Myasthenia gravis

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Overview of neuromuscular diseases

After a brief reminder of the structure of the motor unit and the various modes of inheritance, this document provides short descriptions of the neuromuscular diseases that are part of our scope at AFM-Téléthon, as well as how to manage and treat them. For each group of diseases, as well as in the motor unit … [Read more]

A consensus on the management of autoimmune myasthenia in the five Nordic countries

Clinicians from the five Nordic countries (Norway, Sweden, Finland, Denmark and Iceland) have shared their experience in the management of generalised autoimmune myasthenia with autoantibodies against the acetylcholine receptor, and issued recommendations: symptomatic treatment with pyridostigmine (or equivalent) remains the basis of management, the role of corticosteroid therapy, traditional immunosuppressants and new molecules such as … [Read more]

Sleep disordered breathing in slowly progressive myopathies: beyond alveolar hypoventilation

A French retrospective study of the records of 149 adults with an average age of 46.5 years and suffering from myotonic dystrophy (45) , myasthenia (20), Pompe disease (9), spinal muscular atrophy (8), facioscapulohumeral myopathy (8), Duchenne myopathy (5), Becker myopathy (1) or another neuromuscular disease (53) explored by respiratory polygraphy, nocturnal transcutaneous capnography and … [Read more]

Intermediate data confirm good results for efgartigimod in myasthenia gravis

An open-label extension of the international Adapt trial, Adapt+ involved 151 adults with generalized autoimmune myasthenia gravis treated with cycles of four weekly infusions of Vyvgart® (efgartigimod) over three years, according to a dosage schedule adapted to the course of the disease. After an average of 18 months of treatment and follow-up, the investigators noted … [Read more]

The rs1800628 A allele of TNF-α, a risk factor for myasthenia and a lack of rapid response to corticosteroids

A Chinese team studied TNF-α polymorphisms in 409 adults with autoimmune myasthenia and 487 controls. They found that : the rs1800628 A allele is significantly more frequent in people with myasthenia than in controls ; its frequency was also higher in the subgroups of early onset, myasthenia without thymoma, ocular onset and moderate severity; similarly, … [Read more]

An expert opinion on the (good) results of rozanolixizumab in myasthenia gravis

Three neurologists from Toronto’s Centre for Neuromuscular Diseases have analyzed the findings of various trials of rozanolixizumab (Rystiggo®), a monoclonal antibody directed against neonatal Fc receptors (anti-FcRn), in autoimmune myasthenia with anti-RACh or anti-MuSK antibodies. They conclude that : rozanolixizumab is effective, reducing pathogenic IgG by up to -78%, similar to plasma exchange; it also … [Read more]

Pregnancy planning: an effective way to reduce the serious maternal complications associated with myasthenia gravis

A North American online study gathered 59 women who had 90 pregnancies in the decade following the diagnosis of autoimmune myasthenia : 62.2% of these happy events were planned, the remaining, unplanned pregnancies were accompanied by an increased risk of disease exacerbation (37.7% vs. 13.7%), hospitalization (26.5% vs. 11%) and maternal admission to intensive care … [Read more]

Robotic-assisted versus open thymectomy in myasthenia gravis with thymoma, Italian results

A multicenter retrospective study conducted in Italy on 213 patients with autoimmune myasthenia operated on between 2011 and 2021 for a thymoma, advanced stage (Masaoka-Koga III or IV) for 24 of them, shows that: 110 had an open thymectomy (sternotomy or lateral or posterolateral thoracotomy), the others a robot-assisted thymectomy; open surgery was associated with … [Read more]

Results of phase II trial of nipocalimab in myasthenia gravis published

Following an initial presentation at the 2021 American Academy of Neurology meeting, the final (and more moderate) results of the Vivacity-MG trial were published in the journal Neurology in January 2024 : 57 of the 68 participants completed 57 days of treatment with placebo or nipocalimab 5, 30 or 60 mg; the incidence of side-effects … [Read more]

An update of European recommendations on the use of radiological contrast products in myasthenia gravis

Myasthenia gravis is included in the “precautions for use” of iodinated contrast products because of a possible worsening of the symptoms of the disease caused by their injection. After analysing the sometimes contradictory results of three retrospective studies, the Contrast Products Safety Committee of the European Society of Urogenital Radiology updated its recommendations on the … [Read more]