Accurate, noninvasive measures of body composition are needed for management of patients with spinal muscular atrophy. Fat mass index (fat mass/height2 in kg/m2) was measured in 16 subjects with spinal muscular atrophy using 5 bioelectrical impedance analysis equations and compared with a reference method, dual-energy x-ray absorptiometry. The machine default equation, validated by Cordain, was the primary analysis. Fat mass index calculated by impedance measures differed by between -2.5 kg/m2 and 1.7 kg/m2 from the reference mean (8.3 ± 5.0 kg/m2). The Cordain equation provided the smallest difference (-0.4 ± 2.0 kg/m2), with correlation coefficient of 0.92. The Cordain equation showed high sensitivity (85.7%) and specificity (100%) for prediction of ‘‘at risk for overweight’’ (fat mass index > 85th percentile for age and gender). Although insufficiently accurate for use as a research tool, bioelectrical impedance can have application as a well-tolerated, noninvasive, easily used screening tool for excess adiposity in patients with spinal muscular atrophy.
Emery AEPopulation frequencies of inherited neuromuscular diseases-a world survey . Neuromuscul Disord. 1991;1:19-29.
2.
Pearn J.Incidence, prevalence, and gene frequency studies of chronic childhood spinal muscular atrophy. J Med Genet. 1978 ; 15:409-413.
3.
Pearn JHThe gene frequency of acute Werdnig-Hoffmann disease (SMA type 1). A total population survey in North-East England. J Med Genet . 1973;10:260-265.
4.
Werdnig G.Zwei frühinfantile hereditäre Fälle von progressiver Muskelatrophie unter dem Bilde der Dystrophie, aber auf neurotischer Grundlage . Arch Psychiatrie Nervenkrankheiten. 1891 ;22: 437-480.
5.
Kugelberg E. , Welander L.Heredofamilial juvenile muscular atrophy simulating muscular dystrophy. AMA Arch Neurol Psychiatry . 1956;75:500-509.
6.
Oskoui M., Levy G., Garland CJ, et al. The changing natural history of spinal muscular atrophy type 1. Neurology. 2007;69: 1931-1936.
7.
Wang CH, Finkel RS, Bertini ES, et al. Consensus statement for standard of care in spinal muscular atrophy. J Child Neurol. 2007; 22:1027-1049.
8.
Liusuwan A. , Abresch R., McDonald C.Altered body composition affects resting energy expenditure and interpretation of body mass index in children with spinal cord injury. J Spinal Cord Med. 2004 ;27:S24-S28.
9.
McDonald C. , Abresch-Meyer A., Nelson M., Widman L.Body mass index and body composition measures by dual x-ray absorptiometry in patients aged 10 to 21 years with spinal cord injury. J Spinal Cord Med. 2007;30:S97-S104.
10.
Willig T., Carlier L., Legrand M., et al. Nutritional assessment in Duchenne muscular dystrophy. Dev Med Child Neurol. 1993; 35:1074-1082.
11.
Dopler-Nelson M., Widman L., Abresch R., et al. Metabolic syndrome in adolescents with spinal cord dysfunction. J Spinal Cord Med. 2007;30:S130-S142.
12.
Abresch R., McDonald D., Widman L., et al. Impact of spinal cord dysfunction and obesity on the health-related quality of life of children and adolescents. J Spinal Cord Med. 2007;30:S112-S118.
13.
Zanardi M., Tagliabue A., Orcesi S., et al. Body composition and energy expenditure in Duchenne muscular dystrophy. Eur J Clin Nutr. 2003;57:273-278.
14.
Pichiecchio A., Uggetti C., Egitto M., et al. Quantitative MR evaluation of body composition in patients with Duchenne muscular dystrophy . Eur Radiol. 2002;12:2704-2709.
15.
Messina S., Pane M., De Rose P., et al. Feeding problems and malnutrition in spinal muscular atrophy type II. Neuromuscul Disord. 2008;18:389-393.
16.
Sproule DM, Montes J., Montgomery M., et al. Increased fat mass and high incidence of overweight despite low body mass index in patients with spinal muscular atrophy. Neuromuscul Disord. 2009;19:391-396.
17.
Leroy-Willig A., Willig T., Henry-Feugeas M., et al. Body composition determined with MR in patients with Duchenne muscular dystrophy, spinal muscular atrophy, and normal subjects. Magn Reson Imaging. 1997;15:737-744.
18.
Chan G.Performance of dual-energy x-ray absorptiometry in evaluating bone, lean body mass, and fat in pediatric subjects. J Bone Miner Res. 1992;7:369-374.
19.
Margulies L. , Horlick M., Thornton J.Reproducibility of whole body bone and body composition measures by dual energy X-ray absorptiometry using GE Lunar Prodigy . J Clin Densitometry. 2005;8:298-304.
20.
