CEREBRAL PALSY

INTRODUCTION

Cerebral Palsy a.k.a. “Infantile Cerebral Paralysis” or “Little’s Disease” is the term given to a
diverse group of non-progressive syndromes that affect the brain and cause motor dysfunction beginning
in early infancy. Lesion affects the immature brain and interferes with the maturation of the central nervous
system. CP is the most common form of chronic physical disability of childhood. Although the brain lesions
that result in cerebral palsy are not progressive, the clinical picture of CP may change with time as the
affected individual grows and develops.
The motor disorders of CP are often accompanied by disturbances of sensation, perception,
cognition, communication and behavior as well as seizures and secondary musculoskeletal problems2.
Motor performance can deteriorate once adulthood is reached due to the impact of various secondary
conditions.

ETIOLOGY AND RISK FACTORS

There is no single specific cause of the constellation of symptoms known as CP. Rather, the
potential causes of CP are known to occur in the prenatal stage of development and are also grouped with
congenital problems in the perinatal or neonatal time period, and in the postnatal or post neonatal time
period. Perinatal asphyxia is thought to cause 6% to 8% of CP, with the underlying causes being
unpreventable, and 10% to 18% of CP is thought to be caused postnatal. The cause of CP in the majority
of infants born at term in developing countries is due to prenatal influences and is not associated with
significant neonatal encephalopathy. Risk factors can be present before or during pregnancy, during labor
and birthing, and in the period shortly after the birth of the infant.
Premature birth is a well-recognized risk factor for CP, which results in an increased risk per
infant up to 100-fold. The overall incidence of preterm and very preterm birth is quite low, however, and
prematurity is implicated in half or less of CP cases. The most common CP subtype in premature infants is
spastic diplegia. Although prematurity is a risk factor, the etiology of the CP is often multifactorial and a
direct result of one or multiple environmental, genetic, or maternal factors that caused the preterm delivery.
The life expectancy in CP is reduced depending upon the severity and other associated health
conditions. Several studies have demonstrated that survival is influenced by the presence of severe mental
retardation and reduced mobility.

A. PRENATAL FACTORS
These are the causative factors from the time of conception to the onset of labor.
1. Hereditary factors
 basic pathology being genetically transmitted to developmental defects in the basal
nuclei or in their infarcts
 diseases such as familial athetosis, congenital tremors, familial spastic paraplegia,
atonic diplegias, and familial rigidities
2. Acquired during gestation
a. Irradiation
 X-ray therapeutic irradiation of the lower abdominal area of a pregnant
female during the first trimester produces cerebral damage to the
developing fetus
 total amount of x-ray required to produce severe manifestations of CP is
small
b. Maternal Infection
 Rubella(German Measles) contracted during the 1st trimester of
pregnancy, aside from CP, deafness, cataracts, congenital cardiac
malformation, and auditory aphasia
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 Chicken pox, influenzia, syphilis, mumps, measles, herpes zoster

(cytomegalovirus), and toxoplasmosis
c. Perinatal Anoxia
 any mechanism which interferes with the exchange of oxygen through
the placenta to the fetus
 if sufficiently prolonged, will produce permanent injury to the fetal brain
 mechanisms which produce interference with the free flow of oxygen:
1. placental abnormalities
2. maternal anoxia
3. cord anomalies
4. maternal hypotension
5. maternal anoxia
d. Hemorrhage during pregnancy
e. Fetal cerebral hemorrhage
 hemorrhage within the fetal brain or on the surface of the brain
 may occur as a result of damage to cerebral blood vessels after prolonged
anoxia
 may also be due to trauma to the brain while still in utero
f. Kernicterus
 production of permanent damage to the basal ganglia
 due to improperly treated hyperbilirubinemia in premature babies
 Triad of Kernicterus:
o high frequency hearing loss
o loss of upward gaze (Parinaud’s Syndrome)
o athetosis
g. Prematurity
 said to be the most common perinatal cause of CP
h. Predisposition to miscarriage
i. Metabolic disturbance during pregnancy
 DM is outstanding
 brain damaged children are seen in some diabetic mothers who have
difficulty conceiving
 pregnant diabetics gain excess weight and deliver unusually large babies
 there is difficulty reviving these babies and in postnatal management
during the 1st ninety – six hours after birth
j. Maternal Toxemia
 passes through the placental barrier and the fetus is usually severely
involved

