Aberrant sleep patterns in children with the Rett syndrome
Child; Humans; Adult; Adolescent; Female; Child Preschool; Infant; Rett Syndrome/co [Complications]; Sleep Disorders/co [Complications]; Adolescence; Aging; Rett Syndrome/dt [Drug Therapy]; Sleep Stages/ph [Physiology]; NET Files; human; child; female; adult; Rett Syndrome/complications/drug therapy; Sleep Stages/physiology; Sleep Wake Disorders/complications; sleep disturbance/disorders; Rett syndrome; trajectory; characteristics
Previous studies on the characteristics of disturbed sleep/wake patterns in children with the Rett syndrome have yielded inconsistent findings. In the current study, momentary time sampling procedures were used to measure the sleep/wake patterns of 20 girls with classical Rett syndrome. These patients had significantly more total sleep than age peers (M = 110.1; Zm = 2.58; p = .01), significantly less nighttime sleep (M = 80.8; Zm = -7.53; p less than .0001), and significantly more daytime sleep (M = 24.5; Zm = 8.71; p less than .0001). Night sleep was negatively correlated with age (r = -.59; p less than .01); day sleep was positively correlated with age (r = .54; p = .01). These girls also displayed night wakings on 20.9% of nights, delayed sleep onset on 67.8% of nights and early wakings on 24.5% of nights. These data clearly demonstrate that children with the Rett syndrome have markedly impaired sleep/wake patterns and suggests that the sleep dysfunction may worsen over time.
Piazza C C; Fisher W; Kiesewetter K; Bowman L; Moser H
Brain and Development
1990
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/s0387-7604(12)80213-0" target="_blank" rel="noreferrer noopener">10.1016/s0387-7604(12)80213-0</a>
Neurogenetic disorders and treatment of associated seizures.
Humans; Treatment Outcome; Epilepsies; Angelman Syndrome/dt [Drug Therapy]; Anticonvulsants/tu [Therapeutic Use]; Epilepsies; Rett Syndrome/dt [Drug Therapy]; Seizures/dt [Drug Therapy]; Tuberous Sclerosis/dt [Drug Therapy]; Angelman Syndrome/co [Complications]; Anticonvulsants/ad [Administration & Dosage]; Anticonvulsants/ae [Adverse Effects]; KT Synthesis; Myoclonic/co [Complications]; Myoclonic/dt [Drug Therapy]; Rett Syndrome/co [Complications]; Seizures/co [Complications]; Tuberous Sclerosis/co [Complications]
Seizures are a frequent complication associated with several neurogenetic disorders. Antiepileptic medications remain the mainstay of treatment in these patients. We summarized the available data associated with various antiepileptic therapies used to treat patients with neurogenetic disorders who experienced recurrent seizures. A MEDLINE search was conducted to identify articles and abstracts describing the use of antiepileptic therapy for the treatment of various neurogenetic syndromes. Of all the neurogenetic syndromes, only autism spectrum disorders, Angelman syndrome, Rett syndrome, Dravet syndrome, and tuberous sclerosis complex were identified as having sufficient published information to evaluate therapy. Some efficacy trends were identified, including frequent successes with valproic acid with clonazepam for epilepsy with Angelman syndrome; valproic acid, stiripentol, and clobazam (triple combination therapy) for epilepsy with Dravet syndrome; and vigabatrin for infantile spasms associated with tuberous sclerosis complex. Due to a paucity of information regarding the mechanisms by which seizures are generated in the various disorders, approach to seizure control is primarily based on clinical experience and a limited amount of study data exploring patient outcomes. Although exposure of the developing brain to antiepileptic medications is of some concern, the control of epileptic activity is an important undertaking in these individuals, as the severity of eventual developmental delay often appears to correlate with the severity of seizures. As such, early aggressive therapy is warranted. 2013 Pharmacotherapy Publications, Inc.
2013
Faulkner MA; Singh Sanjay P
Pharmacotherapy
2013
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).
Journal Article
<a href="http://doi.org/10.1002/phar.1201" target="_blank" rel="noreferrer">10.1002/phar.1201</a>
Aberrant sleep patterns in children with the Rett syndrome
Child; Female; Adult; Aging; Preschool; infant; Human; Adolescence; Rett Syndrome/co [Complications]; Sleep Disorders/co [Complications]; Rett Syndrome/dt [Drug Therapy]; Sleep Stages/ph [Physiology]
Previous studies on the characteristics of disturbed sleep/wake patterns in children with the Rett syndrome have yielded inconsistent findings. In the current study, momentary time sampling procedures were used to measure the sleep/wake patterns of 20 girls with classical Rett syndrome. These patients had significantly more total sleep than age peers (M = 110.1; Zm = 2.58; p = .01), significantly less nighttime sleep (M = 80.8; Zm = -7.53; p less than .0001), and significantly more daytime sleep (M = 24.5; Zm = 8.71; p less than .0001). Night sleep was negatively correlated with age (r = -.59; p less than .01); day sleep was positively correlated with age (r = .54; p = .01). These girls also displayed night wakings on 20.9% of nights, delayed sleep onset on 67.8% of nights and early wakings on 24.5% of nights. These data clearly demonstrate that children with the Rett syndrome have markedly impaired sleep/wake patterns and suggests that the sleep dysfunction may worsen over time.
1990
Piazza CC; Fisher W; Kiesewetter K; Bauman L; Moser H
Brain & Development
1990
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).
Journal Article
<a href="http://doi.org/10.1016/s0387-7604(12)80213-0" target="_blank" rel="noreferrer">10.1016/s0387-7604(12)80213-0</a>
Behavioral Treatment of Sleep Dysfunction in Patients with the Rett Syndrome
Child Preschool; Rett Syndrome/co [Complications]; Behavior Therapy; Sleep Disorders/th [Therapy]; Sleep Disorders/et [Etiology]; NET Files; human; child; female; Behavior Therapy; Child; Preschool; Female; Humans; Rett Syndrome/co [Complications]; Sleep Wake Disorders/et [Etiology]; Sleep Wake Disorders/th [Therapy]; sleep disturbance/disorders; Rett syndrome; psychological intervention; Fading procedure
Aberrant sleep patterns are commonly experienced by girls with the Rett syndrome. In this investigation, the problematic sleep of three girls with the Rett syndrome was regulated using a bedtime fading procedure with response cost. The treatment involved systematically delaying the bedtime and utilized a response cost component, removing the child from bed for one hour, when the child did not experience short latency to sleep onset. Daytime sleep was interrupted, except during regularly scheduled naps. A fading procedure was then successfully utilized to advance the bedtimes. This treatment resulted in more regular sleep patterns for the girls by increasing appropriate nighttime sleep, reducing inappropriate daytime sleep and reducing problematic nighttime behaviors (e.g., night wakings). These preliminary findings are important because they suggest that the dysfunctional sleep patterns of girls with the Rett syndrome may be amenable to behavioral treatments.
1991
Piazza CC; Fisher W; Moser H
Brain & Development
1991
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).
Journal Article
<a href="http://doi.org/10.1016/s0387-7604(12)80055-6" target="_blank" rel="noreferrer noopener">10.1016/s0387-7604(12)80055-6</a>