Myology research highlights
RSS feedMyositis 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]
When artificial intelligence helps analyse walking disorders
Japanese researchers have developed an algorithm based on artificial intelligence (AI) to analyse changes in gait in several pathologies: 114 patients, including 45 with neuromuscular pathologies, took part in the study, the application (TDPT-GT) was developed on the iPhone using data supplied by the camera, based on a codified and standardised movement protocol (circular walk, … [Read more]
Vamorolone (Agamree®) authorised in the USA for DMD from the age of 2; a decision expected in Europe soon
Vamorolone is a “dissociative” synthetic steroid developed by Santhera and ReveraGen for Duchenne muscular dystrophy, as an anti-inflammatory treatment alternative to conventional corticosteroids. The FDA has granted marketing authorisation for Agamree® (vamorolone) in DMD patients from the age of 2 (drinkable suspension, 40 mg/ml). Four studies of vamorolone in DMD patients aged between 4 and … [Read more]
Metformin may have a protective effect on statin muscle toxicity
This is the finding of a one-year Korean study which compared 4,092 patients on statins + metformin with 8,161 patients on statins alone. Analysis of data from the Korean national health insurance system revealed an incidence of statin-related muscular symptoms of 201.7 per 1000 people on statins alone and 184.3 per 1000 on statins and … [Read more]
US recommendations on the use of immunoglobulins in MNM
Immunoglobulins are still widely prescribed for certain neuromuscular diseases of autoimmune origin, despite the absence of irrefutable scientific evidence. The scarcity of this type of pharmaceutical product has prompted American doctors to reflect on their proper use in neuromuscular diseases: the initial consensus drawn up in 2009 by the American Association of Myology (AANEM) was … [Read more]
French study highlights neurotoxicity of immune checkpoint inhibitors
Clinicians and researchers belonging to two oncology networks, including one devoted to paraneoplastic syndromes, have studied neurological complications arising during the use of a new class of drugs, immune checkpoint inhibitors: 147 patients were included in the study, reflecting the significant frequency of the phenomenon; complications of the peripheral nervous system were more frequent than … [Read more]
A very high risk of cardiac complications in Emery-Dreifuss muscular dystrophy
An international consortium of researchers and clinicians investigated disorders of cardiac function and/or cardiac rhythm during the course of Emery-Dreifuss muscular dystrophy linked to the gene encoding emerin (EMD1) of X-linked recessive transmission : longitudinal clinical and electrophysiological data from 38 men with EMD1 and 21 symptomatic female EMD1 transmitters were analysed in the study, … [Read more]
