The distribution of muscle weakness in MG is highly heterogeneous and frequently shifts in patients. In 5% of patients MG was restricted to ocular weakness, whereas 7% of patients never had any... Show moreThe distribution of muscle weakness in MG is highly heterogeneous and frequently shifts in patients. In 5% of patients MG was restricted to ocular weakness, whereas 7% of patients never had any form of ocular weakness throughout their disease course. In 83% of MG patients at least one other extra-ocular muscle was involved at the second visit.Patients with late-onset MG and the presence of additional autoimmune diseases had more exacerbations (OR = 47) and emergency treatments (OR = 26) than early-onset MG patients without other autoimmune diseases.MG-ADL (patient questionnaire on ADL) was found to be relatively insensitive to changes in generalized weakness. This led to the absence of a significant treatment response on MG-ADL in the presence of a significant response on QMG (an objective measure of muscle weakness) in the eculizumab study. A new ADL outcome measure, MGII, was shown to have a higher sensitivity for generalized weakness than MG-ADL.RoVEMP, a novel neurophysiological test, was shown to be a specific measure for myasthenic extra-ocular muscle weakness. In addition, we found a significant correlation between magnitude of decrement and the time since the last intake of pyridostigmine, supporting that RoVEMP decrement reflects reversible neuromuscular transmission failure. Show less