[Polygraphic findings in bird-like face syndrome with favorable spontaneous evolution]

The syndrome characterized by acquired micrognathia, hypersomnia and periodic apneas during sleep is a rare consequence of mandibular underdevelopment. The pathogenesis is ascribed to incomplete obstruction of the upper airways associated with a hypoexcitability of the respiratory center. Tracheostomy, with the placement of a permanent tracheal cannula, has proved to be the only treatment producing clinical remission of the syndrome. Polygraphic findings were described in a case spontaneously recovered of hypersomnia and periodic apneas with acquired micrognathia due to a bilateral mastoiditis occurring in early childhood. In this patient three polygraphic recordings were carried out during diurnal and nocturnal sleep; another night sleep was recorded after a spontaneous improvement. In the early three recordings there is a prevalence of light sleep over slow-waves sleep and REM sleep is reduced in nocturnal sleep. There is a lot of periodic apneas during sleep stages. During apneas we observe an increase of heart frequency in NonREM sleep and a decrease in REM sleep. In the recording after clinical recovery we observe an improvement of sleep parameters and a disappearing of apneas in NonREM sleep. A temporary hyposensibility of respiratory centers is considered to be a possible interpretation of clinical and polygraphic improvement.