation du Shi'isme Ismaelien En Inde Et Au Pakistan

Haunted Childhoods

Haunted Childhoods

Impersonals and other Agent Defocusing Constructions in French

Impersonals and other Agent Defocusing Constructions in French

Voyage secret

Voyage secret

Le christianisme bogomile cathare

Le christianisme bogomile cathare

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