CEREBRAL PALSY

Introduction
1. CP was first described by the English physician Sir Francis William Little in1861 and was
known as Little’s disease.

2. The term static encephalopathy refers to a state of cerebral dysfunction after an insult of
limited duration. The resulting lesion does not progress; rather, it remains static or may even
improve somewhat with time.

3. Such an insult may occur during gestation, during the birth process, or during early
childhood.

4. The clinical picture will depend on the site and extent of the lesion and the age at the time of
occurrence. Cerebral palsy results from involvement of motor areas of the brain whereas
mental retardation follows diffuse cerebral involvement.
Definition:
It is a group of motor syndromes resulting from disorders of early brain development and
manifesting as chronic, static impairment of muscle tone, power, coordination and
movements often associated with epilepsy and abnormalities of speech, vision and
intellect.

Time of brain injury

1. Prenatal period: Conception to the onset of labor

2. Perinatal period: 28 weeks intrauterine to 7 days postnatal

3. Postnatal period: First two years of life

Prevalence: 2 per 1000; male/female ratio of 1.4/1.

Risk factors
 ETIOLOGY

1. CP is more common and more severe in boys than girl children. No definite etiologic diagnosis is possible in 25% of cases. 10% are

due to intrapartum asphyxia.

2. Prematurity, LBW - 10 to 28%. Very premature infants weighing <1,000 g, may develop CP (15/1000). In this group intracerebral

hemorrhage and periventricular leukomalacia (PVL) are common.

3. Antenatal Infections- 12%: Chorioamnionitis, Maternal sepsis, Temperature >38°c during labor, Urinary tract infection in mother

4. Inflammatory mediators: functional polymorphism in the interleukin-6 gene has recently been associated with a higher rate of CP

in term infants.

5. Thromboembolism: Inflammation can lead to coagulation abnormalities that could then lead to thromboembolic events. Fetal

stroke, a disruption of blood supply to the developing brain may be due to antiphospholipid antibody syndrome in mother.

6. Genetic factors: functional polymorphism in the interleukin-6 gene has recently been associated with a higher rate of CP in term

infants.

7. Multiple pregnancy was also associated with a higher incidence of CP

8. Infertility treatments has higher rate of CP, probably because of multiple pregnancies.

9. Maternal hypothyroidism
 Pathology

1. Gross malformations are found in only about one-third of autopsied children

with CP

2. Enhanced vulnerability of immature oligodendroglia to oxidative stress caused

by ischemia/infection/inflammatory insults

3. Main pathology:
a. Cortical migrational disorders

b. Periventricular leukomalacia

c. Periventricular hemorrhagic infarction

d. Selective neuronal necrosis

Cortical migrational disorders:
Cerebral palsy may result from disrupted neuronal migration during cortical development.
In periventricular area, embryonal neurons and fetal glial cells migrate outwardly to the
cortex. This results in migration defect which could lead to CP in addition to mental
retardation.
Periventricular leukomalacia:
1. Symmetric, focal necrosis of the white matter dorsal and lateral to the lateral ventricles.
2. Cystic cavities may develop in the more severe cases, diminished myelin and dilated
lateral ventricles
Periventricular hemorrhagic infarction
3. Consists of asymmetric hemorrhagic necrosis of the periventricular white matter plus
germinal matrix or intraventricular hemorrhage.
Selective neuronal necrosis
Neuronal loss and gliosis are found in the neocortex, hippocampus, cerebellum,
brainstem, and spinal cord and may be manifested clinically as quadriparesis.

