Figure 1.

Quantitative assessment of the response of AChR deficiency patients to the addition of salbutamol or ephedrine to their medication. (A) Change in individual QMG severity scores (max = 39) over a 4-year follow-up period. In patients with AChR deficiency, already on optimized therapy with pyridostigmine ± 3,4-diaminopyridine (3,4-DAP), β2-adrenergic agonists (β2-AR, typically salbutamol) were introduced, often following clinical deterioration. (B) Change in percentage of the lower limit of normal (minimum required to achieve 0 in the QMG scale) for the individual quantitative components that contribute to the QMG severity score. FVC = forced vital capacity.