Sleep alterations in juvenile neuronal ceroid-lipofuscinosis

Title

Sleep alterations in juvenile neuronal ceroid-lipofuscinosis

Creator

Hamalainen M; Partinen M; Larsen A; Salmi T; Telakivi T; Kirveskari E; Santavuori P; Sainio K

Publisher

Pediatric Neurology

Date

2000

Subject

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

Description

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.

Rights

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).

Citation

Hamalainen M; Partinen M; Larsen A; Salmi T; Telakivi T; Kirveskari E; Santavuori P; Sainio K, “Sleep alterations in juvenile neuronal ceroid-lipofuscinosis,” Pediatric Palliative Care Library, accessed January 25, 2022, https://pedpalascnetlibrary.omeka.net/items/show/16675.

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