Neurocognitive Disorders and Sleep in Children with Primary Ciliary Dyskinesia

Background: Primary ciliary dyskinesia (PCD) patients have higher incidence of sleep disordered breathing which lead neurocognitive impairments such as attention-deficit/hyperactivity disorder (ADHD). It may effect academic performance of children and may cause impairment in emotional relationships. This study aim to evaluate hyperactivity and attention deficiency in PCD patients and investigate the relationship between sleep and hyperactivity and attention deficiency in PCD patients.

Method: Fifteen PCD patients aged 8-18 years and 31 age-matched healthy controls were compared. Ear, nose, and throat examination and home sleep testing were performed in PCD patients. Pediatric sleep questionnaire, Conners' Parents and Teacher scale and Stroop test were applied in both groups in order to investigate the relation between sleep disordered breathing and ADHD in PCD children.

Results: PCD patients had chronic rhinosinusitis (100%), tonsillar hypertrophy (80%) and adenoidal hypertrophy (60%). FEF25-75 was low in pulmonary function test. Sixty percent of the PCD patients had mild obstructive sleep apnea syndrome in home sleep testing. Mean AHI was 1.54 ± 0.27. Compared with the controls PCD patients had higher PSQ scores. Hyperactivity scores on Conners' Parents scale and inattention findings in Stroop test were higher in PCD patients than the healthy controls (P < 0.05).

Conclusion: Most of PCD children had mild obstructive sleep apnea syndrome. Hyperactivity and inattention findings were higher in PCD patients. Sleep disordered breathing assessment should be a routine part of PCD patients management and these patients should be carefully monitored in terms of hyperactivity and inattention.