Melatonin ineffective in neuronal ceroid lipofuscinosis patients with fragmented or normal motor activity rhythms recorded by wrist actigraphy
Adolescent; Adult; Antioxidants/tu [Therapeutic Use]; Child; Circadian Rhythm; Dose-Response Relationship; Drug Electrophysiology; Female; Humans; Male; Melatonin/tu [Therapeutic Use]; Neuronal Ceroid-Lipofuscinoses/dt [Drug Therapy]; Sleep Wake Disorders/th [Therapy]; 0 (Antioxidants); JL5DK93RCL (Melatonin); sleep disturbance/disorders; NCL3; pharmacologic intervention; melatonin
Melatonin was tested as a sleeping pill in five patients with neuronal ceroid lipofuscinoses. The single-blind, placebo-controlled study consisted of motor activity recordings, sleep logs, and administration of placebo or melatonin (2.5 or 5 mg). Daily motor activity rhythms were measured by wrist actigraphy during four 7-day periods (baseline, placebo, melatonin 2.5 mg, and melatonin 5 mg). The placebo or melatonin was administered in the evenings for 3 weeks, and the recordings were made during the last week of the 3-week treatment. Sleep logs were kept by the caregivers during the recordings. Based on period analyses, the activity recordings were evaluated to display a normal (24-h) or fragmented rhythm. Three patients had normal motor activity patterns during the baseline recordings, and administration of placebo or melatonin did not affect their rest/activity rhythms. Two patients had abnormally fragmented activity rhythms during the baseline periods, and administration of placebo or melatonin did not induce synchronization. According to the actigraphic data, there were no changes in activity rhythms resulting from administration of melatonin. However, based on the observations, three families reported that melatonin slightly improved the sleep quality of the patients. These controversial findings show the difficulties involved in specifying the role of melatonin in modulating sleep. Thus, we conclude that more evidence is required before the significance of melatonin as a sleeping pill is defined.Copyright 1999 Academic Press.
Hätönen T; Kirveskari E; Heiskala H; Sainio K; Laakso M L; Santavuori P
Molecular Genetics and Metabolism
1999
Article information provided for research and reference use only. PedPalASCNET does not hold any rights over the resource listed here. All rights are retained by the journal listed under publisher and/or the creator(s).
<a href="http://doi.org/10.1006/mgme.1999.2815" target="_blank" rel="noreferrer noopener">10.1006/mgme.1999.2815</a>
Sleep alterations in juvenile neuronal ceroid-lipofuscinosis
Age Factors; Male; Severity of Illness Index; Case-Control Studies; Child; Humans; Adult; Adolescent; Female; Brain; Genotype; Sleep Stages; Polysomnography; Sleep REM; Sleep Wake Disorders; Neuronal Ceroid-Lipofuscinoses; sleep disturbance/disorders; NCL3; trajectory; characteristics
In juvenile neuronal ceroid-lipofuscinosis (JNCL), sleep disorders are common. The purpose of this study was to investigate the sleep structure of 28 patients with JNCL compared with healthy controls subjects and to clarify the pathophysiology underlying the sleep disturbances in these patients. Each of 28 patients with JNCL (age range = 6-27 years), with or without sleep complaints, underwent one night of polysomnography. Electroencephalographic, electro-oculographic, electromyographic, and electrocardiographic findings were recorded. Sleep was scored and analyzed visually. The sleep parameters of the patients were compared with those of healthy control subjects. In most of the patients, the total sleep time, sleep efficiency, and percentages of rapid eye movement (REM) and non-REM (NREM) stage 2 sleep were significantly decreased, and the percentages of NREM stage 1 and slow-wave sleep and the number of nocturnal awakenings significantly increased. The percentage of NREM stage 1 and the number of awakenings increased with age and clinical stage. Paroxysmal epileptiform activity during light sleep (NREM stages 1-2) and high-amplitude delta-wave activity with intermingled sharp waves during slow-wave sleep were characteristic of the recordings. The present study revealed that in patients with JNCL, sleep is consistently altered.
Hamalainen M; Partinen M; Larsen A; Salmi T; Telakivi T; Kirveskari E; Santavuori P; Sainio K
Pediatric Neurology
2000
Article information provided for research and reference use only. PedPalASCNET does not hold any rights over the resource listed here. All rights are retained by the journal listed under publisher and/or the creator(s).
<a href="http://doi.org/10.1016/s0887-8994(00)00138-7" target="_blank" rel="noreferrer noopener">10.1016/s0887-8994(00)00138-7</a>