B. PERINATAL FACTORS
Period from birth to 4 weeks
1. Mechanical Cause
a. Prolonged labor
 duration of labor becomes an increasingly important factor in producing
trauma with cerebral hemorrhage whenever the length of labor exceeds
18 hours (Primiparous mother), 12 hours (Multiparous mother)
b. Birth trauma
 differs from head injuries since it is caused by slow molding and
compression of the head rather than violent blows or trauma
2. Fetal Asphyxia
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 undue suppression of the respiratory centers due to excessive premedication of

the mother
 can also be due to prolonged use of anesthetics (i.e. Nitrous Oxide)
 Factors productive to fetal anoxia:
a. mechanical respiratory obstruction
b. injudicious use of analgesics and anesthetics

C. POSTNATAL FACTORS
Period from 1 month to 6 years
1. Traumatic injuries
 only considered CP if trauma occurred during the child’s first 2 years
 it is because by 2 years, the child’s brain (sensory portion) is already fully
developed
 if the trauma occurs after 2 years and has shown symptoms, it is already
considered TBI
2. Infections
 encephalitis, meningitis, and brain abscess are frequent causes of cerebral
infections
3. Toxic Factors
 Toxicity is a very common cause of CP (i.e. Alcohol)
4. Vascular Accident
 occurs in cases where there is congenital aneurysm of the brain, particularly in
the Circle of Willis: MCA
5. Cerebral Anoxia
 due to carbon monoxide poisoning or high altitude anoxia
6. Brain tumors
 includes not only brain tumors but also brain cysts and hydrocephalus

EPIDEMIOLOGY

The majority of cases in term infants do not have an identifiable etiology. The greatest risk factor
for the development of CP is prematurity. Premature infants (born earlier than 37 weeks’ gestation) are
much more likely to develop the condition than term infants, and incidence rates are highest in the very
earliest infants. Rates of CP in premature and low birth-weight infants vary from 40 to 150 per 1,000 live
births.
Forty percent of the children had mental retardation, which, when combined with the slow learners
and persons with learning disabilities, increased to 75%; epilepsy occurred in 35% of the population; visual
impairment in 20%; and hydrocephalus in 9%. Thirteen percent of the children had a combination of two
impairments, and 15% had a combination of three impairments. Additional associated problems include
difficulty with speech in 25% and hearing impairments in 25%. Historically, children were diagnosed with
CP if the insult occurred prenatally, perinatally, and postnatally. On the basis of the current definition, no
upper age limit has been determined for postnatal onset. For this reason, children are being diagnosed with
CP throughout infancy and early childhood.

CLASSIFICATION OF CP
1. Topographic Classification
a. Monoplegia - involves only one limb
b. Paraplegia – LE is either spastic or rigid; UE are normal
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c. Hemiplegia – half of the body is affected and is usually spastic; presence of sensory
involvement in the areas of proprioception, 2-point discrimination, and form perception;
aphasias appear more frequently in (R) > (L) hemiplegia
d. Triplegia - involves 3 extremities usually both legs and one arm, usually spastic
e. Quadriplegia – involvement of 4 extremities
f. Diplegia – legs are more involved than the arms
g. Double Hemiplegia – arms are more involved than the legs

2. Degree of gross motor skills

Gross Motor Function Classification System (GMFCS). In this system, specific descriptions of mobility
functions, based on age, allow each child with CP to be categorized.

