FDA approves first treatment for Lambert-Eaton myasthenic syndrome, a rare autoimmune disorder
FDA approved Firdapse (amifampridine) tablets for the treatment of Lambert-Eaton myasthenic syndrome (LEMS) in adults. LEMS is a rare autoimmune disorder. This is the first FDA approval of a treatment for LEMS.
National Institutes of Health has awarded a research team from Yale and other universities $7.8 million to establish a rare disease network called MGNet.
The original version of this article unfortunately contained a mistake. Fifth sentence of the fourth paragraph in the section “Non-nAChR autoantibody targets in MG” should read as
Hospital admission trends in Myasthenia Gravis are largely unknown, so they were here investigated in Finland between 2004 and 2014 using national mandatory registry data. There were 2989 hospital admissions (59.7% for women) for 861 individuals (median 2 admissions/individual) The annual number of admissions (p = .56), the age of admitted patients (p = .24) or length of stay (p = .20) showed no change during the study period. The proportion of infections as the primary diagnosis increased from 4.5% to 10.4% (p = .0056).
Objectives: Thymic epithelial tumors (TETs) are rare malignant tumors that exhibit heterogeneous histology and clinical behavior. As immune check point inhibitors, drugs targeting anti-programmed cell death protein 1 (PD-1) and programmed death-ligand 1 (PD-L1) have shown remarkable results against many cancers; thus, the importance of PD-1/PD-L1 immunohistochemistry as a predictive or prognostic biomarker has grown. However, limited data on PD-L1 and PD-1 expression in TETs have been reported; moreover, these results have been variable. Here, we examined the expression of PD-1/PD-L1 proteins in TETs and analyzed the clini...
Myasthenia Gravis (MG) – an autoimmune neuromuscular disease – is known by the production of autoantibodies against components of the neuromuscular junction mainly to the acetylcholine receptor, which cause the destruction and compromises the synaptic transmission. This disease is characterized by fluctuating and fati gable muscle weakness, becoming more intensive with activity, but with an improvement under resting. There are many therapeutic strategies used to alleviate MG symptoms, either by improving the transmission of the nerve impulse or by ameliorating autoimmune reactions with e.g.
AbstractAryl hydrocarbon receptor (AhR), a type of transcriptional factor, is widely expressed in immune cells. The activation of AhR signaling pathway depends on its ligands, which exist in environment and can also be produced by metabolism. Normal expressions of AhR and AhR-mediated signaling may be essential for immune responses, and effects of AhR signaling on the development and function of innate and adaptive immune cells have also been revealed in previous studies. Recent studies also indicate that aberrant AhR signaling may be related to autoimmune diseases, including rheumatoid arthritis (RA), systemic lupus eryth...
Condition: Myasthenia Gravis Intervention: Drug: Rozanolixizumab Sponsor: UCB Biopharma S.P.R.L. Not yet recruiting
Consider slow-channel congenital myasthenic syndrome in the differential diagnosis of vocal cord paralysis.
This case report highlights the importance of cholinesterase inhibitors management and neuromuscular block monitoring in the perioperative period for patients with myasthenia gravis.BMC Anesthesiology
Myasthenia gravis (MG) is a neuromuscular autoimmune disease that affects the skeletal muscle through the production of autoantibodies against the nicotinic acetylcholine receptor at the neuromuscular junction, thus causing defective neuromuscular transmission in skeletal muscles . MG occurs in 1: 7500 individuals, affecting women during the second to third decade and men in their fifth and sixth decade of life . Typically, patients experience muscle weakness and fatigue, as well as fluctuating and fatigable extraocular (e.g.