CEREBRAL PALSY

Introduction

• Historically known as static encephalopathy

• A group of motor impairment syndromes resulting from disorders of early braindevelopment.

• Often associated with epilepsy and abnormalities of speech, vision and intellect.

• However, many children and adult with CP function at a high educational and vocational level without sign of cognitive dysfunction

Epidemiology

• CP is the most common and costly form of chronic motor disability

• Prevalence: 2/1000

• Prevalence of CP is increased in low birth weight infants (<1000g)

• CP incidence higher in premature and twin birth

Aetiology

•Infection- German measles

- Shingles

•Diabetes•Toxemia of pregnancy

•Rh incompatibility

•Asphyxia•Birth injury•Prematurity

Caused by developmental, genetic, metabolic, ischemic, infections

Antenatal factors (80%)

Intrapartum(10%)

Postpartum (10%)

• Very high fever

• Brain infection

• Head injury

• Lack of oxygen

• Poisoning

• Intracranial hemorrhage or blood clot

Risk Factors

• Before Pregnancy:- History of fetal wastage- Long menstrual cycle- Maternal thyroid disorder- Family history of mental

retardation

• During Labor and Delivery:- Premature separation of

placenta

• During Early Postnatal Period:- Newborn hypoxic ischemic or

bilirubin (kernicterus) encephalopathy

• During Pregnancy:- Low socioeconomic status- Tx of mother with thyroid

hormone, estrogen or progesterone

- Maternal seizure disorder- Polyhydramnios- Eclampsia- Bleeding in 3rd trimester- Twin gestation- Congenital malformation- Fetal growth retardation- Abnormal fetal presentation

Physiologic

identify forms of motor impairment

Spastic CP

Dyskinetic CP

Ataxic CP

Mixed CP

Distribution

identify location of musculoskeletal involvement

Spastic diplegia

Spastic quadriplegia

Spastic hemiplegia

Classification

Spastic CP

• The most common form of CP (70-80%)

• Due to injury to upper motor neurons of pyrimidal tract

• Often exhibit truncal hypotoniain 1st year of life

•Characterized by at least 2 of following:-Abnormal movement pattern-Increased tone-Pathologic reflexes (Babinski, hyperreflexia)

Dyskinetic CP

• 10-15%

• Result of injury to basal ganglia (associated with kernicterus)

• Characterized by variable tonal abnormalities & involuntary movement (athetosis, chorea)

• Fewer seizures & >normal cognitive function

Ataxic CP

• <5% of CP cases – rare

• Results from cerebellar injury

• Abnormalities of voluntary movement and balance

• Wide-based, unsteady gait, abnormal muscle tone

Mixed CP

• 10-15% of all cases

• > 1 type of motor pattern is present & when 1 pattern does not clearly dominate another

• Associated with > complications: sensory deficits, seizures, cognitive-perceptual impairments

Dystonic CP

• Uncommon

• Characterized by reduced activity and stiff movement (hypokinesia) and hypotonia

Choreoathetotic CP

• Rare

• Caused by excess hyperbilirubinemia

• Dominated by increased and stormy movement (hyperkinesia) and hypotonia

Clinical Manifestations

• Spectrum of developmental abnormalities

• Mental retardation

• Epilepsy

• Motor handicap

•Visual, hearing, speech, cognitive & behavioral abnormalities

Spastic diplegia

• 25-35%

• Bilateral spasticity of legs

• 1st noted when infant begins to crawl

• Child uses arm normally but drag the legs behind

• Application of diaper is difficult

• Unable to sit

• On examination: - Brisk reflexes- Ankle clonus- Bilateral Babinski sign - Scissoring posture of lower extremities• Delayed walking• Feet in equinovarus• Walk on tiptoe• Disuse atrophy

Spastic hemiplegia

• Decreased spontaneous movements on the affected side

• Show hand preference at very early age

• Difficulty in hand manipulation

• Delayed walking

• Circumductive gait

• Growth arrest in hand and thumbnails

• Equinovarus of foot

• Walks on tiptoe

• Ankle clonus

• Babinski sign

• Increase deep tendon reflexes

• Weakness of hand and foot dorsiflexors

• 1/3 have seizure, cognitive abnormalities

Spastic quadriplegia

• The most severe form

• Marked motor impairment of all extremities

• Mental retardation & seizure

• Swallowing difficulty

• Increased tone and spastic

• Decreased spontaneous movement

• Brisk reflexes

• Plantar extensor response

• Flexion contraction of knee and elbow

• Speech & visual abnormalities

• Athetosis

Athetoid CP

• Less common

• Dyskinetic CP

• Associated with birth asphyxia

• Hypotonic with poor head control, head lag

• Increased variable tone with rigidity and dystonia

• Feeding difficulty, tongue thrust, drooling

Diagnosis

• History and PE should preclude progressive disorder of CNS, degenerative disease, metabolic disorders, spinal cord tumor, muscular dystrophy

• MRI scan of brain or spinal cord

• Test of hearing and visual function

• Genetic evaluation

Management• CP cannot be cured

• Family support – educate parents

• Adjunctive therapy:

- Physiotherapy

- Occupational therapy

- Speech therapy

• Surgery

• Psychologist or psychiatrist

•Adaptive equipment

• Drugs:

- Oral dantrolene sodium, benzodiazepines, baclofen – treat spasticity

- Botulinum toxin

- Levodopa

Thank you!!

References:

• Kliegman, Behrman, Jenson & Stanton (2007). Nelson Textbook of Pediatrics. 18th Edition

• Kliegman, Jenson, Marcdante & Berhman (2006). Nelson Essentials of Pediatrics. 5th Edition