cerebral palsy in children. pediatrics

3. Definition
 Cerebral palsy (CP) is defined as “a group of disorders
of the development of movement and posture, causing
activity limitations that are attributed to non-
progressive disturbances that occurred in the
developing fetal or infant brain”

4. Cont.
 There are three major criteria for diagnosis of cerebral
palsy:
 A neuromotor control deficit that alters movement
or posture,
 A static brain lesion,
 Acquisition of the brain injury either before birth or in
the first years of life.

5. Etiology
 Not well understood.
 Factors that may contribute to brain injury and CP
include
 Prematurity,
 Infection,
 Inflammation,
 Coagulopathy.
 There is contributory roles of various biomolecules
and cytokines that accompany infectious or
inflammatory processes.

6. Etiology:-
 Prematurity and low birth wt.
 Stroke.
 Neonatal encephalopathy (with significant neurologic dysfunction,
including respiratory difficulties, altered tone, low consciousness, or
seizure activity)
 Maternal risk factors (chorioamnionitis or fever during labor,
coagulopathy or bleeding, placental infarction, and thyroid disease)
 Postnatal risk factors (social disadvantage, and include trauma in
developed nations infection in developing nations).
 Kernicterus (Rare kind of preventable brain damage in newborns with
jaundice).
 Methyl mercury exposure.
 Genetic.

7. Etiology
 Severe birth asphyxia .
 Term infants described as having birth asphyxia often
manifest certain signs, including acidosis, bradycardia,
or neonatal encephalopathy.
 Intrauterine exposure to infection.
 Birth asphyxia by itself accounts for a small minority of
cases of CP.

8. CLASSIFICATION
According to anatomic distribution:-
 Hemiparesis,
 Diparesis,
 Quadriparesis
Acording to movement:-
 Spastic,
 Dyskinetic,
 Hypotonic,
 Ataxic, and mixed forms.

10. Spasticity
 Spasticity was defined as hypertonia in which one or
both of the following signs are present:
a) Resistance to externally imposed movement
increases with increasing speed of stretch and varies
with the direction of joint movement, and/or
b) Resistance to externally imposed movement rising
rapidly above a threshold speed or joint angle.

11. Dystonia
 Dystonia was defined as a movement disorder in which
involuntary sustained or intermittent muscle
contractions cause twisting and repetitive movements,
abnormal postures, or both.

12. Hypotonic
 Hypotonia is diminished muscle tone. The infant or
child with hypotonic cerebral palsy appears floppy -
- like a rag doll.
 In early infancy, hypotonic can be easily seen by the
inability of the infant to gain any head control when
pulled by the arms to a sitting position (this symptom
is often referred to as head lag)
 Hypotonic forms of CP are rare

13. Ataxia
 Ataxia is a neurological sign consisting of lack of
voluntary coordination of muscle movements that
includes gait abnormality.
 Dysfunction of the parts of the nervous system that
coordinate movement, such as the cerebellum.
 ataxic forms of CP are rare

14. 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.
 GMFCS) I children walk indoors and outdoors and climb stairs
without limitation.
 GMFCS II children walk indoors and outdoors and climb stairs
holding onto a railing but experience limitations walking on
uneven surfaces and inclines.
 GMFCS III children walk indoors or outdoors on a level surface
with an assistive mobility device. Children may climb stairs with
a railing or propel a manual wheelchair.
 GMFCS IV children may walk short distances with a device, but
rely more on wheeled mobility at home and in the community.
 GMFCS V children have no means of independent mobility.

16. Cont.
 A related classification system for upper extremity
function, the Manual Abilities Classification System,
permits categorization by fine motor performance.
 Ideally, each individual with CP will be classified in four
dimensions, including
1. Motor abnormalities,
2. Associated impairments,
3. Anatomical and radiological findings, and
4. Causation and timing of injury.

17. PATHOLOGY
 Neuroimaging.
 Common abnormality is white matter near the lateral
ventricles, often termed periventricular leukomalacia
(PVL).
 Intraventricular hemorrhage in premature infants.
 Corticospinal tract fibers to the lower extremities are
medial to those of the upper extremities in the
periventricular white matter, children with PVL
typically have spastic diparesis.

