PAEDIATRICS

TOPIC DEVELOPMENTAL DELAY: Cerebral Palsy

OBJECTIVES

• A brief overview of developmental milestones• Difference between the terms developmental delay

and regression• Cerebral palsy Etiology Presentation Treatment

DEVELOPMENTAL MILESTONES

DEFINITION: “Important developmental skills attained over time are

called developmental milestones”

Median age

Limit age

Adjustment for prematurity

(Illustrated textbook of Pediatrics, 3rd Edition)

AT BIRTH…

(Illustrations from Developmental Milestones)

Gross motor Fine motor

Hearing and speech Social

When pulling up, Ventral suspension the head lags

Responds to loud noiseComforted by mother’s presence

AT 03 MONTHS…

Gross motor

Lifting head and chest in prone position

Raised head on ventral suspension

Little head lag On sitting up

Fine motor

Speech and hearing

Social

puts hands in mouth

Vocalizes, laughs

recognizes mother

AT O6 MONTHS…

Gross motor

Uses hands and arm for support when prone

Uses shoulders for support when pulled to sit

Can bear most of the weight

Fine motor

Speech and hearing

Social

Palmer grasp for holding objects

Changes position to localize sound

Social smile

AT 01 YEAR…

Gross motor

Fine motor

Walks with one hand held

Walks with feet apart

Pincer grasp and hand preference

Speech and hearing

SocialTurns in response to his own name

Speaks two to six recognizable words

AT 02 YEARS…

Gross motor

Complete coordinated walk and movements

Fine motor

Speech, hearing and social

Drinks independently from a cup

Spectator play

AT 5 YEARS…

Gross motor

Fine motor Hearing speech and social

Bends and touches the toe

Draws a person

Can build complex models

DEVELOPMENTAL DELAY

DEFINITION: “Global developmental delay implies delay in acquisition of

all skill fields (gross motor, vision and fine motor, hearing and speech/language, social/emotional and behavior). It usually becomes apparent in the first 2 years of life”

(Illustrated textbook of Pediatrics, 3RD Edition)

DEVELOPMENTAL REGRESSION

DEFINITION: “ A previously healthy child begins to deteriorate losing

already attained skills with progressive loss of speech, hearing, vision and muscle strength for more than three months”

(National Conference on Neurogenetics, SESSION: REGRESSION OF MILESTONES)

CAUSES OF DEVELOPMENTAL DELAY

GLOBAL DEVELOPMENTAL DELAY

GENETICS Chromosomal disorders Duchene muscular dystrophy Metabolic syndrome

CONGENITAL BRAIN ANOMALIES Hydrocephalus

PRENATAL INSULT Teratogens Congenital infections Hypothyroidism

PERINATAL INSULT Complication of extreme prematurity Birth asphyxia Metabolic disorder

CAUSES OF DEVELOPMENTAL DELAY

CAUSES OF DEVELOPMENTAL DELAY

POST NATAL EVENTS Brain injury CNS infection Metabolic

(Oxford handbook of Pedriatics)

CEREBRAL PALSY

“Cerebral palsy is a diagnostic term used to describe a group of permanent disorders of movement and posture causing activity limitation, that are attributed to non progressive disturbances in the developing infant or fetal brain”

(Nelson Textbook of Pediatrics, 19th Edition.)

DEFINITION

•Most common and most costly form of chronic motor disability

•Incidence 3.6/1000 live births in developed nations •Incidence 1.5 to 6/1000 live births in developing world.

•M:F 1.4:1 •Incidence greater in premature and twin births.

(United Cerebral Palsy Research and Education Foundation” Cerebral Palsy Fact Sheet”)

INCIDENCE

ANTENATAL

•Low socioeconomic status•Low birth weight/ fetal growth retardation(less than 1500g at birth)•Treatment with thyroid hormone, estrogen or progesterone•Multiple births•Maternal fever

RISK FACTORS

PERINATAL

•Congenital malformations/ syndromes•Newborn hypoxic ischemic encephalopathy•Kernicterus

RISK FACTORS

POST NATAL

•Meningitis•Head injury car crashes violence•Stroke

(Nelson Essentials of Pediatrics, 6th Edition)

RISK FACTORS

White matter damagePERIVENTRICULAR LEUKOMALACIA

Abnormal brain developmentCEREBRAL DYSGENESIS

BRAIN DAMAGE

Lack of oxygen supply HYPOXIC ISCHEMIC ENCEPHALOPATHY

Brain hemorrhageINTRACRANIAL HEMORRHAGE

BRAIN DAMAGE

(Nelson textbook of Pedriatics, 19th Edition)

