TY - JOUR
T1 - Sleep cycling alternating pattern (CAP) expression is associated with hypersomnia and GH secretory pattern in Prader-Willi syndrome
AU - Priano, Lorenzo
AU - Grugni, Graziano
AU - Miscio, Giacinta
AU - Guastamacchia, Giulia
AU - Toffolet, Lorenzo
AU - Sartorio, Alessandro
AU - Mauro, Alessandro
PY - 2006/12
Y1 - 2006/12
N2 - Background and purpose: Hypersomnia, sleep-disordered breathing and narcoleptic traits such as rapid eye movement (REM) sleep onset periods (SOREMPs) have been reported in Prader-Willi syndrome (PWS). In a group of young adult patients with genetically confirmed PWS we evaluated sleep and breathing polysomnographically, including cycling alternating pattern (CAP), and we analyzed the potential interacting role of sleep variables, sleep-related breathing abnormalities, hypersomnia, severity of illness variables and growth hormone (GH) secretory pattern. Patients and methods: Eleven males and 7 females (mean age: 27.5±5.5 years) were submitted to a full night of complete polysomnography and the multiple sleep latency test (MSLT). GH secretory pattern was evaluated by a standard GH-releasing hormone plus arginine test. Sixteen non-obese healthy subjects without sleep disturbances were recruited as controls. Results: Compared to controls PWS patients showed reduced mean MSLT score (P <0.001), reduced mean latency of sleep (P = 0.03), increased REM sleep periods (P = 0.01), and increased mean CAP rate/non-rapid eye movement (NREM) (P <0.001). Only four PWS patients had apnea/hypopnea index (AHI) ≥ 10. Conversely, significant nocturnal oxygen desaturation was frequent (83% of patients) and independent from apneas or hypopneas. In the PWS group, CAP rate/NREM showed a significant negative correlation with MSLT score (P = 0.02) independently from arousals, respiratory disturbance variables, severity of illness measured by Holm's score or body mass index (BMI). PWS patients with CAP expression characterized by higher proportion of A1 subtypes presented less severe GH deficiency (P = 0.01). Conclusions: Our study suggests a relationship between hypersomnia and CAP rate, and between CAP expression and GH secretory pattern in PWS, possibly reflecting underlying central dysfunctions.
AB - Background and purpose: Hypersomnia, sleep-disordered breathing and narcoleptic traits such as rapid eye movement (REM) sleep onset periods (SOREMPs) have been reported in Prader-Willi syndrome (PWS). In a group of young adult patients with genetically confirmed PWS we evaluated sleep and breathing polysomnographically, including cycling alternating pattern (CAP), and we analyzed the potential interacting role of sleep variables, sleep-related breathing abnormalities, hypersomnia, severity of illness variables and growth hormone (GH) secretory pattern. Patients and methods: Eleven males and 7 females (mean age: 27.5±5.5 years) were submitted to a full night of complete polysomnography and the multiple sleep latency test (MSLT). GH secretory pattern was evaluated by a standard GH-releasing hormone plus arginine test. Sixteen non-obese healthy subjects without sleep disturbances were recruited as controls. Results: Compared to controls PWS patients showed reduced mean MSLT score (P <0.001), reduced mean latency of sleep (P = 0.03), increased REM sleep periods (P = 0.01), and increased mean CAP rate/non-rapid eye movement (NREM) (P <0.001). Only four PWS patients had apnea/hypopnea index (AHI) ≥ 10. Conversely, significant nocturnal oxygen desaturation was frequent (83% of patients) and independent from apneas or hypopneas. In the PWS group, CAP rate/NREM showed a significant negative correlation with MSLT score (P = 0.02) independently from arousals, respiratory disturbance variables, severity of illness measured by Holm's score or body mass index (BMI). PWS patients with CAP expression characterized by higher proportion of A1 subtypes presented less severe GH deficiency (P = 0.01). Conclusions: Our study suggests a relationship between hypersomnia and CAP rate, and between CAP expression and GH secretory pattern in PWS, possibly reflecting underlying central dysfunctions.
KW - Apnea/hypopnea index
KW - Cycling alternating pattern
KW - GH deficiency
KW - Hypersomnia
KW - Multiple sleep latency test
KW - Prader-Willi syndrome
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U2 - 10.1016/j.sleep.2005.12.004
DO - 10.1016/j.sleep.2005.12.004
M3 - Article
C2 - 17023209
AN - SCOPUS:37849185145
SN - 1389-9457
VL - 7
SP - 627
EP - 633
JO - Sleep Medicine
JF - Sleep Medicine
IS - 8
ER -