Diapositiva 1

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 lowfrequency slow waves
Stadio REM
Normal "hypnogram" of sleep stage changes over the night
key projections of the ventrolateral preoptic
nucleus (VLPO) to the main components of
the ascending arousal system.
Prevalenza attività Gabaergica
Prevalenza attività colinergica
Saper et al Nature 2005
Zee et al 2013
Nap
Achermann and Borbely 1999
Sleep disorders in cerebral palsy
• 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
Questionnaire-based survey
• 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
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 (cerebral
palsy, 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 cerebral
palsy 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 epilepsy
Nocturnal seizures (major and minor seizures, drugs)
• Severity and type of motor disability
(amount and quality of body movements)
• Abnormal Tone and Pain
• Pulmonary aspiration
micro-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 be
appropriately 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 disorders
in 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 the
Child 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 stressed
and 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 sleeprelated 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…..
Awake
Asleep
Trudo et al. AJRCCM 1998;158:1259-70
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 palsy
Risk 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
Sintomi notturni
• cefalea mattutina
• iperattività
• deficit attentivo
• irritabilità
• rinolalia
• sonnolenza (rara)
Sintomi diurni
• ritardo della crescita
• cuore polmonare
• ritardo mentale
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
Actigrafia
SLEEP 2003
Hermann et al 2011
In Lab full VideoPolysomnography
PORTABLE systems