3. Therapeutic Classification of CP

*CLASS A not requiring treatment

*CLASS B needs minimal bracing and treatment
*CLASS C needs bracing, apparatus and rehab team
*CLASS D require a long term institutionalization and treatment

4. Physiologic Classification
a. Spastic
 due to a cortical/pyramidal lesion; Brodmann area 6 affection (65%-80%)
 most common neurologic abnormality seen in CP patients
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 has a lower threshold of the stretch reflex, an enlarged reflexogenic area

 augmented response with clonus and an abnormal EMG record
 greater tendency toward contractures, specifically, the antigravity muscles
 muscle changes can cause secondary disorders such as hip dislocation, scoliosis,
knee contracture , and torsional malalignments of the femur and tibia
Types:
1. Spastic Hemiplegia
 presents with a typical hemiplegic posture
 delayed milestone by 4-6 months
 favorable ambulation by 3 years old
 impaired sensation
 limb impairment (affected limb is smaller)
 with homonymous hemianopsia and astereognosis
 may have moderate intelligence impairment or can be normal
2. Spastic Diplegia
 presents with bunny hopping and/or combat crawl
 with toe walking or scissoring gait
 delayed walking until 3-4 years old
 normal or near intelligence
 strabismus
3. Spastic Quadriplegia
 most severe; poorest prognosis
 characterized by seizures (damage to Basal Ganglia or Occipital area together
with visual impairments), mental retardation and strabismus
 often accompanied by neck and trunk involvement
 cognition vary from normal to severely impaired
 (+) Straphanger sign (shoulder abducted with flexed elbow and fingers)
b. Dyskinetic
2 subtypes:
1. Athetoid
 characterized by abnormal and involuntary movements
 affects subcortical structures and the basal ganglia (20%)
 with high intelligence, a happy disposition and extroverted
 “hand spooning” (hyperextension of fingers)
 Triad of Kernicterus/Hyperbilirubinemia
 may present with normal reflexes, fluctuating tone and normal EMG
2. Ataxic
 primary incoordination due to the disturbance of kinesthetic or balance sense
 affection of the cerebellum and CN VIII
 (+) Romberg’s sign: patient loses balance whenever his/her eyes are closed
 (+) Piano stool test
 hypotonic with normal or decreased DTR’s
 with equilibrium dyssynergias and nystagmus
 exhibit “Rebound Phenomenon” (absence of reflex activation of antagonist muscle
upon release of resistance to the agonist (e.g. upon releasing the resistance to elbow
flexion the patient accidentally hits his face)
c. Mixed – most common is the spastic and athetoid type
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Once a child is classified by the type of movement disorder and anatomical location of impaired
motor function, further differentiation is made on the basis of the severity of motor dysfunction.

ASSOCIATED ABNORMALITIES

1. Oral dental disabilities

 dysphagia – due to spasm or pharyngeal muscle paralysis
 bruxism – indicative of severe mental retardation
 dental carries – enamel dysplasia
2. Speech
 due to maldevelopment of oropharyngeal structures
 oral-motor incoordination
 defective control of respiratory muscles
3. Vision and extraocular movements
 strabismus – present in spastic diplegia and quadriplegia
 nystagmus
 homonymous hemianopsia
4. Hand Astereognosis
5. Seizure
6. Mental retardation
7. Perceptual dysfunction
 agnosia
 apraxia
8. Behavioral disorders
 attention deficits, distractability, impulse disturbance, overt hyperkinesis
9. Deformities
 Straphanger deformity
 Bird wing deformity
 Cortical thumb
 Scoliosis
 Windswept deformity
 Club foot deformity

PROGNOSIS

a. Molnar Prognostication Index for Ambulation

1. Based on the type of CP
*BEST – spastic hemiplegia or ataxic
*POOR - quadriplegic, flaccid/rigid
2. Based on the onset of sitting
*GOOD – if patient can sit by 2 years old
*FAIR – patient can sit between 2-4 years old (50% chance of ambulation)
*POOR – if patient cannot sit by 4 years old

