Increased fat mass and high incidence of overweight despite low body mass index in patients with spinal muscular atrophy

Sproule, Douglas M.; Montes, Jacqueline; Montgomery, Megan; Battista, Vanessa; Koenigsberger, Dorcas; Shen, Wei; Punyanitya, Mark; De Vivo, Darryl C.; Kaufmann, Petra

doi:10.1016/j.nmd.2009.03.009

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Volume 19, Issue 6 , Pages 391-396, June 2009

Increased fat mass and high incidence of overweight despite low body mass index in patients with spinal muscular atrophy

Douglas M. Sproule
- Division of Pediatric Neurosciences, Department of Neurology, SMA Clinical Research Center, Columbia University Medical Center, Harkness Pavilion, HP-514, 180 Fort Washington Avenue, New York, NY 10032-3791, USA
- Corresponding author. Tel.: +1 212 342 6858; fax: +1 212 305 1253.
Jacqueline Montes
- Division of Pediatric Neurosciences, Department of Neurology, SMA Clinical Research Center, Columbia University Medical Center, Harkness Pavilion, HP-514, 180 Fort Washington Avenue, New York, NY 10032-3791, USA
Megan Montgomery
- Division of Pediatric Neurosciences, Department of Neurology, SMA Clinical Research Center, Columbia University Medical Center, Harkness Pavilion, HP-514, 180 Fort Washington Avenue, New York, NY 10032-3791, USA
Vanessa Battista
- Division of Pediatric Neurosciences, Department of Neurology, SMA Clinical Research Center, Columbia University Medical Center, Harkness Pavilion, HP-514, 180 Fort Washington Avenue, New York, NY 10032-3791, USA
Dorcas Koenigsberger
- Division of Pediatric Neurosciences, Department of Neurology, SMA Clinical Research Center, Columbia University Medical Center, Harkness Pavilion, HP-514, 180 Fort Washington Avenue, New York, NY 10032-3791, USA
Wei Shen
- The Obesity Research Center, St Luke’s–Roosevelt Hospital, New York, NY, USA
- Institute of Human Nutrition, Columbia University College of Physicians and Surgeons, New York, NY, USA
Mark Punyanitya
- The Obesity Research Center, St Luke’s–Roosevelt Hospital, New York, NY, USA
Darryl C. De Vivo
- Division of Pediatric Neurosciences, Department of Neurology, SMA Clinical Research Center, Columbia University Medical Center, Harkness Pavilion, HP-514, 180 Fort Washington Avenue, New York, NY 10032-3791, USA
Petra Kaufmann
- Division of Pediatric Neurosciences, Department of Neurology, SMA Clinical Research Center, Columbia University Medical Center, Harkness Pavilion, HP-514, 180 Fort Washington Avenue, New York, NY 10032-3791, USA

Received 29 November 2008; received in revised form 9 March 2009; accepted 27 March 2009.

Abstract

Body composition is sparsely described in spinal muscular atrophy (SMA). Body (BMI, mass/height in m²), fat-free (FFMI, lean mass/height in m²) and fat (FMI, fat mass/height in m²) mass indexes were estimated in 25 children (aged 5–18) with SMA (2 type I, 13 type II, 10 type III) using dual-energy radiograph absorptiometry and anthropometric data referenced to gender and age-matched healthy children (NHANES III, New York Pediatric Rosetta Body Project). BMI was ⩾50th percentile in 11 (44%) and ⩾85th in 5 (20%). FFMI was reduced (p<0.005) and FMI was increased (p<0.005) in the overall study cohort. FMI was ⩾50th, ⩾85th and 95th percentiles in 19 (76%), 10 (40%) and 5 (20%) subjects, respectively. Using a receiver operator characteristic curve, BMI above 75th, 50th and 3rd percentiles maximized sensitivity and specificity for FMI ⩾95th, ⩾85th and ⩾50th percentiles, respectively. Children with SMA have reduced lean and increased fat mass compared to healthy children. Obesity is a potentially important modifiable source of morbidity in SMA.