Status marmoratus exhibits similar pathology localized to the basal ganglia and
thalamus and adds a degree of hyper myelinization to these regions, resulting in a
marbled appearance. This lesion is also seen in children with kernicterus. Dyskinetic
cerebral palsy may develop.
 Anatomical or topographic classification

1. Hemiplegia: Focal infarct or cortical, subcortical damage; Upper and lower extremity on one side of

body

2. Diplegia: Periventricular leukomalacia; Four extremities, legs more affected than the arms

3. Quadriplegia: Periventricular leukomalacia Ischemia, Multicystic encephalomalacia; infection; Four

extremities plus the trunk, neck and face

4. Triplegia: Both lower extremities and one upper extremity

5. Monoplegia: One extremity (rare)

6. Double hemiplegia: Four extremities, arms more affected than the legs

7. Athetoid: Kernicterus; Basal ganglia

 Physiologic classification:

1. Pyramidal (spastic) - 70% to 80%; Increased muscle tone is the defining characteristic of
this type. The muscles are stiff (spastic), and movements are jerky or awkward
2. Extra pyramidal (non spastic)
a. Dyskinetic (basal ganglia) - 10% to 15%
b. Athetoid- the child has uncontrolled movements that are slow and writhing. The
movements can affect any part of the body, including the face, mouth, and tongue
c. Dystonic- child has hypotonia with hyperreflexia
d. Ataxic (cerebellar)- 5%; this type affects balance and coordination; pure cerebellar
types are uncommon
3. Mixed
Spastic CP
1. Spasticity is defined as an increase in the physiological resistance of muscle to
passive motion. It is part of the upper motor neuron syndrome characterized by
hyperreflexia, clonus, extensor plantar responses and primitive reflexes. Spastic CP is
the most common form of CP.
2. Approximately 70% to 80% of children with CP are spastic. Spastic CP is anatomically
distributed into three types.
a. Spastic hemiplegia
b. Spastic diplgia
c. Spastic quadriplegia
 Spastic hemiplegia

1. Decreased spontaneous movements on the affected side and show hand preference at a very early age
2. Arm more involved than legs
3. Walking may be delayed up to 2yrs
4. Circumduction gait
5. Growth arrest (limb shortening) in affected limbs
6. Equinovarus deformity of foot
7. Tiptoe walking
8. Ankle clonus and Babinski sign
9. Increased DTR
10. Weakness of hand and foot dorsiflexors
11. 1/3 have epilepsy; ¼ have mental retardation
12. Family history may be suggestive of inherited clotting disorders such as factor V Leiden mutation.
CT scan:
13. Focal cerebral infarction: thromboembolic disorder: anticardiolipin antibodies
14. Calcification: intrauterine infection
 Spastic diplegia
1. Bilateral spasticity of legs greater than arms
2. First noticed when infant begins to crawl. While crawling infant drags the legs (commando crawl)
3. Application of diaper is difficult due to adductor spasm
4. Child is unable to sit due to paraspinal muscle involvement
5. Brisk reflexes, ankle clonus, bilateral Babinski
6. Scissoring posture on suspension by axillae
7. Walking is delayed
8. Equinovarus deformity of feet with tiptoe walking
9. Less growth of lower limbs and normal development of upper torso
10. Intellectual development for these patients is good, and the likelihood of seizures is minimal. Child may have learning disabilities
and deficits in vision, due to disruption of multiple white matter pathways that carry sensory as well as motor information.
Pathology: Periventricular leukomalacia near internal capsule with compensatory enlargement of the cerebral ventricles.
MRI: MRI with diffusion tensor imaging (DTI) is being used to map white matter tracks more precisely in patients with spastic diplegia,
and this technique has shown that thalamocortical sensory pathways are often injured as severely as motor corticospinal pathways
 Spastic quadriplegia
1. Most severe form of CP
2. Marked motor involvement of all four limbs with Increased tone in all limbs and decreased
movements
3. Severe mental retardation and seizures
4. Supranuclear bulbar palsy (pseudobulbar) with difficulty in swallowing and frequent
aspiration pneumonias
5. Hyperreflexia
6. Plantar extensor
7. Later: flexion contractures in limbs
8. Speech and visual abnormalities
9. Flexion contractures of the knees and elbows are often present by late childhood.
10. May have athetosis
11. Pathology: PVL and multicystic cortical encephalomalacia with cystic changes in cortex
 Athetoid CP (extrapyramidal CP):
1. Less common- 15-20% of patients with CP. More likely associated with birth asphyxia
2. Limbs are hypotonic with poor head control.
3. Dystonia refers to the abnormality in tone in which muscles are rigid throughout their range of motion and involuntary
contractions can occur in both flexors and extensors leading to limb positioning in fixed postures.
4. Unlike spastic diplegia, the upper extremities are generally more affected than the lower extremities in extrapyramidal
CP.
5. Difficulty in feeding, tongue thrust and drooling are prominent
6. Speech is slurred. Seizures are uncommon. Intellect is preserved
7. Athetoid CP can also be caused by kernicterus. Extrapyramidal CP can also be associated with metabolic genetic
disorders such as mitochondrial disorders and glutaric aciduria.
8. Dihydroxyphenylalanine (DOPA)-responsive dystonia (Segawa disease), causes prominent dystonia that can resemble CP.
9. Pathology: The lesions have a marbled appearance caused by neuronal loss and an overgrowth of myelin in the
putamen, caudate, and thalamus. It is described as status marmoratus and seen most often with kernicterus
 Associated disorders
1. Seizures – 40% in hemiplegic type

