Assessment of self-reported and objective daytime sleepiness in adult-onset myotonic dystrophy type 1

Valeria A Sansone; Paola Proserpio; Luca Mauro; Andrea Lizio Biost; Erica Frezza; Andrea Lanza; Paola Rogliani; Gabriella Pezzuto; Elisa Falcier; Carola Ferrari Aggradi; Alice Pirola; Fabrizio Rao; Elisabetta Roma; Claudia Galluzzi; Matteo Spanetta; Federica Cattaneo; Annalisa Rubino; Elio Clemente Agostoni; Federica Amico; Alice Zanolini; Francesca Izzi; Giulia Greco; Andrea Romigi; Claudio Liguori; Lino Nobili; Fabio Placidi; Roberto Massa

doi:10.5664/jcsm.9438

Assessment of self-reported and objective daytime sleepiness in adult-onset myotonic dystrophy type 1

Valeria A Sansone, Paola Proserpio, Luca Mauro, Andrea Lizio Biost, Erica Frezza, Andrea Lanza, Paola Rogliani, Gabriella Pezzuto, Elisa Falcier, Carola Ferrari Aggradi, Alice Pirola, Fabrizio Rao, Elisabetta Roma, Claudia Galluzzi, Matteo Spanetta, Federica Cattaneo, Annalisa Rubino, Elio Clemente Agostoni, Federica Amico, Alice ZanoliniFrancesca Izzi, Giulia Greco, Andrea Romigi, Claudio Liguori, Lino Nobili, Fabio Placidi, Roberto Massa

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Abstract

STUDY OBJECTIVES: Excessive daytime sleepiness (EDS) in myotonic dystrophy type 1 (DM1) is mostly of central origin but it may coexist with sleep-related breathing disorders. However, there is no consensus on the sleep protocols to be used, assessments vary and only a minority of patients are regularly tested or are on treatment for EDS. Our study presents data on self-reported and objective EDS in adult-onset DM1.

METHODS: 63 adult-onset DM1 patients were subjected to EDS-sleep assessments (polysomnography, PSG; Multiple Sleep Latency Test, MSLT; Epworth Sleepiness Scale, ESS. Correlation coefficients were computed to assess the relationship between sleep and sleepiness test results, fatigue, quality of life.

RESULTS: 33% and 48% of patients had EDS based respectively on the ESS and the MSLT with a low concordance between these tests (k = 0.19). Thirteen patients (20%) displayed 2 or more sleep-onset REM periods on MSLT. Patients having EDS by MSLT had a shorter disease duration (p<0.05), higher total sleep time, sleep efficiency and lower WASO (Wake After Sleep Onset) on PSG. Patients with self-reported EDS reported significantly higher fatigue score compared to patients without EDS (p<0.05). No other difference was found in demographic, clinical and respiratory features.

CONCLUSIONS: EDS test results are contradictory making treatment options difficult. Combining quantitative tests and self-reported scales may facilitate physicians in planning EDS care with patients and families.

Original language	English
Pages (from-to)	2383-2391
Number of pages	9
Journal	Journal of clinical sleep medicine : JCSM : official publication of the American Academy of Sleep Medicine
Volume	17
Issue number	12
DOIs	https://doi.org/10.5664/jcsm.9438
Publication status	Published - Dec 1 2021

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Sansone, V. A., Proserpio, P., Mauro, L., Biost, A. L., Frezza, E., Lanza, A., Rogliani, P., Pezzuto, G., Falcier, E., Aggradi, C. F., Pirola, A., Rao, F., Roma, E., Galluzzi, C., Spanetta, M., Cattaneo, F., Rubino, A., Agostoni, E. C., Amico, F., ... Massa, R. (2021). Assessment of self-reported and objective daytime sleepiness in adult-onset myotonic dystrophy type 1. Journal of clinical sleep medicine : JCSM : official publication of the American Academy of Sleep Medicine, 17(12), 2383-2391. https://doi.org/10.5664/jcsm.9438

Assessment of self-reported and objective daytime sleepiness in adult-onset myotonic dystrophy type 1. / Sansone, Valeria A; Proserpio, Paola; Mauro, Luca et al.

