Sleep disorders in adult-onset myotonic dystrophy type 1: a controlled polysomnographic study

Abstract

Background: Sleep disturbances and excessive daytime somnolence are common and disabling features in adult-onset myotonic dystrophy type 1 (DM1). Methods: Our study used questionnaires, ambulatory polysomnography and the multiple sleep latency test to evaluate sleep-wake cycle and daytime sleepiness in unselected adult-onset DM1 patients. We recruited 18 patients affected by adult-onset DM1 and 18 matched controls. Results: Sleep efficiency was <90% in 16/18 patients, and it was significantly reduced when compared with controls. Reduced sleep efficiency was associated with abnormal respiratory events (5/18 patients) and/or periodic limb movements (11/18 patients). The Periodic Limb Movement Index was significantly increased in DM1 versus controls. A significantly lower mean MSLT sleep latency was detected in DM1 versus controls, but it did not reach pathological levels. Conclusions: Our controlled study demonstrated sleep alterations in unselected consecutive DM1 patients. Periodic limb movements in sleep are commonly associated with sleep disturbance in adult-onset DM1, and it may represent a marker of CNS neurodegenerative processes in DM1

Sleep disorders in spinal and bulbar muscular atrophy (Kennedy’s disease): a controlled polysomnographic and self-reported questionnaires study

“…Polysomnographic and sleep quality evaluation All participants underwent a 48-h dynamic ambulatory polysomnogram (A-PSG) in order to evaluate diurnal and nocturnal sleep (Somnoscreen, SOMNOmedics GmbHRandersacker, Germany) as described elsewhere [5]. Since the first night was considered as an adaptation night, only the results from the second one were used for analysis.…”

“…A-PSG was followed by the multiple sleep latency test (MSLT) to objectively evaluate daytime somnolence [5].…”

“…No patients showed and reported diurnal naps during the first 24-h session potentially interfering with sleep architecture of the second night. Sleep analysis, apnea/hypopnea events and leg movements were scored according to standard criteria [5][6][7][8]. Apnea-Hypopnea Index (AHI) [5 and Periodic Limb Movements Index (PLMI) [15 were considered pathological [5].…”

“…Sleep analysis, apnea/hypopnea events and leg movements were scored according to standard criteria [5][6][7][8]. Apnea-Hypopnea Index (AHI) [5 and Periodic Limb Movements Index (PLMI) [15 were considered pathological [5]. REM sleep without atonia (RSWA) is empirically defined as excessive amounts of sustained or intermittent elevation of submental electromyogram (EMG) tone or excessive phasic submental EMG twitching with the other features of REM sleep that are desynchronized EEG and rapid eye movements.…”

Effects of epilepsy treatment on sleep architecture and daytime sleepiness: An evidence‐based review of objective sleep metrics

“…Therefore, there is a growing evidence that unattended portable devices are useful in different clinical settings. [7][8][9] An experienced sleep technologist/ technician must apply the sensors. Manual scoring review of the raw data should be performed by board certified sleep specialists.…”