We performed sequential studies of two methods used to estimate muscle mass in 34 patients with progressive neuromuscular disease for periods of up to 52 months. Creatinine excretion and total body potassium were low at the outset in virtually all patients. Creatinine excretion continued to decline in most patients, but total body potassium did not decline significantly. Creatinine excretion may measure declining muscle mass more accurately than total body potassium, but both measurements can estimate muscle mass for metabolic studies or therapeutic trials.