The Neuromuscular diseases Unit of Hospital Sant Pau publishes an article in the Brain Journal.
The doctors Xavier Suárez-Calvet, Eduard Gallardo and Isabel Illa of the Sant Pau Neuromuscular Unit, signed the paper entitled “JAK inhibitor improves type I interferon induced damage: proof of concept in dermatomyositis” published in the prestigious Brain Journal. This international collaboration study establishes the bases for the treatment with Ruxolitinib of patients with dermatomyositis resistant to conventional therapies. Ruxolitinib is an inhibitor of JAK1 / 2 which is a key molecule in the type I interferon signaling.
The article entitled “Muscle MRI in patients with dysferlinopathy: pattern recognition and implications for clinical trials” has just been published in the journal of Neurology Neurosurgery and Psychiatry. This study analyses whole-body muscle resonances of 182 patients participating in the international study of the natural history of muscular dystrophy 2B due to dysferlin deficiency (LGMD-2B). This study, known as COS study, aims to make a detailed analysis of the evolution of patients with this genetic disease. The objective is to identify valuable resonance patterns that allow us to develop clinical trials with drugs. In this publication, Dr. Díaz-Manera has coordinated the analysis of MRIs at the baseline visit. This study has allowed us to know the different fat muscle infiltration patterns in patients with LGMD-2B and to correlate changes in magnetic resonance with muscle function tests that are usually used in clinical trials, such as the 6MWT or the Brooke score.