In: Journal of clinical sleep medicine : JCSM : official publication of the American Academy of Sleep Medicine, Vol. 17, No. 12, 01.12.2021, p. 2383-2391.

Research output: Contribution to journal › Article › peer-review

Sansone, VA, Proserpio, P, Mauro, L, Biost, AL, Frezza, E, Lanza, A, Rogliani, P, Pezzuto, G, Falcier, E, Aggradi, CF, Pirola, A, Rao, F, Roma, E, Galluzzi, C, Spanetta, M, Cattaneo, F, Rubino, A, Agostoni, EC, Amico, F, Zanolini, A, Izzi, F, Greco, G, Romigi, A, Liguori, C, Nobili, L, Placidi, F & Massa, R 2021, 'Assessment of self-reported and objective daytime sleepiness in adult-onset myotonic dystrophy type 1', Journal of clinical sleep medicine : JCSM : official publication of the American Academy of Sleep Medicine, vol. 17, no. 12, pp. 2383-2391. https://doi.org/10.5664/jcsm.9438

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title = "Assessment of self-reported and objective daytime sleepiness in adult-onset myotonic dystrophy type 1",

abstract = "STUDY OBJECTIVES: Excessive daytime sleepiness (EDS) in myotonic dystrophy type 1 (DM1) is mostly of central origin but it may coexist with sleep-related breathing disorders. However, there is no consensus on the sleep protocols to be used, assessments vary and only a minority of patients are regularly tested or are on treatment for EDS. Our study presents data on self-reported and objective EDS in adult-onset DM1.METHODS: 63 adult-onset DM1 patients were subjected to EDS-sleep assessments (polysomnography, PSG; Multiple Sleep Latency Test, MSLT; Epworth Sleepiness Scale, ESS. Correlation coefficients were computed to assess the relationship between sleep and sleepiness test results, fatigue, quality of life.RESULTS: 33% and 48% of patients had EDS based respectively on the ESS and the MSLT with a low concordance between these tests (k = 0.19). Thirteen patients (20%) displayed 2 or more sleep-onset REM periods on MSLT. Patients having EDS by MSLT had a shorter disease duration (p<0.05), higher total sleep time, sleep efficiency and lower WASO (Wake After Sleep Onset) on PSG. Patients with self-reported EDS reported significantly higher fatigue score compared to patients without EDS (p<0.05). No other difference was found in demographic, clinical and respiratory features.CONCLUSIONS: EDS test results are contradictory making treatment options difficult. Combining quantitative tests and self-reported scales may facilitate physicians in planning EDS care with patients and families.",

author = "Sansone, {Valeria A} and Paola Proserpio and Luca Mauro and Biost, {Andrea Lizio} and Erica Frezza and Andrea Lanza and Paola Rogliani and Gabriella Pezzuto and Elisa Falcier and Aggradi, {Carola Ferrari} and Alice Pirola and Fabrizio Rao and Elisabetta Roma and Claudia Galluzzi and Matteo Spanetta and Federica Cattaneo and Annalisa Rubino and Agostoni, {Elio Clemente} and Federica Amico and Alice Zanolini and Francesca Izzi and Giulia Greco and Andrea Romigi and Claudio Liguori and Lino Nobili and Fabio Placidi and Roberto Massa",

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doi = "10.5664/jcsm.9438",

language = "English",

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TY - JOUR

T1 - Assessment of self-reported and objective daytime sleepiness in adult-onset myotonic dystrophy type 1

