Sleep · 2009
Polysomnographic abnormalities in succinic semialdehyde dehydrogenase (SSADH) deficiency.
СтатьяПоддержка NIH: внешняя программаResearch Support, N.I.H., IntramuralПоддержка исследования: вне правительства СШАНазвание и аннотация: Перевод в очереди
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Pearl Phillip L, Shamim Sadat, Theodore William H, Gibson K Michael, Forester Katherine, Combs Susan E, Lewin Daniel, Dustin Irene, Reeves-Tyer Patricia, Jakobs Cornelis, Sato Susumu
Аннотация
OBJECTIVES: Patients with SSADH deficiency, a disorder of chronically elevated endogenous GABA and GHB, were studied for sleep symptoms and polysomnography. We hypothesized that patients would have excessive daytime somnolence and decreased REM sleep.
DESIGN: Polysomnography and MSLT were performed on patients enrolled for comprehensive clinical studies of SSADH deficiency.
SETTING: Sleep studies were obtained in the sleep laboratories at CNMC and NIH.
PATIENTS: Sleep recordings were obtained in 10 patients with confirmed SSADH deficiency.
INTERVENTIONS: Thirteen overnight polysomnograms were obtained in 10 patients (7 male, 3 female, ages 11-27 y). Eleven MSLT studies were completed in 8 patients.
MEASUREMENTS AND RESULTS: Polysomnograms showed prolongation of REM stage latency (mean 272 +/- 89 min) and decreased percent stage REM (mean 8.9%, range 0.3% to 13.8%). Decreased mean sleep latency was present in 6 of 11 MSLTs.
CONCLUSIONS: SSADH deficiency is associated with prolonged latency to stage REM and decreased percent stage REM. This disorder represents a model of chronic GABA and GHB accumulation associated with suppression of REM sleep.
DESIGN: Polysomnography and MSLT were performed on patients enrolled for comprehensive clinical studies of SSADH deficiency.
SETTING: Sleep studies were obtained in the sleep laboratories at CNMC and NIH.
PATIENTS: Sleep recordings were obtained in 10 patients with confirmed SSADH deficiency.
INTERVENTIONS: Thirteen overnight polysomnograms were obtained in 10 patients (7 male, 3 female, ages 11-27 y). Eleven MSLT studies were completed in 8 patients.
MEASUREMENTS AND RESULTS: Polysomnograms showed prolongation of REM stage latency (mean 272 +/- 89 min) and decreased percent stage REM (mean 8.9%, range 0.3% to 13.8%). Decreased mean sleep latency was present in 6 of 11 MSLTs.
CONCLUSIONS: SSADH deficiency is associated with prolonged latency to stage REM and decreased percent stage REM. This disorder represents a model of chronic GABA and GHB accumulation associated with suppression of REM sleep.