Ka. Waters et al., TREATMENT OF OBSTRUCTIVE SLEEP-APNEA IN ACHONDROPLASIA - EVALUATION OF SLEEP, BREATHING, AND SOMATOSENSORY-EVOKED POTENTIALS, American journal of medical genetics, 59(4), 1995, pp. 460-466
The occurrence of obstructive sleep apnea (OSA) in achondroplasia has
been linked to brain stem compression, Overnight sleep studies (11 sub
jects) and somatosensory-evoked potentials (SEP's, 10 subjects) were r
ecorded before and after conventional treatment of OSA in achondroplas
ia, The two groups were derived from 30 subjects who underwent diagnos
tic sleep studies and SEPs, including 15 females and 15 males with a m
edian age 6.6 of years (range 1.0.47.6) at the time of the first study
, In 30 initial studies there was no correlation between severity of O
SA and abnormalities on SEP evaluation, Treatment of 17 subjects inclu
ded adenotonsillectomy (n = 3), weight loss (n = 1), and nasal-mask co
ntinuous positive airway pressure (CPAP) (n = 13), Sleep studies in 11
subjects after a delay of 8.8 +/- 2.8 months showed a reduction in re
spiratory disturbance index (RDI) from 38.4 +/- 6.9 to 6.5 +/- 1.8 eve
nts hr(-1) (p < 0.001) and movements/arousals fell from 10.4 +/- 2.2 t
o 4.8 +/- 0.2 hr(-1) (p < 0.04), Obstructive events were reduced from
33.7 +/- 6.9 to 2.4 +/- 1.0 hr(-1) (p < 0.001), Improvement of respira
tory indices was associated with an increased proportion of slow-wave
sleep from 25.2 +/- 4.0% to 32.3 +/- 2.4% (p = 0.01), and decrease in
stage 1-2 sleep from 59.3 +/- 5.8% to 46.6 +/- 1.9% (p = 0.03), There
was no increase in the percentage of REM sleep (15.2% to 21.2%), Repea
t SEP studies in 10 subjects, after clinically effective treatment of
OSA, showed improvement of SEP score of at least 1 grade, in 5 of 7 (7
1%) with initially abnormal values, We conclude that treatment to reli
eve upper airway obstruction improves OSA in achondroplasia, accompani
ed by changes in sleep structure and, in some cases, improved studies
of neurological function. (C) 1995 Wiley-Liss, Inc.