Nutritional assessment of patients with neuromuscular diseases.

OBJECTIVE: To study the nutritional status of patients with Duchenne muscular dystrophy and amyotrophic lateral sclerosis. DESIGN: A total of 34 Duchenne muscular dystrophy and seven amyotrophic lateral sclerosis patients were studied. Body mass index, patient's body weight for zero muscle mass as a percentage of the theoretical weight for zero muscle mass, and creatinine-height index were calculated. RESULTS: Substantial differences were found between body mass index and percentage of expected weight for zero muscle mass. No amyotrophic lateral sclerosis patients were classified as overweight by body mass index, whereas five were overweight by the percentage of expected weight for zero muscle mass method. Five Duchenne muscular dystrophy patients were classified as overweight by body mass index, and 30 were overweight by the percentage of expected weight for zero muscle mass. According to the creatinine-height index, no patient with amyotrophic lateral sclerosis or Duchenne muscular dystrophy showed normal body muscle mass. No correlation was found between creatinine-height index, percentage of expected weight for zero muscle mass, and body mass index. CONCLUSIONS: The body mass index should be used with caution for the evaluation of the nutritional status of patients with amyotrophic lateral sclerosis and Duchenne muscular dystrophy. Indices that incorporate the assessment of the compartmental distribution of muscle and fat are more sensitive.

Peak flow and peak cough flow in the evaluation of expiratory muscle weakness and bulbar impairment in patients with neuromuscular disease.

OBJECTIVE: To study the expiratory muscle force and the ability to cough estimated by the peak expiratory flow and peak cough flow in patients with Duchenne muscular dystrophy and amyotrophic lateral sclerosis. DESIGN: A total of 27 patients with amyotrophic lateral sclerosis and 52 patients with Duchenne muscular dystrophy were studied. From the group of 144 normal subjects of this laboratory, we selected 38 for comparison. RESULTS: The maximal inspiratory pressure in patients with Duchenne muscular dystrophy and amyotrophic lateral sclerosis was 64.5 +/- 24.7% and 37.8 +/- 21.8%, respectively, and maximal expiratory pressure was 64.2 +/- 32.5% and 37.7 +/- 21.6%, respectively. Patient groups showed a significant lower peak expiratory flow than normal subjects. Higher peak cough flow than peak expiratory flow was found in all groups. The peak cough flow-peak expiratory flow difference was 46 +/- 18% in normal subjects, 43 +/- 23% in patients with Duchenne muscular dystrophy, and 11 +/- 17% in patients with amyotrophic lateral sclerosis. The peak expiratory flow and peak cough flow were not different in bulbar onset amyotrophic lateral sclerosis. In patient groups, the dynamic and static behavior correlated positively. CONCLUSIONS: These results suggest that peak cough flow-peak expiratory flow is useful to monitor expiratory muscle weakness and bulbar involvement and to assess its evolution in these patients.