Les experts de l’Institut au congrès de la WMS 2022

Le 27ème Congrès International de la World Muscle Society (WMS) se tiendra du 11 au 15 octobre 2022 à Halifax au Canada ainsi qu’en version virtuelle sur la plateforme du congrès. Des experts internationaux du muscle seront réunis pour échanger sur la recherche fondamentale, les essais cliniques, les avancées thérapeutiques dans les maladies neuromusculaires. Les chercheurs et cliniciens de l’Institut seront présents, à la fois comme modérateurs au cours de sessions mais également comme ayant pris part à des travaux qui seront mis en avant lors de communications orales et de posters :

 

Mercredi 12 octobre 2022

  • 11:00-13:00 – Session New Developments in Congenital Myopathies 2 – Invited lectures and Selected oral presentations
    Modératrices: Kristl Claeys et Andreea Seferian

Présentation orale O.06 : Long term outcomes for X-Linked Myotubular Myopathy (XLMTM) with gene replacement therapy, resamirigene bilparvovec: preliminary results from ASPIRO. P. Shieh; N. Kuntz; J. Dowling; W. Müller-Felber; A. Blaschek; C. Bönnemann; R. Foley; D. Saade; A. Seferian; L. Servais; A. Bowden; M. Sarazen; J. Coats; N. Lakshman; C. Han; S. Prasad; S. Rico; W. Miller

 

  • 14:30-16:00 – Session Imaging and new tools and approaches for NMDs
    Modérateur: Jordi Díaz-Manera

Présentation d’un « Flash poster » sélectionné : From the Muscle Atlas to an AI-based diagnostic tool. C. Meyer; E. Lacene; M. Beuvin; T. Evangelista; J. Laporte; A. Jeannin-Girardon; P. Collet; O. Poch; N. Romero; K. Chennen; B. Cadot

 

Poster 31 : A multiparametric quantitative NMR study at rest and during exercise in subjects between 22 and 65 years of age: Preliminary results. A. Lopez Kolkovsky; B. Matot; P. Baudin; H. Reyngoudt; B. Marty; Y. Fromes

 

Poster 32 : Impact of age on muscle volume and T2-relaxation time during adulthood in mice using quantitative MRI. B. Matot; E. Caldas de Almeida Araujo; P. Baudin; H. Reyngoudt; B. Marty; Y. Fromes

 

16:00-17:30 Session DMD – Biomarkers/Outcome Measures
Modératrice: Linda Lowes

Présentation d’un « Flash poster » sélectionné : Application for primary endpoint qualification of the 95th centile of stride velocity (SV95C) in Duchenne muscular dystrophy. M. Annoussamy; D. Eggenspieler; A. Seferian; E. Mercuri; V. Straub; F. Muntoni; M. Scoto; M. Poleur; A. Daron; N. Butoianu; A. Mirea; N. Goemans; S. Previtali; M. Tulinius; A. Nascimento; P. Heydemann; M. Panzara; T. Singh; P. Strijbos; L. Servais

 

Poster 57 Longitudinal changes in fat fraction histograms using quantitative MRI in Duchenne muscular dystrophy. H. Reyngoudt; P. Baudin; E. Caldas de Almeida Araujo; B. Wong; P. Carlier; B. Marty

 

Poster 62 Increased skeletal muscle extracellular volume fraction in patients with Becker muscular dystrophy assessed by quantitative magnetic resonance imaging. B. Marty; P. Baudin; Y. Fromes; K. Wahbi; H. Reyngoudt

 

Virtual Poster 24 T Cell-mediated immune response to dystrophin in Duchenne muscular dystrophy – A natural history study. K. Anthony; P. Ala; F. Catapano; J. Meng; J. Domingos; M. Perry; V. Ricotti; K. Maresh; L. Phillips; V. Straub; M. Guglieri; L. Servais; A. Seferian; S. De Lucia; I. de Groot; Y. Krom; J. Verschuuren; E. Niks; T. Voit; J. Morgan; F. Muntoni

 

