I disturbi del sonno nelle Paralisi Cerebrali infantili
Transcript of I disturbi del sonno nelle Paralisi Cerebrali infantili
I disturbi del sonno nelle
Paralisi Cerebrali infantili
Lino Nobili
Centro di Medicina del Sonno
Centro per la Chirurgia dell’Epilessia
Dipartimento di Neuroscienze
Ospedale Niguarda “Ca Granda”
Milano
Milano, 22.09.2015
The wake-sleep transition is characterized by clear-cut modifications of EEG activity :
from low-amplitude high-frequency activity to sleep spindles and high-amplitude low-
frequency slow waves
Stadio REM
Normal "hypnogram" of sleep stage changes over the night
Saper et al Nature 2005
key projections of the ventrolateral preoptic
nucleus (VLPO) to the main components of
the ascending arousal system.
Prevalenza attività Gabaergica
Prevalenza attività colinergica
Zee et al 2013
Nap
Achermann and Borbely 1999
• Disturbi dell’inizio e del mantenimento del sonno
• Disturbi del ritmo sonno veglia
• Disturbi respiratori in sonno
Didden R, et al J Intellect Disabil Res. 2002; Wiggs and Stores G J Intellect Disabil Res. 1996; Simard-Tremblay et al J Child Neurology 2011
Sleep disorders in cerebral palsy
• It is estimated that 13% to 85% of children with neurodevelopmental disabilities, including cerebral palsy, suffer from a clinically significant sleep problem.
• It remains unclear whether cerebral palsy, among a population of children with neurodevelopmental disabilities, represents a risk factor for the presence of sleep problems
Didden R, et al J Intellect Disabil Res. 2002; Wiggs and Stores G J Intellect Disabil Res. 1996; Simard-Tremblay et al J Child Neurology 2011
Sleep disorders in cerebral palsyQuestionnaire-based survey
Questionnaire-based survey
• 23% of the children with cerebral palsy had an abnormal total sleep score, and 44% of children had at least one clinically significant sleep disorder.
• 48% of children with physical disabilities (cerebralpalsy, spina bifida, muscular dystrophy, and other diagnoses) had sleep problems; 24% had moderate or severe difficulties.
Wiggs and Stores G J Intellect Disabil Res. 1996; Newman CJ et al Dev Med Child Neurol. 2006; Hemmingsson H et al Child Care Health Dev. 2009; Simard-Tremblay et al J Child Neurology 2011
Developmental Medicine & Child Neurology 2006, 48: 564–568
Why children with cerebralpalsy have more apparent sleep difficulties?
• Dysfunctions of the neuronal circuits regulating sleep (cyrcadian rhythm, REM sleep)Brainstem dysfunction: cardiac and respiratory control may be altered as well as level of alertness;
• Visual impairment
• Comorbid epilepsyNocturnal seizures (major and minor seizures, drugs)
• Severity and type of motor disability (amount and quality of body movements)
• Abnormal Tone and Pain
• Pulmonary aspirationmicro-aspiration and cough may disrupt sleep architecture.
• Intellectual disabilities (conflicting results)Poor communication skills are a better predictor of sleep problems than the degree of cognitive limitations.
Piazza CC et al J Child Neurol. 1996; Lindblom N, et al. J Sleep Res. 2001; Levi R, et al Paraplegia. 1995; Simard-Tremblay et al J Child Neurology 2011
Sleep problems may not always beappropriately addressed in clinical practice
only 19% of the parents of a child with a current sleep problem had received any advice about their child’s sleep disorder from a health care
professional.
Didden R, et al J Intellect Disabil Res. 2002; Simard-Tremblay et al J Child Neurology 2011
The consequences of sleep disordersin children with cerebral palsy affect both
the child and the family.
Sleep dysfunction has a negative effect on daytime behavior and on school
performance of both typically developing children and children with cerebral palsy.
Richdale A, et al J Intellect Dev Disabil. 2000; Gozal D. Pediatrics 1998; Kotagal S et al Dev Med Child Neurol 1994;Simard-Tremblay et al J Child Neurology 2011
Consequences of Sleep Problems on theChild and the Family
• Increased irritability, hyperactivity, aggression, screaming, and impulsivity.
• Reduction of school performance.
• Increased frequency of sleep disorders in the parents.