AU - Sansone, Valeria A

AU - Proserpio, Paola

AU - Mauro, Luca

AU - Biost, Andrea Lizio

AU - Frezza, Erica

AU - Lanza, Andrea

AU - Rogliani, Paola

AU - Pezzuto, Gabriella

AU - Falcier, Elisa

AU - Aggradi, Carola Ferrari

AU - Pirola, Alice

AU - Rao, Fabrizio

AU - Roma, Elisabetta

AU - Galluzzi, Claudia

AU - Spanetta, Matteo

AU - Cattaneo, Federica

AU - Rubino, Annalisa

AU - Agostoni, Elio Clemente

AU - Amico, Federica

AU - Zanolini, Alice

AU - Izzi, Francesca

AU - Greco, Giulia

AU - Romigi, Andrea

AU - Liguori, Claudio

AU - Nobili, Lino

AU - Placidi, Fabio

AU - Massa, Roberto

PY - 2021/12/1

Y1 - 2021/12/1

N2 - STUDY OBJECTIVES: Excessive daytime sleepiness (EDS) in myotonic dystrophy type 1 (DM1) is mostly of central origin but it may coexist with sleep-related breathing disorders. However, there is no consensus on the sleep protocols to be used, assessments vary and only a minority of patients are regularly tested or are on treatment for EDS. Our study presents data on self-reported and objective EDS in adult-onset DM1.METHODS: 63 adult-onset DM1 patients were subjected to EDS-sleep assessments (polysomnography, PSG; Multiple Sleep Latency Test, MSLT; Epworth Sleepiness Scale, ESS. Correlation coefficients were computed to assess the relationship between sleep and sleepiness test results, fatigue, quality of life.RESULTS: 33% and 48% of patients had EDS based respectively on the ESS and the MSLT with a low concordance between these tests (k = 0.19). Thirteen patients (20%) displayed 2 or more sleep-onset REM periods on MSLT. Patients having EDS by MSLT had a shorter disease duration (p<0.05), higher total sleep time, sleep efficiency and lower WASO (Wake After Sleep Onset) on PSG. Patients with self-reported EDS reported significantly higher fatigue score compared to patients without EDS (p<0.05). No other difference was found in demographic, clinical and respiratory features.CONCLUSIONS: EDS test results are contradictory making treatment options difficult. Combining quantitative tests and self-reported scales may facilitate physicians in planning EDS care with patients and families.

AB - STUDY OBJECTIVES: Excessive daytime sleepiness (EDS) in myotonic dystrophy type 1 (DM1) is mostly of central origin but it may coexist with sleep-related breathing disorders. However, there is no consensus on the sleep protocols to be used, assessments vary and only a minority of patients are regularly tested or are on treatment for EDS. Our study presents data on self-reported and objective EDS in adult-onset DM1.METHODS: 63 adult-onset DM1 patients were subjected to EDS-sleep assessments (polysomnography, PSG; Multiple Sleep Latency Test, MSLT; Epworth Sleepiness Scale, ESS. Correlation coefficients were computed to assess the relationship between sleep and sleepiness test results, fatigue, quality of life.RESULTS: 33% and 48% of patients had EDS based respectively on the ESS and the MSLT with a low concordance between these tests (k = 0.19). Thirteen patients (20%) displayed 2 or more sleep-onset REM periods on MSLT. Patients having EDS by MSLT had a shorter disease duration (p<0.05), higher total sleep time, sleep efficiency and lower WASO (Wake After Sleep Onset) on PSG. Patients with self-reported EDS reported significantly higher fatigue score compared to patients without EDS (p<0.05). No other difference was found in demographic, clinical and respiratory features.CONCLUSIONS: EDS test results are contradictory making treatment options difficult. Combining quantitative tests and self-reported scales may facilitate physicians in planning EDS care with patients and families.

U2 - 10.5664/jcsm.9438

DO - 10.5664/jcsm.9438

M3 - Article

C2 - 34170223

VL - 17

SP - 2383

EP - 2391

JO - Journal of Clinical Sleep Medicine

JF - Journal of Clinical Sleep Medicine

SN - 1550-9389

IS - 12

ER -

Assessment of self-reported and objective daytime sleepiness in adult-onset myotonic dystrophy type 1

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