Sleep-disordered breathing in spinal muscular atrophy types 1 and 2

Maria Beatrice Chiarini Testa, Martino Pavone, Enrico Bertini, Albino Petrone, Marco Pagani, Renato Cutrera

Research output: Contribution to journalArticlepeer-review


Objective: Our aim was to assess the respiratory pattern during sleep in patients affected by spinal muscular atrophy types 1 and 2 and to compare their apnea-hypopnea indices with those of controls. Design: All consecutively referred patients underwent polysomnography. Sleep stages were defined as either wake, quiet sleep (QS), or active sleep (AS). As measures of thoracoabdominal coordination, we measured: phase angle during QS and AS (Ph Angle QS and AS), phase relation during inspiration and expiration during QS and AS: (Ph RIB QS, Ph RIB AS, Ph REB QS; Ph REB AS) and the apnea-hypopnea index. Results: The 14 consecutively referred infants and small children (age, 11.7 ± 11.4 mos) showed a higher apnea-hypopnea index (P <0.001), Ph Angle QS (P <0.001 ), Ph Angle AS (P <0.001 ), Ph RIB QS (P <0.001), Ph RIB AS (P <0.001), Ph REB QS (P <0.001), and Ph REB AS (P <0.001) compared with 28 healthy controls (age, 10.1 ± 8.9 mos). Conclusions: Patients affected by types 1 and 2 spinal muscular atrophy had significantly higher apnea-hypopnea indices than controls. Thoracoabdominal asynchrony was present during the inspiratory and expiratory phases in both quiet and active sleep. Measures of thoracoabdominal coordination may be useful for the evaluation and monitoring of therapeutic interventions for these patients.

Original languageEnglish
Pages (from-to)666-670
Number of pages5
JournalAmerican Journal of Physical Medicine and Rehabilitation
Issue number9
Publication statusPublished - Sep 2005


  • Polysomnography
  • Respiratory Inductive Plethysmography
  • Sleep-Disordered Breathing
  • Spinal Muscular Atrophy
  • Thoracoabdominal Coordination

ASJC Scopus subject areas

  • Rehabilitation
  • Health Professions(all)
  • Orthopedics and Sports Medicine
  • Physical Therapy, Sports Therapy and Rehabilitation


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