Cerebral Palsy

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

When pulling up, the head lags

Hearing and speech Responds to loud noise

Ventral suspension

Social Comforted 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 puts hands in mouth

Speech and hearing Social 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

Palmer grasp for holding objects

Social 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

Turns in response to his own name

Social

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

CAUSES OF DEVELOPMENTAL DELAY PRENATAL INSULT Teratogens Congenital infections Hypothyroidism PERINATAL INSULT Complication of extreme prematurity Birth asphyxia Metabolic disorder

CAUSES OF DEVELOPMENTAL DELAY

POST NATAL EVENTS Brain injury CNS infection Metabolic

(Oxford handbook of Pedriatics)

CEREBRAL PALSY

DEFINITION

“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.)

INCIDENCE •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”)

RISK FACTORS 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)

BRAIN DAMAGE

White matter damage PERIVENTRICULAR LEUKOMALACIA

Abnormal brain development CEREBRAL DYSGENESIS

BRAIN DAMAGE

Lack of oxygen supply HYPOXIC ISCHEMIC ENCEPHALOPATHY

Brain hemorrhage INTRACRANIAL HEMORRHAGE

(Nelson textbook of Pedriatics, 19th Edition)

PRESENTATION

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

(Illustrated textbook of Pediatrics, 3rd Edition)

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

Less common Damage to the upper motor neurons of the extra pyramidal tract DYSKINETIC CEREBRAL PALSY

Least common Damage to cerebellar pathways ATAXIC CEREBRAL PALSY (Nelsons Essentials of Pediatrics, 6TH Edition)

CLASSIFICATION OF CEREBRAL PALSY Major syndrome

Neuropathology/MRI

Major causes

SPASTIC DIAPLEGIA(35%)

Periventricular leukomalacia Periventricular cysts or scars White matter scars

Prematurity Ischemia Infection Endocrine/metabolic

SPASTIC QUADRIPLEGIA(20%)

Periventricular leukomalacia Multicystic encephalomalacia Cortical malformations

Ischemia, infection Endocrine/metabolic Genetic/developmental

HEMIPLEGIA(25%)

Stroke; in utero or neonatal Focal infarct Cotical malformations

Thrombophilic disorders Infection Genetic/developmental Hemorrhagic infarct

EXTRAPYRIMIDAL(15%)

Asphyxia; scars in putamen and thalamus Kernicterus; scars in globus pallidus, caudate, putamen

Asphyxia Kernicterus Mitochondrial Genetic/metabolic

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

SPASTIC CEREBRAL PALSY DIPLEGIA

•Legs are affected more than arms. Legs adducted and internally rotated.

SPASTIC CEREBRAL PALSY 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

NON-SPASTIC CEREBRAL PALSY ATAXIC CEREBRAL PALSY Intention tremors

DYSKINETIC CEREBRAL PALSY Chorea Athetosis

COMMON ASSOCIATIONS

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

(Illustrated textbook of Pediatrics, 3rd Edition)

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

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

INVESTIGATIONS

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

TREATMENT Multidisciplinary approach • • • •

Medical therapy Surgical therapy Rehabilitation Continuous monitoring

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

MEDICAL THERAPY FOR SPASTICITY Benzodiazepines Baclofen Oral diazepam Dantrolene FOR DYSTONIA Levodopa Trihexyphenidyl Reserpine Tetrabenzene

MEDICAL THERAPY Intrathecal baclofen

Botulinum toxin

SURGICAL THERAPY Derotation osteotomy Dorsal 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