Sleep in the Prader-Willi syndrome. Clinical and polygraphic findings
A. Vela-Bueno, A. Kales, C. R. Soldatos, B. Dobladez-Blanco, J. Campos-Castello, P. Espino-Hurtado and J. Olivan-Palacios
Nine patients with the Prader-Willi syndrome, ranging in age from 3 to 21
years, were examined clinically as well as studied in the sleep laboratory.
They had striking disturbances of sleep-wakefulness patterns. All patients
except one had the symptom of excessive daytime sleepiness. The most
striking finding was the presence in five patients of rapid-eye-movement
(REM) sleep occurring at sleep onset (SOREM). None of the patients had the
condition of sleep apnea. One patient, however, demonstrated severe
hypoventilation during REM sleep; the lowest value recorded for O2
saturation was 40%, with a consistent value below 50% for as long as ten to
15 minutes. Previous findings have indicated that the Prader-Willi syndrome
is of hypothalamic origin. We hypothesize that both the SOREM and O2
desaturation findings in our patients with the Prader-Willi syndrome are
also a result of hypothalamic changes.
