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Institut de la Myologie

Newsletter #78

The Institute of Myology teams are mobilising to tackle Covid-19!

Welcome to our 78th newsletter.

Our health professionals and researchers, who are on the frontline at the Pitié-Salpêtrière Hospital in Paris, are mobilising to help patients.

All the health professionals and care practitioners in the Neuro-Myology Department have met to identify, as a priority, the most fragile neuromuscular patients in order to ensure their continued care and follow-up in a strengthened health framework, and thus to provide them with all the necessary help they need (reception, tele-consultation, follow-up, etc.). If you have any questions related to your neuromuscular disease, please contact your country’s health authorities.

It is worth noting that almost 30 individuals have also volunteered from the Institute of Myology teams, to offer their expertise and to share their skills, in order to participate in the national effort against the epidemic. Researchers, technicians, engineers and laboratory staff have all mobilised in large numbers to support the Paris Public Hospital system (AP-HP) teams in their missions and to drive back the virus.

Also, all the Institute of Myology support teams and the staff from various organisations and partners (AIM, AP-HP, CEA, INSERM, Sorbonne University) have mobilised via the Continuity of Operations Plan to continue to support patients and their families, care and research in the context of an epidemic that is presenting a number of health challenges.

This wonderful show of solidarity is an example to us all. Let us all remain attentive and responsive, so that we can contribute to the collective effort. It is by pooling our vital forces and our resources, together in a committed manner, that we will succeed in combating this epidemic and defeating the virus!

In order to help health professionals to face this epidemic, there is a single rallying cry: Follow the recommendations made by your competent authorities and stay at home!

At the Institute
Development of MyoQuad to evaluate quadriceps strength – Interview with J.-Y. Hogrel Jean-Yves Hogrel, Head of the Physiology and Neuromuscular Evaluation Laboratory, has just published an article relating to the validation of the MyoQuad device among patients with inclusion body myositis, a tool created and developed in this laboratory and a recent addition to the Myotools family. Read more

Summer School of Myology 2020 postponed For obvious reasons related to the Covid-19 outbreak, the organizers have decided to postpone the Summer School of Myology, initially scheduled for next June in Paris. Read more

Our latest news
An international consensus of experts is proposing to advance the diagnostic criteria for dermatomyositis and divide the disease into six subtypes The current diagnostic criteria for dermatomyositis are solely clinical and histological (perifascicular atrophy, vasculopathy), and do not take into account myositis-specific autoantibodies identified in the last few years. Read more

Clinical, morphological and genetic characterization of Brody disease Brody disease is an autosomal recessive myopathy characterized by exercise-induced muscle stiffness due to mutations in the ATP2A1 gene. Little is known about the natural history of the disorder, genotype-phenotype correlations, and the effects of symptomatic treatment. Read more

Brody myopathy demonstrates a pseudo-increment on repetitive nerve stimulation Diagnosis of Brody myopathy relies on needle electromyography showing electrical silence, muscle biopsy with decreased sarcoplasmic reticulum calcium adenosine triphosphatase activity, and genetic analysis. Read more

Adenylosuccinic acid therapy improves mdx mice ASA treatment significantly improved the histopathological features of murine DMD by reducing damage area, the number of centronucleated fibres, lipid accumulation, connective tissue infiltration and Ca2+ content of mdx tibialis anterior. Read more

Increased dystrophin production with golodirsen in patients with DMD The first results of the clinical trial evaluating golodirsen (Vyondys 53 - SRP-4053) in Duchenne muscular dystrophy show a production of dystrophin in the muscle after 48 weeks of treatment. Read more

Estrogen, estrogen-like molecules and autoimmune diseases Mainly prevalent in women, autoimmune diseases are due to thymic tolerance processes breakdown. The female sex hormone, estrogen, is involved in this AD female susceptibility. Read more

Role of aldehyde dehydrogenases in skeletal muscle homeostasis Aldehyde dehydrogenases (ALDHs) are key players in cell survival, protection, and differentiation via the metabolism and detoxification of aldehydes. Read more

More breaking news
QuantiMus: a machine learning-based approach for high precision analysis of skeletal muscle morphology The authors introduce QuantiMus, a free software program that uses machine learning algorithms to quantify muscle morphology and molecular features with high precision and quick processing-time. Read more

Zilucoplan obtains positive results in moderate to severe generalized myasthenia gravis with anti-RACh autoantibodies A clinical trial, including 44 patients conducted in 25 investigation centers in the US and Canada, concludes with the positive effects of zilucoplan in generalized autoimmune myasthenia gravis. Read more

Spinal muscular atrophy
Duchenne muscular dystrophy
Inflammatory myopathies
Pompe disease
Other neuromuscular diseases
In brief
ERN EURO-NMD bursary scheme The European Reference Network EURO-NMD launches a call for applications for grants intended to allow young researchers and doctors to attend scientific meetings on neuromuscular diseases. Read more

Exercise & NMD: next EURO-NMD webinar - 23 April 2020 This webinar will be delivered by Nicole Voet from Klimmendaal – Polikliniek Neuromusculaire Aandoeningen in the Netherlands. Read more

World Muscle Society 2020 - 30 September-4 October 2020 – Halifax, Canada WMS25 will take place in Halifax, Canada, next fall. Read more

World Duchenne Awareness Day - 7 September 2020 7 September 2020 marks Duchenne World Myopathy Day, an opportunity to take part in the event and share your stories. Read more

Job opportunities The Institute of Myology is recruiting a Helpdesk Assistant (M/F) and a Grant office manager (M/F). Read more

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Every two months, the newsletter from the Institute of Myology informed you on developments in myology research, with a summary of the latest scientifics news, medical, political and community about neuromuscular diseases. You can read our newsletter by subscribing. You can unsubscribe here.
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This is the newsletter of current medical science from the Institute of Myology. It is published every two months. Chief Editor: Vincent Varlet. Editorial Board: Bertrand Fontaine; Emmanuelle Guiraud; Delphine Olivier; J. Andoni Urtizberea; Vincent Varlet. Editorial staff: Anne Berthomier. Also participate: Lawrence Chambers; Hala Alameddine. Do you have any questions? Would you like to share some news? Please contact us. © 2020 - AFM - Institut de Myologie. ISSN 1772-9866