Virtual Poster 26 Potential of the MyoSuit, a lightweight wearable lower-limb cable-actuated exoskeleton in patients with neuromuscular disorders: preliminary findings. R. Feigean; C. Afroun; E. Gasnier; O. Benveniste; J. Hogrel; G. Bassez; D. Bachasson

 

Virtual Poster 27 Potential of the Keeogo+, a lightweight wearable powered assistive exoskeleton in patients with neuromuscular disorders: preliminary findings. R. Feigean; C. Afroun; E. Gasnier; O. Benveniste; G. Bassez; J. Hogrel; D. Bachasson

 

16:00-17:30 Session Metabolic Myopathies

Virtual Poster 34 Two-year follow-up of muscle strength and function in patients with glycogen storage disease type IIIa. V. Decostre; M. Masingue; P. Laforêt; R. Ben Yaou; P. Labrune; J. Hogrel

 

16:00-17:30 Session Immune mediated – and NMJ-related NMDs

Poster 93 Effect of rapamycine on quantitative MRI outcome measures in inclusion body myositis. H. Reyngoudt; D. Bachasson; J. Hogrel; P. Baudin; Y. Allenbach; P. Carlier; O. Benveniste; B. Marty

 

Virtual Poster 40 Exploring barriers and facilitators to physical exercise in autoimmune myasthenia gravis: the MYaEX study. S. Birnbaum; A. Archer; C. Stalens; J. Lejeune; J. Hogrel

 

16:00-17:30 Session SMA – Therapy

Poster 109 FIREFISH Parts 1 and 2: 36-month safety and efficacy of risdiplam in Type 1 spinal muscular atrophy (SMA). L. Servais; G. Baranello; O. Boespflug-Tanguy; J. Day; N. Deconinck; A. Klein; R. Masson; M. Mazurkiewicz-Bełdzińska; E. Mercuri; K. Rose; D. Vlodavets; H. Xiong; E. Zanoteli; M. El-Khairi; M. Gerber; K. Gorni; H. Kletzl; L. Palfreeman; A. Dodman; E. Gaki; B. Darras

 

Jeudi 13 octobre 2022

14:30-16:00 Session Other myopathies & muscular dystrophies

Poster 145 Identification of potential genetic modifiers underlying phenotypic variability in a French family with striated muscle laminopathies. L. Benarroch; A. Bertrand; M. Beuvin; I. Nelson; N. Naouar; F. Simonet; C. Dina; C. Pionneau; J. Schott; R. Ben Yaou; G. Bonne

 

Poster 157 Dominant HSPB6 mutation in a myopathy patient. J. Sarparanta; P. Jonson; A. Vihola; H. Luque; A. Vainio; R. Villar-Quiles; T. Stojkovic; N. Romero; B. Eymard; B. Udd

 

Virtual Poster 70 OPALE: a patient registry for laminopathies and emerinopathies in France. R. Ben Yaou; F. Anselme; A. De Sande-Giovannoli; E. Campanna- Salort; P. Charron; C. Chikhaoui; I. Jeru; F. Labombarda; F. Leturcq; S. Quijano-Roy; C. Stalens; P. Richard; C. Vigouroux; G. Bonne; K. Wahbi

 

14:50-16:00 Session LGMD
Modérateur: Nicholas Johnson

Présentation d’un « Flash poster » sélectionné : Clinical outcome study of dysferlinopathy: correlation between MRI fat fraction in lower limbs and clinical outcome assessments over a 3 year period. F. Smith; H. Reyngoudt; J. Díaz Manera; M. James; I. Wilson; E. Caldas de Almeida Araujo; C. Bolano Diaz; H. Gordish Dressman; L. Rufibach; A. Mayhew; K. Jones; E. Salort Campana; M. Walter; T. Stojkovic; M. Yoshimura; E. Pegoraro; J. Mendell; V. Straub; A. Blamire; P. Carlier

 

Présentation d’un « Flash poster » sélectionné : Myostatin concentration is unreliable as a biomarker of disease progression in dysferlinopathy. U. Moore; E. Fernandez Simon; J. Day; K. Jones; D. Bharucha-Goebel; A. Pestonk; M. Walter; C. Paradas; T. Stojkovic; E. Bravver; E. Pegoraro; J. Mendell; M. Guglieri; V. Straub; J. Díaz Manera

