Impaired mandibular function in spinal muscular atrophy type II: need for early recognition

J Child Neurol. 2011 Nov;26(11):1392-6. doi: 10.1177/0883073811407696. Epub 2011 May 18.

Abstract

The aim of the study is to assess mandibular function in young patients with spinal muscular atrophy type II. A total of 12 children and young adults with spinal muscular atrophy type II and 12 healthy matched controls participated. The mandibular function impairment was moderate to severe in 50% of patients. A limited mouth opening (≤30 mm) was observed in 75% of the patients. In patients with a severe reduction of the mandibular range of motion the temporomandibular joint mainly rotated during mouth opening instead of the usual combination of rotation and sliding. The severity of the limited active mouth opening correlated with the severity of the disease (motor function measure scores). This study shows that mandibular dysfunction is common among young patients with spinal muscular atrophy type II. Early recognition of mandibular dysfunction may help to prevent complications such as aspiration as a result of chewing problems.

MeSH terms

  • Adolescent
  • Case-Control Studies
  • Child
  • Child, Preschool
  • Female
  • Humans
  • Male
  • Mandible / pathology
  • Mandibular Diseases / diagnosis*
  • Mandibular Diseases / etiology*
  • Motor Activity / physiology
  • Motor Skills / physiology
  • Severity of Illness Index
  • Spinal Muscular Atrophies of Childhood / complications*
  • Surveys and Questionnaires
  • Young Adult