Samenvatting

Introduction: Neuromuscular diseases (NMD) are a heterogeneous group of acquired or inherited diseases affecting the peripheral nervous system and muscle mass. The consequences of NMD are loss of muscle mass, function, and posture. Loss of muscle mass causes weakness, a decrease in mobility and cardiac and respiratory function. These consequences influence the quality of life in a negative way. An adequate dietary protein intake stimulates the protein synthesis of muscle mass or at least, inhibit the muscle tissue from breaking down during disease. There is a gap in science, concerning the dietary protein intake among patients with NMD. The main goal of this study is to gather as much information as possible about the dietary protein intake, in order to point out deviant values. The final product contains recommendations about dietary protein intake for the established high risk groups within this study. Methodology: This study studied 146 patients with NMD. The included NMD are Amyotrophic Lateral Sclerosis, Progressive Spinal Muscular Atrophy, Hereditary Spastic Paraparesis, Primary Lateral Sclerosis, Arthrogryposis Multiplex Congenita, Ataxia of Friedreich, Chronic Idiopathic Axonal Polyneuropathy, Congenital and Metabolic Muscular Dystrophies, Small-Fiber Peripheral Neuropathy, hereditary muscle dystrophies and distal myopathies (Duchenne), Hereditary Polyneuropathies, Facioscapulohumeral Muscular Dystrophy, Guillain Barré Syndrome/Chronic Inflammatory Demyelinizing Polyneuropathy, Myotonic Dystrophy, Myasthenia, Myositis, Post-Polio Syndrome and Spinal Muscular Atrophy. The NMD were categorized into three groups (1: slowly progressive , 2: quickly progressive and 3: regressive). The dietary protein intake, source and timing were studied with the following variables; age, sex, weight, height, BMI and mobility. Results: The study population included 57 men and 89 women, with an age range of 2 to 84 years old. The current dietary protein intake of patients with NMD is 79.4 +/- 31.3 grams per day . This equals 16.2 energy percent per day and 1.19 +/- 0.52 grams per kilogram bodyweight per day, which is above the recommended daily protein intake for healthy adults in The Netherlands (0.8 grams per kilogram bodyweight per day). The mean dietary protein intake of category 1 is 76.3 +/- 30.3 grams per day, category 2 is 87.3 +/- 35.7 grams per day and category 3 is 85.2 +/- 24.9 grams per day. The mean dietary protein intake during breakfast is 13 grams, during lunch 19 grams, during dinner 32 grams and during snacks 6 grams. The primary source of protein is animal-based (65%). The majority of patients is ambulatory (74%) and has the highest mean dietary protein intake (82.9 +/- 33.4 grams). Conclusion: Although, the mean dietary protein intake in patients with NMD is well above the recommended daily allowance of 0.8 grams per kilogram bodyweight per day, still a significant 5 proportion (59.5% of the adults) does not meet the higher recommended intake of 1.2 grams per kilogram bodyweight per day. Dietary protein intake may be improved by increasing protein intake during breakfast, lunch and snacks. This is recommended to reach an equal and higher distribution of high- quality dietary protein intake during the day. Children among this study do reach the general dietary protein recommendation in energy percent. This implies there is no need to improve their dietary protein intake.