18. Cont.
 Deep grey matter lesions of basal ganglia and
thalamic region are associated with dystonic CP
(12% of children)
 Athetoid CP follows a diagnosis of kernicterus,
These cases are far less common with advancements
in the treatment of neonatal jaundice.
 Focal cortical infarcts involving both the grey and
white matter are found almost exclusively in patients
with hemiparesis, and are typically related to
middle cerebral artery strokes.

19. Cont.
 Brain malformations can be found on neuroimaging in
approximately 10% of children with CP.
 Neuronal migrational disorders early in pregnancy can
result in lissencephaly, polymicrogyria,
schizencephaly, or holoprosencephaly.
 Some in utero infections, such as those caused by
cytomegalovirus, can also cause distinctive brain
malformations.
 Brain malformations are more commonly found in
cases of term infants and hemiparesis.

20. Cont.
 Infection and ischemia are two of the more common
causes of generalized encephalomalacia.
 Findings present on magnetic resonance imaging
(MRI), including multiple cysts, cortical thinning,
white and grey matter loss, and microcephaly.
 Children with diffuse brain lesions or anomalies
typically demonstrate spastic quadriparesis and are
at high risk for additional medical and cognitive
problems.

21. INITIAL EVALUATION
AND CLINICAL FINDINGS
 Early identification of children who have CP allows for
early therapeutic intervention and screening for associated
conditions.
 CP is a descriptive term that does not infer a single etiology,
pathology, or prognosis, there is no specific diagnostic test.
 Diagnosis is 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.

22. Cont.
 As the cortex matures in the second half of the first
year, the diagnosis typically becomes more apparent.
 The first step in the evaluation for suspected CP is a
comprehensive history, including a detailed account of
potential risk factors and family history.
 A thorough history of developmental milestones is
also important.

23. Signs and Symptoms:-
 A significant delay in attaining motor milestones.
 Prematurity.
 A discrepancy between motor and cognitive milestones.
 Certain deviations in developmental milestones are associated
with CP. For example, early hand preference or asymmetric use
of the extremities may be the first indication of hemiparesis.
 Early head control, rolling, or rigid standing are all associated
with abnormally increased tone and/or exaggerated primitive
reflexes.
 Unusual means of mobility, such as bunny hopping, combat
crawling, or bottom scooting.
 loss any skills or milestones, thus can be neurodegenerative
disorder.

24. Cont.
 In infancy, the neurologic exam focuses on tone and infantile
developmental reflexes.
 Deep tendon reflexes, plantar responses, and the presence of
clonus are more informative in the older child.
 Tone should be assessed by gently moving the infant’s joints
through their appropriate range of motion and evaluating the
amount of resistance.
 Frog leg position.
 Persistent fisting or scissoring may be observed with increased
tone.
 Most infants will undergo an early stage of mild or moderate
hypotonia prior to more traditional signs of CP.
 A prolonged period of hypotonia or fluctuating tone is more
typical of dyskinetic CP.

25. Cont.
The earliest indication of CP may be
 A delay in the disappearance of primitive infantile reflexes
(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.

26. Imaging
 Neuroimaging can be helpful in determining the etiology
of CP and the timing of the insult.
 Neuroimaging can also be useful in determining an
etiology in children suspected of having CP outside of the
neonatal period.
Recommendations:
 Preterm neonate include a screening cranial
ultrasonography on all infants <30 weeks gestation
between 7 and 14 days of age and again between 36 and 40
weeks’ postmenstrual age.
 Evidence of grade 3 or 4 intraventricular hemorrhage,
periventricular cystic lesions, or moderate to severe
ventriculomegaly.

28. CONT.
 In term infants with neonatal encephalopathy, the
practice parameter recommends a non-contrast
computed tomography (CT) to detect hemorrhagic
lesions when there is a history of birth trauma, low
hematocrit, or coagulopathy.
 If the CT is inconclusive, MRI should be performed
between day of life 2 to 8 in order to assess the location
and extent of injury.
 Abnormalities of the thalamus and basal ganglia were
associated with increased neurodevelopmental
disability at 1 to 2 years of age.

