Feeding problems and malnutrition in spinal muscular atrophy type II

Neuromuscul Disord. 2008 May;18(5):389-93. doi: 10.1016/j.nmd.2008.02.008. Epub 2008 Apr 16.


The aim of the study was to conduct a survey using a dedicated questionnaire to assess feeding difficulties and weight gain in a population of 122 Spinal Muscular Atrophy (SMA) type II patients, aged between 1 and 47 years. All the answers were entered in a database and were analysed subdividing the cohort into age groups (1-5, 6-10, 11-14, 15-19, 20-29, and 30-50 years). Six out of our 122 patients (5%), all younger than 11 years, had weights more than 2SD above the median for age matched controls, whilst 45 (37%) had weights less than 2SD below the median. Chewing difficulties were reported in 34 of the 122 patients (28%) and limitation in the ability to open the mouth in 36 (30%) and both were increasingly more frequent with age. Swallowing difficulties were reported in 30 patients (25%). The results of our survey suggest that a number of patients with SMA type II have limited jaw opening, and chewing and swallowing difficulties. Our findings raise a few issues concerning standards of care that should be implemented in the monitoring and management of feeding difficulties and weight gain.

MeSH terms

  • Adolescent
  • Adult
  • Aged
  • Child
  • Child, Preschool
  • Cohort Studies
  • Deglutition Disorders / etiology*
  • Feeding and Eating Disorders / classification
  • Feeding and Eating Disorders / etiology*
  • Humans
  • Infant
  • Malnutrition / etiology*
  • Malnutrition / therapy
  • Middle Aged
  • Nutritional Status
  • Nutritional Support
  • Spinal Muscular Atrophies of Childhood / complications
  • Spinal Muscular Atrophies of Childhood / physiopathology*
  • Spinal Muscular Atrophies of Childhood / therapy
  • Surveys and Questionnaires
  • Weight Gain