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