29. Laboratory Findings
 Metabolic or genetic causes.

30. DIFFERENTIAL DIAGNOSIS
Young infants with CP often present with hypotonia. The
differential diagnosis for the floppy infant is vast. The most
common etiologies include:
 Central nervous system disorders such as CP,
 Neuromuscular disorders,
 Genetic disorders, and
 Metabolic disorders.
Clues to a neuromuscular disorder include
 Diminished deep tendon reflexes,
 Weakness (which may result in absent infantile reflexes),
 A positive family history.

31.  Metabolic disorders may present at any age, but are
most likely to present in infancy.
 Metabolic acidosis.
 Hypoglycemia.
 Hepatic involvement.
 Cardiac involvement.
 Mitochondrial disorders.
 Glutaric aciduria type I.
 Lesch-Nyhan syndrome,
 Homocystinuria.

32. ASSOCIATED DISORDERS
 Sensory Impairments
Deficits in
 Two-point discrimination,
 Proprioception, and
 Stereognosis.
 Sensory deficits are most common in children with
hemiparesis.

33.  Visual Impairments
 Strabismus
 Retinopathy of prematurity in premature infants,
 Cortical visual impairment in hypoxic ischemic
encephalopathy
 Homonymous hemianopsia in hemiparesis.
 Children with the most severe CP were at greatest risk
for high myopia, absence of binocular fusion,
dyskinetic strabismus, severe gaze dysfunction, and
optic neuropathy or cortical visual impairment.

34.  Hearing Impairments
 Relatively rare in CP.
 Sensorineural hearing loss is most commonly associated with
congenital TORCH (toxoplasmosis, rubella, cytomegalovirus, and
herpes) infections, bacterial meningitis, and ototoxic drugs.
 Cognitive Impairments
 Assessment of intellectual functioning can be difficult in patients with
severe motor and communication difficulties, which may lead to an
underestimation of cognitive function.
 Cognitive function can occur in patients who are socially responsive.
 Severe neuromuscular impairments greater risk for cognitive
impairments,
 Patients with severe motor impairments can have normal cognition.
 It is important to attempt an accurate assessment of intelligence in
order to assist in appropriate educational and vocational plans.

35. Cont.
 Psychological Impairments
 A wide variety of behavior and emotional disorders are
possible, including attention deficit disorder, passivity,
immaturity, anger, sadness, impulsivity, emotional lability,
low self-esteem, and anxiety.
 Children with CP were found to have fewer reciprocated
friendships, fewer sociable and leadership behaviors, and
were more isolated and victimized by their classmates
 Epilepsy
 Variety of types of seizures are possible (risk factors and
seizure frequency or type)

36.  Oromotor Impairments
 A weak suck, poor coordination of the swallowing mechanism,
tongue thrusting, and a tonic bite reflex may all lead to feeding
difficulties and increased risk for aspiration.
 Speech disorders range from mild articulation disorders to
anarthria
 Difficulty controlling oral secretions and drooling, which may
negatively affect social interactions.
 Dental malocclusion and difficulty with oral hygiene, leading to
an increased risk of periodontal disease.
 Nutritional Disorders
 The assessment of growth and nutrition in children with CP can
be difficult due to the lack of a reliable means of measuring
stature in children with contractures and scoliosis and the lack
of appropriate reference data or growth curves specific to CP.

37.  Genitourinary Disorders
 Urinary incontinence
 Incontinence was the most common complaint, but
frequency, urgency, hesitancy, and urinary retention
may also be present.
 Frequency and urgency are often associated with
spasticity of the detrusor muscle, causing small,
frequent voids.
 Detrusor overactivity and a small bladder capacity
were the most common findings on urodynamic
studies in children referred for voiding dysfunction

38.  Bone and Mineral Density Disorders
 Decreased bone mineral density (BMD) and increased risk
of fracture with minimal trauma is common in patients
with moderate to severe CP
 Respiratory Disorders
 Impaired control of respiratory muscles, ineffective cough,
and aspiration due to an impaired swallow;
gastroesophageal reflux; or seizures all increase the risk for
chronically increased airway secretions.
 Increased airway secretions may lead to wheezing,
atelectasis, recurrent aspiration pneumonia, restrictive
lung disease, or bronchiectasis.
 Bronchopulmonary dysplasia in an infant born
prematurely will also increase the risk for respiratory
disorders.

