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To identify the periodicity in the diurnal rest-activity rhythms, the motor activity level was recorded by activity monitors continuously for a 1-week period with concomitant sleep logs. In addition, whole-night polysomnographic recordings were performed. The patients under 20 years of age had an excess of nocturnal sleep (the mean of the usual duration of nighttime sleep was 10.0 hours) and frequent daytime naps. Frequent shifts of the longest sleep period into the daytime hours and fragmented diurnal rest-activity patterns with no distinct rhythm occurred in the older patients. The progressive disease may damage the internal circadian timing system and also impair the ability of patients with variant late infantile neuronal ceroid lipofuscinosis to use external time cues for synchronization of their sleep and environmental time. (J Child Neurol 2001;16:707-713).<\/jats:p>","DOI":"10.1177\/088307380101601001","type":"journal-article","created":{"date-parts":[[2008,3,31]],"date-time":"2008-03-31T18:40:10Z","timestamp":1206988810000},"page":"707-713","source":"Crossref","is-referenced-by-count":17,"title":["Sleep and Its Disturbance in a Variant Form of Late Infantile Neuronal Ceroid Lipofuscinosis (CLNS)"],"prefix":"10.1177","volume":"16","author":[{"given":"Erika","family":"Kirveskari","sequence":"first","affiliation":[{"name":"Department of Pediatric Neurology, Helsinki University Central Hospital, Hospital for Children and Adolescents, Department of Clinical Neurophysiology, Helsinki University Central Hospital"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Markku","family":"Partinen","sequence":"additional","affiliation":[{"name":"Haaga Neurological Research Center, Helsinki, Finland"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Pirkko","family":"Santavuori","sequence":"additional","affiliation":[{"name":"Department of Pediatric Neurology, Helsinki University Central Hospital"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"179","published-online":{"date-parts":[[2001,10,1]]},"reference":[{"key":"atypb1","unstructured":"Zeman W., Donohue S., Dyken P., Green J.: The neuronal ceroid-lipofuscinoses (Batten Vogt-syndrome), in Vinken PJ, Bruyn GW (eds): Handbook of Clinical Neurology, Vol 10. 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