2. Mental retardation- 25 to 75%

3. Aphasia, dysarthria & apraxia (motor defect of speech)

4. Strabismus, refractive errors, and visual field defects

5. Deafness is in children caused by kernicterus

 Differential Diagnosis
1. CP is a static disorder and physical examination should rule out a
progressive disorders such as degenerative diseases, metabolic disorders,
spinal cord tumor, or muscular dystrophy.

2. MRI will rule out cervical spinal cord lesions like tumors and anomalies of
base of the skull and other congenital malformations
 Investigations & Diagnosis
Developmental Monitoring:
1. Physician monitoring the motor and intellectual functions during regular visits:
a. Listening to parenteral concern
b. Developmental Screening: screened for developmental delays during regular well-child office visits at: 9
months, 18 months and 24 or 30 months
Developmental and Medical Evaluations:
1. Child’s motor skills, muscle tone, reflexes, and posture
2. Intellectual disability; seizures; or vision, hearing, or speech problems
3. Cranial USG is useful in the differential diagnosis if the fontanelle is open. It does not require sedation. It
evaluates the ventricles, basal ganglia, corpus callosum for white matter ischemic injury and
intraventricular hemorrhage.
MRI in CP:
4. Loss of periventricular white matter& cystic changes,
5. Ventricular dilatation
6. Thinning of carpus callosum
EEG for seizures
Chromosomal disorders will have abnormal karyotype
Screening for metabolic disorders (amino acids, organic acids disorders and urea cycle disorder; arginase
deficiency)
 Early indicators of cerebral palsy

1. Episodes of inconsolable crying, chewing movements, excessive sensitivity to light or sound

2. Absent smile of recognition of mother at 2 months
3. Persistent primitive reflexes esp. Moro, tonic neck posture beyond 5- 6 months
4. Infants may exhibit either hypotonia or hypertonia,
5. Clenched fists (Cortical thumb) beyond 8 weeks
6. Lack of cuddliness
7. Tightness of the hips, making it difficult to separate the infant's legs to diaper him;
8. Kicking the legs in unison, bringing the knees up to the chest together, rather than the
alternating leg, bicycle style kicking of normal infants.
9. Evidence of lack of vision, inability to focus or to track moving objects.
10. Tongue thrust, excessive drooling.
 Abnormal Patterns or Delayed Motor Development
1. Failure to achieve head control, or to lift head and chest from a prone position when the
child is on his stomach, if older than 5 months.
2. Failure to reach for objects or to transfer objects from one hand to the other, if older than
7 months.
3. Inability to roll from back to front, if older than 6 months.
4. Hand preference occurs after 15 months; if it occurs before 12 months of age spastic
hemiplegia may be present.
5. Commando walk: good use of hands and arms, but drags legs from 6-7 months
6. Trembling or inaccurate aim when reaching for an object may indicate athetoid cerebral
palsy.
7. Walking on tiptoes. Young infants typically stand on their toes when held in a standing
position in an adult's lap. By the time the child learns to walk, heels should be flat on the
floor. A persistent toe-walking reflex may be indicative of cerebral palsy.
8. Slow head growth
 Grading of CP: Gross Motor Function Classification System (GMFCS)

