Myasthenia gravis

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The value of eculizumab in refractory myasthenia gravis and a possible switch to ravulizumab

Japanese clinicians report their real-life experience of administering new anti-complementary molecules in a series of 36 adults with a refractory form of myasthenia gravis (3% of a total cohort of 1,106 myasthenic patients): the 36 patients concerned had received eculizumab (Soliris®) as first-line treatment for an average of 35 months, clinical benefit was observed in … [Read more]

Preserving oral health when taking anti-CD20 drugs

A team from Nice University Hospital reports: a series of six patients aged 34 to 79 who presented with oral disorders (pain, dental abscess or fracture, periodontitis…) 10 days to two years after starting anti-CD20 therapy (rituximab or ocrelizumab) for autoimmune myasthenia, multiple sclerosis or neuromyelitis optica ; the Vigibase worldwide pharmacovigilance database has recorded … [Read more]

Can a simple blood count predict a myasthenic crisis?

According to a retrospective study conducted in Germany on 15 patients who had suffered a total of 21 myasthenic attacks and 43 patients who had not suffered any: an increase in basophils, neutrophils, total leucocytes and platelets was associated with a higher risk of myasthenic attacks, conversely, other haemogram parameters, CRP levels, sex and type … [Read more]

A Canadian survey highlights the very varied levels of knowledge of many adult neurologists regarding corticosteroid therapy

Adult neurologists specialising in neuromuscular diseases often prescribe long-term corticosteroids, particularly for autoimmune diseases (myositis, myasthenia gravis, etc.): an online survey of 99 Canadian neurologists practising in their country of origin or elsewhere was carried out, analysis of the responses from the 71 actual participants revealed shortcomings both in the screening of patients before starting … [Read more]

Chinese study highlights importance of cognitive impairment in myasthenia gravis

The existence of cognitive disorders in autoimmune myasthenia is still debated. Chinese researchers have investigated this question using a battery of tests: 41 patients with a generalized form of myasthenia participated in a study that also included a control group of 45 people matched for gender, age and education level, the protocol consisted of neuropsychological … [Read more]

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]