Abstract
Background: Spinal muscular atrophy type 1 (SMA1) is characterized by a severe hypotonia. Affected children never attains the ability to sit independently and can develop early acute respiratory insufficiency. Sleep breathing disorders are an additional cause of morbidity. Few studies about sleep patterns of SMA 1 patients have been published..
Aim: to evaluate the sleep architecture by Cyclic Alternating Pattern (CAP) in SMA1 patients comparing to a control group age-sex mathed.
Methods: 12 SMA1 patients (6 males, mean age 5.9 months) and 10 control subjects (5 males, mean age 4.8 months) underwent full polysomnography.
Results: the macrostructure parameters showed an higher sleep latency and apnoea/hypopnea index in SMA1 patients (p<0.003 and 0.046 respectively). Regarding microstructure, there is a significant increase in A1% (p<0.000), A2 mean duration (p<0.000) and of CAP sequences mean duration (p<0.014); furthermore a reduction of A3% (p<0.000) and A2 (p<0.05) and A3 indexes (p<0.02) were demonstrated.
Conclusion: our results demonstrated an abnormality of sleep microstructure in SMA 1 patients, with an increase of phase A1 and a reduction of phase A2 and A3 indexes. We postulate that SMA1 patients have a reduced arousability during NREM sleep, that could be interpreted as an additional proof of the central nervous system involvement in this disease.
- © 2014 ERS