Efficacy analysis of polysomnography and improvement after nocturnal non-invasive ventilation in treating children of spinal muscular atrophy with moderate-severer sleep disordered breathing

Abstract

Objective To learn about polysomnography (PSG) of the patients with spinal muscular atrophy (SMA), and the improvement of the moderate-severe sleep disordered breathing(SDB) patients after non-invasive ventilation (NIV) treatment. Methods The PSG examination of 11 SMA patients in Children′s Hospital Affiliated to Capital Institute of Pediatrics was performed.The PSG results and the levels of breathing disorder of these patients were collected.These patients were divided into 2 groups as follows.The mild group: apnea hypopnea index(AHI) was between 5 to 10 times/h, or average pulse oxygen saturation(SpO2)>95%; the moderate-severe group: AHI>10 times/h, and average SpO2≤95%.The moderate-severe levels of the patients were treated with NIV.Then the improvement of PSG after the treatment was evaluated. Results Within the 2 cases of type 1 SMA, 1 was moderate-severe, the other 1 was mild.There were 2 cases moderate-severer and 6 cases mild SDB cases of type 2 SMA.The only 1 case of type 3 was categorized as mild disorder.The average hypopnea index(6.3 times/h) was higher than apnea index(4.9 times/h). The indicators to distinguish the moderate-severe and mild groups were as follows: AHI, average SpO2, hypopnea index, and arousal index associated with respiratory case, and there were statistically significant differences(all P<0.05). Significant improvement in PSG of the 3 NIV-treated patients was observed and it remained in normal range.The following indicators also remarkably improved after NIV treatment were hypopnea index [(1.5±0.1) times/h vs.(11.5±4.6) times/h, t=3.741, P<0.05], AHI [(2.4±1.1) times/h vs.(17.4±8.1) times/h, t=3.196, P<0.05], and average SpO2 [(96.7±0.6)% vs.(94.3±1.2)%, t=-3.130, P<0.05]. Conclusions There are different levels of SDB within SMA patients.The major characteristic of SMA patients is hypopnea.There are more moderate-severer SDB in type 1 than those in type 2.The average SpO2, arousal index associated with respiratory incidents, AHI, and hypopnea index can reflect the levels of SDB of SMA patients.Besides, the results indicate NIV treatment can significantly improve SDB of SMA patients. Key words: Spinal muscular atrophy; Sleep disordered breathing; Polysomnography; Non-invasive ventilation