Samenvatting

Introduction: Neuromuscular disease is a progressive muscle disease, whereby patients suffer from a decrease in muscle mass. Although muscle diseases can hardly be cured, treatment is possible with adequate protein intake. However, there is no general nutritional guideline available for patients with NMD. Therefore, we first had to evaluate the daily dietary protein intake, distribution during the day and consumed protein sources of patients with NMD. Method: Dietary intake of 105 patients with an NMD - 35 men and 70 women with a minimum age of 18 years - was evaluated using 3-day nutritional diary. This observational study included the 15 most common types of NMD in the Netherlands. The study population was divided into three groups; ALS, PSMA, HSP, and PLS group (19 patients), MD group (65 patients), and remainder NM diseases (21 patients). Results: Daily dietary protein intake averaged 71.5 +/- 17.9 g/day, and 1.07 g/kg-bw/day in the total study population. The average recommendation of the total study population was 0.84 g/kg-bw/day. The ALS, PSMA, HSP, and PLS group had an average of 79.5 g/day, MD group 71.2 g/day, and remainders 65.2 g/day and accounted for 17.3%, 16.1%, and 15.7% of their total energy intake. Dietary protein intake averaged during breakfast 12.3 g, during lunch 18.5 g, during dinner 30.7 g, and 11 g divided over snack times. The majority of consumed protein is from animal sources (63.5%). Dominated protein sources during the day are meat, cheese, and bread. Conclusion: The average daily dietary protein intake of patients with an NMD was 71.5 g/day and represents 1.07 g/kg-bw/day. The intake is above the current recommendation of patients with NMD, but the requirement could be higher for an optimal protein muscle synthesis. Therefore, expanding the protein intake - with high-quality protein - during breakfast could improve the daily protein intake.