39. Musculoskeletal Disorders
 Foot/ankle
 Equinus deformity:
Increased tone or contractures of the gastrosoleus complex.
 Equinovarus foot deformity
Combination of spasticity of the posterior tibialis muscle and the
gastrocsoleus complex, resulting in inversion and supination of the foot and a
tight heel cord. It is common in a child with hemiparesis.
 Equinovalgus
It is due to spasticity of the gastrocsoleus complex and the peroneal
muscles, as well as weakness in the posterior tibialis muscle. It is common in
older children with spastic diparesis and quadriparesis.
 Hallux valgus deformities
Valgus deformities of the foot, which may lead to a painful bunion at the head
of the first metatarsal.

40.  Knee
 due to spasticity in the hamstring muscles and static
positioning in a seated position.
 If a severe knee flexion is present, hip flexion will be
limited, resulting in lumbar kyphosis in the seated
position.
 Flexion contractures at the knee are associated with
hip and ankle flexion contractures and patella alta.
 Genu valgus may also occur, and is most commonly
associated with excess femoral anteversion.

41.  Hip
 Acquired hip dysplasia is common in CP.
 Leads to progressive subluxation and possible dislocation.
 Hip subluxation can begin as early as age 2 years and
should be monitored closely by exam and serial
radiographs.
 On exam, passive hip abduction of less than 35 degrees and
a hip flexion contracture of more than 20 degrees
 Causative factors include persistent excessive femoral
anteversion, a dysplastic acetabulum, and muscle
imbalance from overactive hip adductors and flexors.
 These factors cause the hip to be adducted, flexed, and
internally rotated, placing it at risk for posterior
dislocation.

42.  CP may also develop a “windswept deformity” of
their hips.
 It’s an Adduction deformity of the elevated hip and an
abduction deformity of the opposite hip, which also
tends to be externally rotated and commonly results in
pelvic obliquity.
 The hip on the elevated side is at significant risk for
dislocation, and positioning can be challenging.
 Hip dislocation with pelvic obliquity is often
associated with scoliosis.

44.  Spine:
 Kyphosis:
 Is often seen in conjunction with significant weakness of the
spinal extensor muscles and tightness in the hamstrings, leading
to a posterior pelvic tilt.
 Lordosis:-
 Is frequently associated with hip flexion contractures.
 Scoliosis:-
 The likelihood of scoliosis increases with the severity of CP.
 Curves greater than 40 degrees tend to progress, regardless of the
patient’s skeletal maturity.
 The risk of progression is greatest for patients with
quadriparesis, increased spasticity, a larger curve, a younger age,
poor sitting balance, or pelvic obliquity.

45.  Upper extremity
 Spasticity and muscle imbalances can often lead to joint deformities in the
upper extremity.
 The shoulder is often positioned in an adducted and internally rotated
position.
 Spasticity in the biceps, brachioradialis, and the brachialis frequently
result in elbow flexion contractures.
 Elbow flexion contractures less than 30 degrees rarely have functional
significance.
 Forearm pronation deformities are common and can significantly affect
functional use of the hand.
 The most common deformity of the wrist is flexion, typically with ulnar
deviation.
 The most common finger deformities are flexion and swan neck
deformities due to hand intrinsic muscle spasticity.
 A thumb in palm deformity is commonly seen with adduction at the
carpometacarpal joint, which may be associated with hyperextension of
the metacarpophalangeal and interphalangeal joints.

46. Gait Impairments

47. TREATMENT
 General Principles
 The treatment of a child with CP requires a multidisciplinary approach.
 Once the diagnosis is made, the infant or child should be evaluated by a
comprehensive rehabilitation team.
 The members of this team will vary, depending upon site and availability.
 A Physiatrist,
 Developmental pediatrician,
 Orthopedist,
 Neurologist,
 Physical therapist,
 Occupational therapist,
 Speech and language pathologist,
 Therapeutic recreation specialist,
 Orthotist,
 Psychologist,
 Social worker, and
 A nutritionist.