• Parents of children with sleep problems feel more stressedand irritable.
• Family stressors may negatively affect a child’s sleep pattern.
Didden et al J Intellect Disabil Res. 2002; Zucconi M and Bruni O Semin Pediatr Neurol. 2001; Chervin et al Sleep 1997; Simard-Tremblay et al J Child Neurology 2011
2011
Child and maternal sleep disturbance were significantly correlated.
2013
Treatment of Sleep Problems
• Behavioral interventions– graduated extinction, parent education, positive bedtime routines, sleep
hygiene
• Antihistamines, Benzodiazepines, (?)
• 5-hydroxytryptophan, Melatonin
• Baclofen, botulinum (spasticity)
Overall the literature reports
indicate that melatonin is a safe
treatment with no adverse side
effects reported, although it has been
suggested that there may be
a theoretical risk of delayed puberty at
doses well in excess of those
normally prescribed
Kalsa et al J Physiol 2003
Treatment approaches for delayed sleep-phase disorder
and advanced sleep-phase disorder
Zee et al 2013
Sleep related breathing disorders
• Higher risk for sleep-related breathing disorders in children with cerebral palsy
• abnormal upper airway muscle tone
• primary central abnormality affecting the central control of breathing
Kohrman MH and Carney PR. Pediatric Neurol. 2000; Kotagal S et al Dev Med Child Neurol 1994; Dorris L et al Dev Neurorehabil. 2008
Sleep related breathing disorders
14.5% of children cerebral palsy had a pathologic score for the disorders of sleep-
related breathing on the Sleep Disturbance Scale for Children.
Newman CJ et al Dev Med Child Neurol. 2006
Sindrome delle Apnee Ostruttive in sonno
…Apnee Ostruttive in Sonno: ripetuti episodi di ostruzione delle alte vie aeree che occorrono in
sonno e sono usualmente associate a ipossiemia…..
Trudo et al. AJRCCM 1998;158:1259-70
Awake Asleep
collasso delle pareti dell’alto faringe durante sonno
• I bambini con ostruzione delle alte vie respiratorie hanno più frequentemente ipopnee che a volte durano parecchi minuti configurando una ipoventilazione ostruttiva con ipercapnia.• L’ipertrofia del tessuto adenotonsillare è il più comune fattore di rischio nella genesi dei disturbi respiratori nel sonno nei bambini.
Sleep related breathing disorders in cerebral palsyRisk Factors
• Adenotonsillar hypertrophy
• Disproportionate midface anatomy
• Mandibular alterations
• Skeletal deformity, scoliosis
• Abnormality of upper airway tone
(hypotonia, hypertonia or dystonia)
• Abnormal central control of respiration
• Obesity
• Drugs that depress upper airway muscle tone.
Simard-Tremblay et al J Child Neurology 2011
Cardio-respiratory monitoring
snoring
Nasal airflow
Thoracic movements
SpO2
Heart rate
Tone of respiratory muscles
Principles and Practice Sleep Medicine Kryget Roth Dement 2011
ASPETTI CLINICI• respiro orale• respiro rumoroso o russamento• sonno agitato• sudorazione profusa in sonno• parasonnie
• cefalea mattutina• iperattività • deficit attentivo• irritabilità• rinolalia• sonnolenza (rara)
• ritardo della crescita• cuore polmonare• ritardo mentale
Sintomi notturni
Sintomi diurni
Complicanze
Obstructive sleep apnea is a multifactorial disorder in children with cerebral palsy
• Individualized treatment depending on the underlying neurologic abnormalities and on the site of obstruction.
- Adenotonsillar hypertrophy
- Mandibular advancement(consider the increased risk of postsurgical complications)
- CPAP, Bi-level
- Control of abnormal tone
- Management of comorbid conditions
gastroesophageal reflux, hypersalivation, obesity
Simard-Tremblay et al J Child Neurology 2011; Cohen SR. Et al Plast Reconstr Surg. 1997
Research in Developmental Disabilities 29 (2008) 133–140
Moro-reflex, Sleep startles
normal children
Cerebral Palsy
Sleep assessment
Diari del sonno
Hirshkowitz et al 2011
Hermann et al 2011
ActigrafiaSLEEP 2003
In Lab full Video-
Polysomnography
PORTABLE systems