Mayo Clinic 2 - 88 - Sleep Disorder In Infants And Children

Front 1

Prevalence of sleep disorders in children?

Back 1

Nightly - 12%

Occasionally - 76%

Front 2

Populations that are at higher risk of sleep disorders? (6)

Back 2

1. Down syndrome

2. Neuromuscular disease

3. Craniofacial abnormalities

4. Intracranial tumors

5. Epilepsy

6. Developmental delay

Front 3

How does sleep affect children?

Back 3

Direct association of cognitive deficits and shortened life span

2. Total sleep time directly correlates with high academic achievement and enjoyment, infrequent illness, and decreased absenteeism

Front 4

Sleep relation to epilepsy? (4)

Back 4

1. Sleep is generally considered an activating state for seizure activity

2. Higher rate of sleep-disordered breathing, estimated at more than 40% in children with epilepsy

3. AEDs tend to cause weight gain, increasing risk for OSA

4. Both central and obstructive sleep apnea are more frequent; however, it is unclear whether central sleep apnea leads to seizures or vice versa

Front 5

Seizures related to sleep

Back 5

1. Paroxysmal arousals

2. Nocturnal paroxysmal dystonia

3. Episodic nocturnal wandering

Front 6

EEG of Landau-Kleffner syndrome?

Back 6

Continuous spike-and-wave activity in non REM sleep but absent in REM sleep and in the awake state (electrical status epilepticus in slow-wave sleep)

Front 7

Normal sleep behaviours not covered earlier that often leads to referral (4)

Back 7

1. Benign sleep myoclonus of infancy - myoclonus in an otherwise healthy infant with normal EEG

2. Nocturnal psychogenic nonepileptic seizures - episodes of bizarre norhythmic movements arising out of wakefulness even though they appear during sleep without an EEG accompaniment

3. Sandifer syndrome - Nocturnal arousal with posturing and stiffening; often due to GERD at ages 2 months to 5 years

4. Jactatio capitus (head banging) - Headbaning, along with body rocking, that occurs in wakefulness or during sleep (typical in autistic children)

Front 8

Sleep-disordered breathing in children with neuromuscular disease can be due to (2)

Back 8

1. Anatomical anomalies (e.g., craniofacial abnormalities)

2. Disturbance in physiologic function of neuromuscular control

Front 9

Test that is useful for determining whether the child has an associated sleep disorder and whether further testing and treatment should be done?

Back 9

Pulmonary function test; usually restrictive pattern and vital capacity less than 60% predicted

Front 10

Next step when ___ falls below __%?

Back 10

FEV1; 40%; arterial blood gas: PaCO2 of 45mm Hg or more was a sensitive and more specific test than FEV1; next step is PSG if 45 mm Hg or more

Front 11

Strongest predictor of the onset of sleep-disordered breathing?

Back 11

Inspiratory vital capacity of less than 60%

Front 12

Strongest predictor for nocturnal hypercapnic hypoventilation?

Back 12

Inspiratory vital capacity of less than 40%

Front 13

Severely disrupted sleep-wake pattern may be what syndrome?

Back 13

Smith-Magenis; congenital disorder characterized by an inverted pattern of melatonin secretion

Front 14

Facial features of Smith-Magenis syndrome?

Back 14

1. Brachycephaly

2. Down-slanting palpebral dissures

3. Flat midface

4. Down-turned mouth

Front 15

Smith-Magenis syndrome progression (4)

Back 15

1. Easy-going and excellent sleeper

2. By age 1-2 complaints of frequent night awakenings and daytime naps

3. By childhood, aggressive behaviours, temper tantrums and self-injurious behaviours

4. Global developmental delay early

Front 16

Characteristics of Angelman syndrome (5)

Back 16

(Happy puppet syndrome)

1. Problems with sleep maintenance and seizures

2. Balance problems

3. Wide-based gait

4. Puppet-like hand movements

5. Cheery disposition

Front 17

Characteristics of Prader-Willi syndrome? (5)

Back 17

1. Hyperphagia

2. Obesity

3. Hypogonadism

4. Mental retardation

5. Excessive daytime sleepiness

Front 18

Early severe insomnia, seizures and aggressive behaviours?

Back 18

MPS III (Sanfilippo syndrome)

Front 19

Genetic disorders with associated sleep disturbances? (7)

Back 19

1. Autism spectrum disorders

2. Prader-Willi and Angelman

3. Mucopolysaccharidoses

4. ADHD

5. Rett syndrome

6. Down syndrome

7. Chiari malformation

Front 20

Pathognomic breathing disorder of Rett syndrome

Back 20

While awake periods of hyperventilation followed by apneas, but do not have musch sleep-disordered breathing at night

Front 21

Sleep disturbances in Rett syndrome? (5)

Back 21

1. Laughter

2. Seizures

3. Bruxism

4. Nighttime screaming

5. Frequent daytime napping

Front 22

Course of Rett syndrome

Back 22

Girl with normal development for 1-2 years, start walking and begins to talk, over time parents notice hand flapping or wringin movements, and the child will lose the speech ability

Front 23

Hallmarks of Rett syndrome?

Back 23

It is considered a neurodegenerative disease

1. Microcephaly

2. Loss of purposeful hand function

3. Seizures that are difficult to control

Front 24

Why are Down syndrome patients at increased risk of sleep breathing disorder?

Back 24

Posteriorly placed tongue, small nose, and absent frontal and sphenoid sinuses form a relatively crowded oropharyngeal airway

Front 25

How common is OSA in patients with Down syndrome?

Back 25

Common enough that some experts recommend PSG after the age of 3 years in all children with this diagnosis

Front 26

Role of MRI in chiari malformation?

Back 26

MRI of the craniocervical junction in both flexion and extension can aid in treatment guidance by providing dynamic imaging