CEREBRAL PALSYReported By:

FELVEE M. BASIBAS, PTRP MD

CEREBRAL PALSY

Three part definition: A disorder of movement and posture Caused by a non-progressive injury To the immature brain

Change in muscle tone and posture, both at rest and with voluntary activity.

First year or two of life – included in most definitions

Upper age limit of postneonatal brain insult- unclear

CP: EPIDEMIOLOGY

One of the most common disabling conditions affecting children.

1-2.3/1,000 live births Diagnosis is not made at any specific

age Can resolve in up to 50% of children

diagnosed prior to 2 years of age Or the brain insult might not occur

until later in childhood.

RISK FACTORS ASSOCIATED WITH CEREBRAL PALSY

GENERAL • Gestational age < 32 weeks• Birth weight <2500 g

MATERNAL HISTORY• Mental retardation• Seizure disorder• Hyperthyroidism• Two or more prior fetal deaths• Sibling with motor deficits

DURING GESTATION• Twin gestation Chorionitis • Fetal growth retardation Increased urine protein excretion • Third-trimester bleeding Low placental weight•Premature placental separation

FETAL FACTORS• Abnormal fetal presentation•Fetal malformations•Fetal bradycardia•Neonatal seizures

CP: ETIOLOGY

Brain injury can occur in the following periods:a. Prenatalb. Perinatalc. Postnatal

PRENATAL PERIOD- wherein most causes of CP occur. TORCH infections Intrauterine stroke Genetic malformations

The most common currently understood causes are related to brain injury occurring in children born prematurely.

CP: ETIOLOGY

Immature Fragile brain musculature

Physical stresses of prematurity

Compromised cerebral blood flow( blood vessels in the water shed zone next to lateral ventricles in the capillaries of the

germinal matrix)

INTRAVENTRICULAR HEMORRHAGE

Bleeding is arterial in origin Transfontanelle cerebral high-resolution UTZ

Grades of Intraventricular Hemorrhage in the Premature Brain

GRADE HEMORRHAGE

1 Isolated to germinal matrix

2 With normal ventricular size

3 With ventricular dilatation

4 With parenchymal hemorrhage

Very LBW infants – increased risk of periventricular hemorrhagic infarction

Periventricular hemorrhagic infarction Hemorrhagic necrosis lateral to the external angle of

the lateral ventricle. Of venous origin Usually asymmetric With healing – Periventricular leukomalacia ( PVL)

can develop. PVL

One of the strongest predictors of CP in the premature neonate.

Almost always associated with a history of prematurity.

PVL

Extremely LBW infants:Are also at increased

risk of CP.Especially with a history

of resuscitation and prolonged ventilation.

Protective factors: prenatal care and steroids.

CP IN TERM INFANTS

Almost ½ of children with CP were born term.

Cause of brain injury is often elusive. Severe anoxic/ischemic brain injury:

Mechanical difficulties of the placentaUmbilical cordActual delivery itself

Intrapartum asphyxiaMust be severe and prolonged to cause

CP.More global and more severe disability.

ETIOLOGY: ATHETOID CP

Injury to the basal ganglia.

Hyperbilirubinemia. Hearing loss. Incidence has declined

since testing and treatment for Rh incompatibility

Now, relatively rare

ETIOLOGY: POSTNATAL CEREBRAL INJURY AND CP

Major causes:CNS infectionsVascular causesHead injury

Other Causes:AnoxiaIschemiaInflammation

CLASSIFICATION OF CP

CLASSIFICATION of CEREBRAL PALSY TYPES

By TONE ABNORMALITIES By BODY PARTS INVOLVED

a.Spasticb.Dyskinetic (Athetoid,

Choreiform, Ballistic, Ataxic)

c.Hypotonicd.Mixed

a.Diplegiab.Quadriplegiac.Triplegiad.Hemiplegia

GROSS MOTOR FUNCTIONAL CLASSIFICATION

LEVEL 1 Walks without restriction, Limitations in high-level skills

LEVEL 2 Walks without devices, Limitations in walking outdoors

LEVEL 3 Walks with devices, Limitations walking outdoors

LEVEL 4 Limited mobility, Power mobility outdoors

LEVEL 5 Very limited self-mobility, even with assistive technology

SPASTIC SUBTYPE Most common 75% of children with CP Spasticity:

Velocity-dependent increased toneAssociated with UMN syndrome

findings Increased muscle stretch reflexesBabinski responseWeaknessDifficulty with coordination.

