Kaplan Meier survival estimates of 268 untreated adults with Pompe disease from study entry until end of study, start of ERT or death by RHS score. (RHS score was divided into tertiles for comparison. RHS1 = score <23, RHS2 = score 23-30, RHS3 = score >30). Twenty-three patients died during follow-up. P-value denotes result from log-rank test for trend.