 

Poster 163 Quantitative MRI in upper limb muscle of patients with dysferlinopathy: preliminary baseline results of the natural history study Jain COS2. H. Reyngoudt; F. Smith; I. Wilson; E. Caldas de Almeida Araujo; B. Marty; P. Baudin; J. Díaz-Manera; L. Rufibach; H. Gordish Dressman; H. Hilsden; H. Sutherland; G. Querin; T. Stojkovic; V. Straub; P. Carlier; A. Blamire

 

Poster 164 Clinical outcome study of dysferlinopathy 2: characterising involvement of the intrinsic muscles of the hand in LGMDR2. M. James; A. Mayhew; H. Gordish-Dressman; L. Rufibach; K. Wong; W. Roper; S. Holsten; L. Lowes; T. Duong; C. Yochai; A. Zabala Pardo; Y. Ogasawara,; K. Rudolph; S. Alarcon; J. Weber; E. Montiel Morillo; I. Pedrosa- Hernandez; S. Birnbaum; J. Rojas Rojas; J. Day; V. Straub

 

Poster 165 Clinical outcome study of dysferlinopathy: lower limb water T2 predicts functional decline in patients with dysferlinopathy. U. Moore; E. Caldas de Almeida Araujo; H. Reyngoudt; H. Gordish-Dressman; F. Smith; J. Wilson; M. James; A. Mayhew; L. Rufibach; T. Stojkovic; A. Blamire; V. Straub; P. Carlier; J. Díaz Manera

 

Poster 173 Preliminary natural history quantitative MRI data in lower limb muscle and heart of patients with limb-girdle muscular dystrophy type R9. H. Reyngoudt; Y. Fromes; M. Granier; P. Baudin; G. Querin; V. Straub; T. Stojkovic; S. Olivier; J. Vissing; B. Marty

 

Poster178 Clinical classification of variants in the valosin containing protein gene associated with multisystem proteinopathy. J. Díaz Manera; M. Schiava; C. Ikenaga; T. Stojkovic; I. Nishino; S. Nair; G. Manousakis; C. Quinn; Z. Sahenk; M. Monforte; A. Oldfords; E. Pal; B. Velez Gomez; J. de Bleecker; M. Farrugia; M. Harms; S. Ralston; J. Sotoca Fernandez; J. Bevilacqua; C. Weihl & the VCP M Study Group

 

17:00-18:00 Session Neuropathies and Non-5q Motor Neuron Diseases

Poster 217 Cervical spinal cord MRI parameters as predictors of early degeneration in asymptomatic C9Orf72 carriers: a longitudinal study. G. Querin; D. Saracino; D. Rinaldi; F. Salachas; V. Marchand-Pauvert; J. Cohen Adad; I. Le Ber; P. Pradat

 

Vendredi 14 octobre 2022

17:10-17:20 Myotonic Dystrophy
Modérateur : Benedikt Schoser

Présentation d’un « Flash poster » sélectionné : Exploring the role of MuscleBlind-Like proteins in the regulation of CaVB1 isoform expression in adult skeletal muscle. A. Vergnol; A. Sureau; M. Traore; G. Gourdon; D. Furling; F. Pietri-Rouxel; S. Falcone

 

Samedi 15 octobre 2022

8:30-9:00 Keynote Lecture
Modérateurs: Volker Straub et Teresinha Evangelista

13:00-13:10 Session Late Breaking News
Modérateurs: Ichizo Nishino et Lindsay Alfano

Présentation orale sélectionnée 7 Repeat expansions in the 5’UTR of ABCD3 cause oculo-pharyngo-distal myopathy with possible sex-biased penetrance. Cortese A, Beecroft S, Cabrera-Serrano M, Curro R, Facchini S, Stevanovski I, Chintalaphani S, Delatycki M, Storey E, Stojkovic T, Houlden H, Kennerson M, McLean C, Deveson I, Laing N, Lamont P, Lockhart P, Fahey M, Bugiardini E, Ravenscroft G

 

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