b. Bleck’s Prognosis for Ambulation

*GOOD (+) Parachute, (-) Primitive
*FAIR (+) Parachute, (+) 1 Primitive
*POOR (+) Parachute, (+) 2 Primitive
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PATHOPHYSIOLOGY
Several factors, alone or in combination, can produce brain damage that leads to cerebral palsy.
Prenatal cerebral hypoxia can be responsible for systemic degeneration of immature areas of the brain and
can interfere with cell maturation. The severity of the damage depends on the gestational age at the time of
the injury and the degree of injury sustained.
The underlying brain lesion in premature infants classically manifests as awhite matter abnormality
on neuroimaging. These white matter changes are a consequence of both arterial hemorrhage from fragile
capillaries in the watershed zone next to the lateral ventricles known as the germinal matrix, and
periventricular hemorrhagic infarction of venous origin. The highest risk for periventricular hemorrhage is
between 23 and 32 weeks’ gestation. The extent of the bleeding into the germinal matrix is described by a
grading system. Intraventricular hemorrhage can be detected early in the clinical course via trans fontanelle
cerebral high-resolution ultrasound (US). Hemorrhagic infarction of venous origin in the periventricular
region is usually asymmetric and located lateral to the external angle of the lateral ventricle. With healing
of the periventricular hemorrhage, symmetric necrosis of the white matter adjacent to the lateral ventricles
ensues, resulting in Periventricular Leukomalacia (PVL). The prevalence of CP in infants born before 36
weeks of gestation and weighing less than 2000g has been reported to be the strongest independent risk
factor.
Quadriplegic CP can be caused by any etiology that induces a diffuse bilateral insult to the brain,
such as severe anoxic or ischemic brain injury. Intrapartum or birth asphyxia must be severe and prolonged
to cause CP and accounts for less than 10% of quadriplegic CP cases. Because of the diffuse nature of injury
to cortical and subcortical areas of the brain in quadriplegic CP, cognitive deficits, seizures, and the degree
of disability are most severe in this group.
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SIGNS AND SYMPTOMS

Early identification of children who have CP allows for early therapeutic intervention and screening
for associated conditions. Because CP is a descriptive term that does not infer a single etiology, pathology,
or prognosis, there is no specific diagnostic test. It is a diagnosis of exclusion based on a careful history and
physical exam. It can be difficult to make a definitive diagnosis in infants less than 6 months old. Prior to
this time, the infant has a limited repertoire of volitional movements, which makes milder delays in motor
development difficult to detect. In addition, abnormalities in tone and reflexes are often subtle in early
infancy. As the cortex matures in the second half of the first year, the diagnosis typically becomes more
apparent.
Often the parent’s initial concern is a significant delay in attaining motor milestones such as rolling
over, raising head, sitting up, and crawling. Prematurity must be considered when evaluating development
because milestones are generally corrected for the degree of prematurity. A discrepancy between motor
and cognitive milestones should always raise suspicion for CP. Certain deviations in developmental
milestones are associated with CP.
The earliest indication of CP may be a delay in the disappearance of primitive infantile reflexes.
Commonly examined primitive reflexes include the Moro reflex, palmar grasp reflex, asymmetric tonic
neck reflex, and tonic labyrinthine reflex. During the first six months of life, maturation of the cortex
gradually overrides these primitive responses, and voluntary motor activity should increase. Persistence of
these primitive reflexes past six months of age, asymmetry of the response, or an obligatory response at any
age should be considered highly suspicious for a significant motor impairment. As the primitive reflexes
become suppressed, postural or protective reactions such as the parachute and the equilibrium or tilting
reactions should emerge. In children with CP, postural reactions may be less effective, appear later than
usual, or fail to develop.
A definitive diagnosis of CP should be made cautiously, especially in the first six months of life.
Infants who are suspected of having CP should be followed closely with serial developmental evaluations
and physical exams until the diagnosis is clear. Further evaluation, including neuroimaging, should be
considered to help clarify the diagnosis.