Schmelzle H. , Fusch C.Body fat in neonates and young infants: validation of skinfold thickness versus dual-energy X-ray absorptiometry . Am J Clin Nutr. 2002;76:1096-1100.
21.
Freedman D. , Wang J., Maynard L., et al. Relation of BMI to fat and fat-free mass among children and adolescents. Int J Obes ( Lond)2005;29:1-8.
22.
Freedman D. , Wang J., Ogden C., et al. The prediction of body fatness by BMI and skinfold thicknesses among children and adolescents . Ann Hum Biol. 2007;34:183-194.
23.
Mei Z., Grummer-Strawn LM, Wang J., et al. Do skinfold measurements provide additional information to body mass index in the assessment of body fatness among children and adolescents ? Pediatrics. 2007;119:e1306-e1313.
24.
Hildreth HG , Johnson RK, Goran MI, Contompasis SHBody composition in adults with cerebral palsy by dual energy X-ray absorptiometry, bioelectrical impedance analysis, and skinfold anthropometry compared with the 18O isotope-dilution technique. Am J Clin Nutr. 1997;66:1436-1442.
25.
McDonald CM , Carter GT, Abresch RT, et al. Body composition and water compartment measurements in boys with Duchenne muscular dystrophy . Am J Phys Med Rehabil. 2005;84:483-491.
26.
Fuller NJ, Fewtrell MS, Dewit O., et al. Segmental bioelectrical impedance analysis in children aged 8-12 y: 1. The assessment of whole-body composition. Int J Obes Relat Metab Disord. 2002;26:684-691.
27.
Cleary J., Daniells S., Okely AD, et al. Predictive validity of four bioelectrical impedance equations in determining percent fat mass in overweight and obese children. J Am Diet Assoc. 2008;108:136-139.
28.
Mok E., Béghin L., Gachon P., et al. Estimating body composition in children with Duchenne muscular dystrophy: comparison of bioelectrical impedance analysis and skinfold-thickness measurement. Am J Clin Nutr. 2006;83:65-69.
29.
Buchholz AC , McGillivray CF, Pencharz PBThe use of bioelectric impedance analysis to measure fluid compartments in subjects with chronic paraplegia. Arch Phys Med Rehabil. 2003;84:854-861.
30.
Veugelers R. , Penning C., van Gulik ME, et al. Feasibility of bioelectrical impedance analysis in children with a severe generalized cerebral palsy. Nutrition. 2006;22:16-22.
31.
Esper GJ, Shiffman CA, Aaron R., et al. Assessing neuromuscular disease with multifrequency electrical impedance myography. Muscle Nerve. 2006;34:595-602.
32.
Desport JC, Preux PM, Bouteloup-Demange C., et al. Validation of bioelectrical impedance analysis in patients with amyotrophic lateral sclerosis. Am J Clin Nutr. 2003;77:1179-1185.
33.
Cordain L., Whicker RE, Johnson JEBody composition determination in children using bioelectrical impedance . Growth Dev Aging. 1988;52:37-40.
34.
Deurenberg P., Van Der Kooy K., Leenan R., et al. Sex and age specific prediction formulas for estimating body composition from bioelectrical impedance: a cross validation study. Obes Relat Metab Disord . 1991;15:17-25.
35.
Deurenberg P., Kusters CSL, Smit H.Assessment of body composition by bioelectrical impedance in children and young adults is strongly age-dependent. Eur J Clin Nutr. 1989;44:261-268.
36.
Schaefer F. , Georgi A., Zieger A., Scharer K.Usefulness of bioelectric impedance and skinfold measurements in predicting fat-free mass derived from total body potassium in children. Pediatr Res . 1994;35: 617-624.
37.
Houtkooper LB, Going SB, Lohman TG, et al. Bioelectrical impedance estimation of fat free body mass in children and youth: a cross validation study. J Appl Physiol. 1992;72: 366-373.
38.
Mazess R., Barden H., Bisek J., Hanson J.Dual-energy x-ray absorptiometry for total-body and regional bone-mineral and soft-tissue composition. Am J Clin Nutr. 1990;51:1106-1112.
39.
Bland JM, Altman DGApplying the right statistics: analyses of measurement studies. Ultrasound Obstet Gynecol. 2003;22: 85-93.
40.
Bland JM, Altman DGStatistical methods for assessing agreement between two methods of clinical measurement. Lancet. 1986;1:307-310.
41.
Kuczmarski R., Ogden C., Guo S.2000CDC growth charts for the United States: methods and development. Vital Health Stat11. 2002:1-190.
42.
Schutz Y., Kyle UU, Pichard C.Fat-free mass index and fat mass index percentiles in Caucasians aged 18-98 y. Int J Obes Relat Metab Disord. 2002 ;26:953-960.
43.
Goran MIMeasurement issues related to studies of childhood obesity: assessment of body composition, body fat distribution, physical activity, and food intake . Pediatrics. 1998;101:505-518.