Level Ability

1. Walks without restrictions

2. Walks without assistive devices but limitations in community

3. Walks with assistive devices

4. Transported or uses powered mobility

5. Severely limited dependent on wheelchair

 TREATMENT

1. Multidisciplinary approach: Paediatrician. Orthopaedician, Ophthalmologist, ENT surgeon with

occupational and physical therapists, speech pathologists, social workers, educators, and
developmental psychologists

2. Parental guidance (eg. “Spastics” society)

3. Physiotherapy

4. Occupational therapy

5. Tendon release

6. Appliances

7. Selected dorsal rhizotomy for spasticity

8. Ophthalmic management
 Drugs
1. For spasticity: Oral diazepam (0.5-7.5 mg/dose, BID or QID), baclofen (0.2-2 mg/kg/day, BID or TID) or dantrolene (0.5-10/kg/day, BID).

2. Levodopa (0.5-2 mg/kg/day) can be used to treat dystonia or DOPA-responsive dystonia.

3. Artane (trihexyphenidyl, 0.25 mg/day) for treating dystonia and can increase use of the upper extremities and vocalizations.

4. Reserpine (0.01 μg/kg/day, BID) or tetrabenazine (12.5-25 mg, BID or TID) can be useful for hyperkinetic movement disorders including

athetosis or chorea.

5. Intrathecal baclofen delivered with an implanted pump has been used successfully in many children with severe spasticity, and can be

useful because it delivers the drug directly around the spinal cord where it reduces neurotransmission of afferent nerve fibers.

6. Botulinum toxin injected into specific muscle groups for the management of spasticity shows a very positive response in many patients.

Botulism toxin injected into salivary glands may also help reduce the severity of drooling

7. Patients with rigidity, dystonia, and spastic quadriparesis sometimes respond to levodopa, and children with dystonia may benefit from

carbamazepine or trihexyphenidyl.

8. Communication skills may be enhanced by the use of Bliss symbols, talking typewriters, electronic speech generating devices, and

specially adapted computers including artificial intelligence computers to augment motor and language function.

9. Strabismus, nystagmus, and optic atrophy are common in children with CP; an ophthalmologist should be included in the initial

assessment.
 Prognosis

1. Mortality in CP is extremely variable. Life expectancy could be normal in diplegic

and hemiplgic conditions.

2. Poor prognosis in patients with paralysis of all four limbs (quadriplegia), severe
intellectual impairment and epilepsy have a worse outcome.

3. Approximately one quarter of CP patients will have minimal or no functional

limitation.

4. Half will be moderately impaired and although their functional capacity will be
satisfactory, achieving complete independence is unlikely.

5. The remaining quarter will be severely disabled and require full time care.
Causes of mortality:
1. Status epilepticus

2. Aspiration pneumonia

3. Delayed detection of life-threatening infections

4. Malnutrition

5. Mortality due to underlying genetic or factors

Prevention:
6. Prevention of preterm & LBW involves improving maternal anemia and malnutrition

7. Antenatal intra natal care to prevent birth asphyxia and sepsis.

8. Effective management of hyperbilirubinemia and meningitis in infancy

9. Prenatal treatment of the mothers with magnesium (neuroprotective agent) lowers the
prevalence of CP in their children and neuroprotection of NB with birth asphyxia (See HIE)