TREATMENT

The severity of the child’s impairments, their functional limitations, as well as the child and
family’s goals all influence the design of an effective treatment plan. There is no singular recommended
intervention for any specific category of CP as each infant and child presents a unique array of functional
competencies, desired outcomes, functional limitations, and impairments. It is common and necessary to
use principles from a variety of treatment approaches for an effective treatment.
 Physical Therapy
1. Stretching
2. Strengthening
3. Electrical Stimulation
o Neuromuscular electrical stimulation (NMES) - utilizes electrical current to produce a
visible muscle contraction
o Functional electrical stimulation (FES) - if NMES is used to make a muscle contract
during a functional activity, it is termed FES. FES is commonly used at the anterior tibialis
muscle to increase dorsiflexion during ambulation
o Threshold electrical stimulation (TES) - a low-level electrical stimulus, often applied
during sleep that does not result in a visible muscle contraction. The proposed mechanism
of TES is that increasing blood flow during a time of heightened trophic hormone secretion
results in increased muscle bulk
4. Hypertonia Management
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 Chemical Denervation
o Botulinum Neurotoxin (BoNT) - muscles commonly treated with BoNT include
the gastrocsoleus complex, hamstrings, hip adductors, and flexor synergy muscles
of the upper extremity. Intramuscular injections can be localized by surface
landmarks, electromyographic guidance, and/or ultrasound. Following injection,
muscle relaxation is evident within 48 to 72 hours and persists for a period of 3 to
6 months. Dosing is dependent upon both body weight and size of the target
muscle(s). Injections are typically spaced a minimum of three months apart due to
concerns of antibody formation in an estimated 5% of patients, resulting in
potential resistance.
o Selective Dorsal Rhizotomy (SDR) - the selection of rootlets for cutting is based
on the lower extremity muscular response to electrical stimulation of the rootlets.
Ideal candidates for SDR include children between the ages of 3 and 8 years of age
who are GMFCS level III or IV.
 Oral Medications - are often used as an early treatment strategy for global spasticity.
Medications that are most frequently used include baclofen (Lioresal), dantrolene sodium
(Dantrium), clonidine, diazepam (Valium), and tizanidine (Zanaflex). All of these
medications work through the central nervous system, with the exception of dantrolene
sodium and, therefore, have the potential for sedation. None of these medications have
been found to be universally effective in relieving spasticity, and evidence related to
functional improvement is extremely sparse. The choice of medications is, therefore, often
based on the impact of potential side effects on the individual patient.
 Orthopedic Management
Orthopedic surgical management of persons with CP can differ depending on the
functional abilities of the individual, but share the common goal of maximizing quality of
life and function by addressing the secondary musculoskeletal complications that result.
Orthopedic surgical procedures in CP consist of a combination of muscle releases and
lengthenings, split tendon transfers, osteotomies, and arthrodesis. The timing of surgical
intervention is determined by CNS maturation, ambulation potential, and the rate at which
the deformity is developing. Physical therapy can help minimize the need for orthopedic
surgery, thereby reducing the number of surgeries a child may need.
 Orthoses
o Upper Extremity (UE) Orthoses - static wrist hand orthosis (WHO) are
commonly used in CP to improve hand position for functional activities and to
maintain range of motion. Dynamic WHO are much less commonly used because
children are often reluctant to use them for functional activities, in part due to the
decreased sensory feedback caused by the orthosis
o Lower Extremity (LE) Orthoses - many different types of LE orthoses are
utilized in the management of CP, including supramalleolar orthotics (SMOs),
solid ankle foot orthotics (AFOs), hinged AFOs, posterior spring-leaf AFOs, and
ground-reactive AFOs. Knee ankle foot orthoses and hip knee ankle foot orthoses
are rarely used in CP. Rotational-control orthoses, both twister cables and rotation
straps, are also used occasionally in children with cerebral palsy. Twister cables
have a pelvic band with attached cables of twisted spring steel, with torque
typically applied to provide an external rotation force by attaching to the shoes or
AFOs. Rotation straps are elastic and attach to buckles on AFOs or to an eyelet
attachment on shoestrings, and can provide internal or external rotation forces
depending on the application of wrapping the straps around the lower extremities.
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Saunders Company.
2. DeLisa, J. (2005). Rehabilitation Medicine: Principles and Practice 5th Ed. Gans, B.M., Robinson,
L.R. & Walsh, N.E. (Eds.). Philadelphia, PA: Lippincott Williams & Wilkins.
3. Molnar, G. (2010). Pediatric rehabilitation: Principles and Practice 4th Ed. Alexander, M.A.,
Matthews, D.J. (Eds.). New York, NY: Demos Medical Publishing.
4. Tecklin, J. (2015). Pediatric Physical Therapy 5th Ed. Philadelphia, PA: Lippincott Williams &
Wilkins.
5. Levitt, S. (2010). Treatment of Cerebral Palsy and Motor Delay 5th Ed. Malden, MA: Wiley-
Blackwell.