Blog Archives
Efgartigimod active on different muscle groups in myasthenia gravis
A post-hoc analysis of the results of the Adapt trial, which was conducted in France in 167 adults with a generalised form of autoimmune myasthenia gravis, showed that efgartigimod resulted in : an improvement, greater than on placebo, in each of the muscle sub-domains (respiratory, ocular, bulbar, limb) assessed by the MG-ADL score, observed from … [Read more]
The development of arimoclomol stops in inclusion myositis
With a prevalence of 1 in 200,000 in Europe, sporadic inclusion myositis is the most common idiopathic myositis after the age of 50. It is characterised by muscle damage, often asymmetric with amyotrophy, and begins in the quadriceps and/or finger flexors. Muscle biopsy shows two phenomena: inflammation and degeneration with accumulation of various proteins (beta-amyloid, … [Read more]
Louise Benarroch wins Elsevier Runner Up Award at WMS 2023 – Interview
Louise Benarroch is a post-doctoral researcher in the “Myomatrix & Myonucleus Related Diseases: Genetics & Pathophysiology” team led by Gisèle Bonne in the Institute’s CRM. Her latest work on the characterisation of a cellular model for muscle diseases was published* at the end of August, and was also presented at the 28th International Congress of … [Read more]
An overview of the management of mitochondrial diseases in Europe
To gain a better understanding of the needs in terms of diagnosis, management and training in primary mitochondrial myopathies in Europe, a survey was carried out among 220 healthcare professionals in 31 European countries. Its findings highlight persistent difficulties: the diagnosis and management of these diseases remain complex and vary from country to country. Comprehensive … [Read more]
Myositis patients prefer subcutaneous Ig at home to intravenous infusions in hospital
“I have regained control of my life”. This verbatim summarises the very consensual view of a group of seven adults with polymyositis or dermatomyositis on the home administration of polyvalent immunoglobulins (Ig) subcutaneously, using an infusion pump or syringe pump. Followed at the Pitié-Salpêtrière Hospital (Paris), these patients took part in a qualitative study, which … [Read more]
Hypokalemic periodic paralysis may present as progressive muscle weakness with or without episodes of periodic paralysis
A Danish team followed 37 people with hypokalaemic periodic paralysis due to CACNA1S mutations for three years (26 to 52 months): two were asymptomatic 21 had only episodes of periodic paralysis, two had isolated permanent muscle weakness, 12 had episodes of periodic weakness associated with a permanent muscle strength deficit. During follow-up : two remained … [Read more]
Oculopharyngodistal and oculopharyngeal myopathies do not have the same pattern of muscle involvement on imaging
Oculopharyngodistal myopathy (OPDM), which is very rare, differs from oculopharyngeal myopathy (OPMD) both genetically and clinically. Japanese researchers sought to identify these differences using magnetic resonance imaging (MRI): 54 patients with one of the three subtypes of OPDM and 57 patients with OPMD were included in a comparative study, OPDM patients had the same MRI … [Read more]
Three studies establish links between intestinal microbiota and myasthenia gravis
The role of intestinal flora is the subject of sustained attention in medical research, particularly in dysimmune diseases such as myasthenia gravis. Various publications point to the existence of dysbiosis in this disease: The results of the German Mybiom study show, in cases of myasthenia (n=42), a lower diversity of intestinal flora than in a … [Read more]
22nd meeting of the French network of “Emery-Dreifuss muscular dystrophies and other nuclear envelope pathologies” on November 10
The 22nd French network meeting on “Emery-Dreifuss muscular dystrophies and other pathologies of the nuclear envelope” was held on Friday, November 10, 2023, from 9:15 a.m. to 4:00 p.m., in a hybrid format: face-to-face in the auditorium of the Institut de Myologie and remote via videoconferencing. This one-day event will cover the various aspects of … [Read more]
M&M’s – Muscle Monday Seminar – 20 November – Johan Auwerx (Switzerland)
Ceramide de novo synthesis links muscle disorders with mitochondrial and protein homeostasis 20th November 2023, from 12 to 1pm Johan Auwerx, M.D., Ph.D. (Ecole Polytechnique Fédérale de Lausanne, Switzerland) > More information on the presentation and the speaker On prior registration for people outside the Institute of Myology: medecine-umrs974-myologie@sorbonne-universite.fr Organised by the Myology Centre for … [Read more]