•Delayed motor milestones•Abnormal limb tone/trunk posture •Slowing of head growth•Feeding difficulties•Abnormal gait•Asymmetrical hand function•Persistent primitive reflexes

PRESENTATION

(Illustrated textbook of Pediatrics, 3rd Edition)

Most common form Damage to the upper motor neuron of the pyramidal tract.SPASTIC CEREBRAL PALSY

Less commonDamage to the upper motor neurons of the extra pyramidal tractDYSKINETIC CEREBRAL PALSY

Least commonDamage to cerebellar pathwaysATAXIC CEREBRAL PALSY

TYPES OF CEREBRAL PALSY

(Nelsons Essentials of Pediatrics, 6TH Edition)

Major syndrome Neuropathology/MRI Major causesSPASTIC DIAPLEGIA(35%) Periventricular leukomalacia

Periventricular cysts or scarsWhite matter scars

PrematurityIschemiaInfectionEndocrine/metabolic

SPASTIC QUADRIPLEGIA(20%)

Periventricular leukomalaciaMulticystic encephalomalaciaCortical malformations

Ischemia, infectionEndocrine/metabolicGenetic/developmental

HEMIPLEGIA(25%) Stroke; in utero or neonatalFocal infarctCotical malformations

Thrombophilic disordersInfectionGenetic/developmentalHemorrhagic infarct

EXTRAPYRIMIDAL(15%) Asphyxia; scars in putamen and thalamusKernicterus; scars in globus pallidus, caudate, putamen

AsphyxiaKernicterusMitochondrialGenetic/metabolic

CLASSIFICATION OF CEREBRAL PALSY

(Table 591-1, Nelsons textbook of Pediatrics,19th Edition)

SPASTIC CEREBRAL PALSY

DIPLEGIA

•Legs are affected more than arms.

Legs adducted and internally rotated.

QUADRIPLEGIA•All four limbs affected•Arms affected more than legs Extensor posturing Poor head control Low central tone

SPASTIC CEREBRAL PALSY

HEMIPLEGIA

• Unilateral involvement of arm and leg • Arm affected more than leg• Face spared

Fisting of the affected hand Flexed arm Pronated forearm

SPASTIC CEREBRAL PALSY

NON-SPASTIC CEREBRAL PALSY

ATAXIC CEREBRAL PALSY Intention tremors

DYSKINETIC CEREBRAL PALSY Chorea Athetosis

•Learning difficulties (about 60%) •Epilepsy (40%) •Squints (30%) •Visual impairment from errors of refraction and cortical damage (20%) •Hearing impairment (20%)

COMMON ASSOCIATIONS

(Illustrated textbook of Pediatrics, 3rd Edition)

•Neuroimaging Cranial USG MRI Brain MRI Spinal Cord CT•Conduction studies •Laboratory tests Blood tests •Hearing and visual assessment

INVESTIGATIONS

(Forfars and Arneils Textbook of Pediatrics, 3rd Edition)

USG Cranium: PVL

MRI brain TS: Periventricular Leukomalacia

INVESTIGATIONS

MRI Brain TS: Infarct

INVESTIGATIONS

MRI Brain TS: Cerebral Malformation

INVESTIGATIONS

MRI Brain TS: Resolving Hemorrhage

INVESTIGATIONS

TREATMENT

Multidisciplinary approach

• Medical therapy• Surgical therapy• Rehabilitation• Continuous monitoring

(Forfars and Arneils Textbook of Pediatrics, 3rd Edition)

MEDICAL THERAPY

FOR SPASTICITYBenzodiazepinesBaclofenOral diazepamDantrolene

FOR DYSTONIALevodopaTrihexyphenidylReserpineTetrabenzene

Intrathecal baclofen

Botulinum toxin

MEDICAL THERAPY

SURGICAL THERAPY

Derotation osteotomyDorsal root rhizotomy

REHABILITATION

REFERENCES•Nelson Textbook of Pediatrics, 19th Edition•Nelsons Essentials of Pediatrics, 6th Edition•Illustrated Textbook of Pediatrics, 3rd Edition•Oxford Handbook of Pediatrics•Forfar’s and Arneil’s Textbook of Pediatrics, 6th Edition•Cerebralpalsy.com•Illustrations from Developmental milestones Forfar’s and Arneil’s Textbook of Pediatrics Neuroimaging copyrights Indiana University•All original photographs taken with parental consent•Courtesy AFIRM