Can be associated with extensor or flexor posturing ( decerebrate and decorticate posturing)

DYSTONIA A dyskinetic tone abnormality With alternating tone or cocontraction in the

antagonist and agonist muscle groups Causing varied abnormal postures and often

fluctuating tone. Other dyskinetic forms:

1. Athetosis2. Choreiform3. Choreoathethoid

ATHETOSIS

Involuntary constant rotatory or writhing movements of the distal extremities.

Basal ganglia involvement.

Stop during sleep. Incidence has been

reduced by the advent of treatment for Rh incompatibility.

ATAXIC TYPE

Rare Must be

differentiated from degenerative processes of the cerebellum.

MIXED TYPE

Spasticity + Dystonia

HYPOTONIC TYPE

Needs to be differentiated from those with identifiable causes of neonatal hypotonia:Muscle diseaseMetabolic disordersGenetic syndromes

Many of these children develop spastic or extrapyramidal-type disorders after the first few months of life.

CLINICAL EFFECTS

a. Hypotonia and motor delay- often seen as early signs of CP.

b. UMN injury features Positive findings

Increased tone and reflexes

Positive Babinski’s reflex

Negative findings Reduced strength Selective motor control Balance and

coordination.

c. Tone in the extremities

d. Retention of Primitive Reflexes

e. Irritabilityf. Lethargyg. Weak suck with

tongue thrusth. Poor head controli. High-pitched cryj. Oral hypersensitivityk. Tonic bitel. Asymmetric

movementsm. Unusual posturing

n. Rolling for mobilityo. Combat crawlingp. W sittingq. Bunny hopping r. Adopting a hand

preference before the first birthday

s. Trunk or central hypotonia

t. Muscle weaknessu. Abnormal posturingv. Sensory deficits

Contractures Deformities Kyphosis Scoliosis Respiratory

compromise Atlantoaxial

instability Foot deformities

ASSOCIATED MEDICAL AND FUNCTIONAL PROBLEMS

a. Visual Sequelae Strabismus Exotropia/ Esotropia Hemianopsia Retinopathy of prematurity

b. Hearing impairment Ototoxic drug exposure Hyperbilirubinemia TORCH infections in utero

c. Abnormalities of oral motor function Due to weakness and incoordination

of lips, tongue and masticatory and facial muscles.

Drooling, dysphagia and dysarthria Treatments:

Behavioral techniques Speech therapy Anticholinergic medications Botulinium toxin A injections Surgical redirection of the salivary

ducts

d. Dysphagia A problem in the following:

Oral Phase (chewing and bolus preparation)

Laryngeal Phase (propulsion and airway protection problems)

Aspiration - ischemia

e. Undernutrition/ Malnutrition 1/3 of patients with Hemiplegia and

Diplegia (undernourished) > 2/3 of patients with Quadriplegia

(undernourished) 27% of patients – malnourished Treatment:

Gastrostomy Gastrojejunostomy tube

f. GI Symptoms:1. Gastroesophageal reflux

Can result to episodic emesis Interferes with adequate nutrition

and growth Treatment:

a. Medicationsb. Surgical: Nissen fundoplication

or Jejunostomy

2. Chronic constipation Neuromuscular control of the bowel Exaggerated by immobility and

abnormal diet and fluid intake Anorectal manometry Complications:

Large bowel megacolon Volvulus

Treatment: Increase activity Increase fluid and fiber intake

medications

g. Urinary Symptoms: 1/3 of patients – frequency,

incontinence or difficulty urinating. Detrussor instability, vesicoureteric

reflux, DSD

h. Cognitive Impairments: 30% of patients- mental retardation Risk is directly proportional to

severity of motor disability. 20-30% - have specific learning

disabilities 40-50% of Normal BW CP- cognitive

abnormalities

i. Seizure Disorders 1/3 of children with CP Hemiplegic> Quadri > Diplegic Reflects a greater extent of cortical

brain injury Treatment:

Antiseizure techniques Grid mapping and resection of

seizure foci.

j. Osteoporosis Secondary to the following factors:

Feeding difficulties – deficient Ca and Vit D Decreased weight bearing/ Immobilization Muscle stresses Antiseizure meds Weight percentile/ Low triceps skinfold

Treatment: Ca and Vit D supplementation Bisphosphonates (Pamindronate)

Bone age was not found to be significantly different than chronologic age in children with CP

k. Pain Can go undiagnosed and untreated

in patients who are non-verbal. Increased in patients in GMFCS

Levels 3-5 Frequency is correlated with the

following: Severity of motor impairment Presence of gastrostomy Days of schools missed Days spent in bed

l. Mortality Risk factors:

Lack of independent mobility such as rolling

Use of tracheostomy

Lack of any hand function

Prevention: Teach basic life skills (feeding and mobility)

Predictors of survival:Cognitive levelAbility to speakAbility to recognize voices

Ability to interact with persons

Physical activity and mobility

Tube feedingIncontinenceSeizures

m. MorbidityInjuries, abuse and neglect

FUNCTIONAL PROGNOSIS

FUNCTIONAL PROGNOSIS

Children typically develop motor skills craniocaudally.

The age at which these skills are developed help to predict the eventual outcome.

75% of spastic CP eventually ambulate 85% in diplegics 70% in quadriplegics

PROGNOSIS FOR AMBULATION

Hemiplegics/ Ataxic pxs

Achievement of all motor skills by age of 8.

Independent sitting before 2 years

Persistence of fewer than 3 of the primitive reflexes at age 18 months.

Quadriplegics Did not attain

independent sitting by age 4.

Persistence of primitive reflexes beyond 18 months

GOOD Prognosis POOR Prognosis

CHARACTER

Inefficient gaitShorter step lengthDecreased ROM of the

hips and knees Increased energy

expenditure Decreased velocity Decreased endurance

 

OUTCOME MEASURES

OUTCOME MEASURES FOR CEREBRAL PALSY REHABILITATION

OUTCOME OF INTEREST

MEASURE

SPASTICITY Ashworth ScoreTardieu angles

RANGE of MOTION Goniometer

DYSTONIA Barry Albright Dystonia Scale

SRENGTH Medical Research Council Muscle Grade (0-5)Modified SphygmomanometerHydraulic Strength or TorqueMaximun 10 Repetitions Weight Lift

1.      No increase in muscle tone.

2.      Slight increase in tone giving a “catch” when affected part is moved in flexion or extension.

3.      More marked increase in tone but affected part is easily flexed.

4.      Considerable increase in tone; passive movement difficult.

5.      Affected part is rigid in flexion or extension.

0. No increase in muscle tone

1. Slight increase in tone with a catch and release or minimal resistance at end of range

2. As 2 but with minimal resistance through range following catch

3. More marked increase tone through ROM

4. Considerable increase in tone, passive movement difficult.

5. Affected part rigid

Ashworth Scale The Modified Ashworth scale

OUTCOME MEASURES FOR CEREBRAL PALSY REHABILITATION

OUTCOME OF INTEREST

MEASURE

COGNITION IQ

SPEECH Intelligibility

HEALTH Short Form 12 or 36

SOCIAL AND SELF-CARE SKILLS

WeeFIM, Pediatric Outcomes Data CollectionInstrumentCanadian Occupational Performance Measure

PAIN Faces Pain Scale Non-Verbal Pain Scale

OUTCOME MEASURES FOR CEREBRAL PALSY REHABILITATION

OUTCOME OF INTEREST

MEASURE

COMMUNITY INTEGRATION

Child Health QuestionnaireDemographics, Craig Handicap Assessment and Reporting Technique

HAND and ARM FUNCTION

Melbourne Upper Extremity TestQuality of Upper Extremity Skills Test

GROSS MOTOR FUNCTION

Gross Motor Function MeasurePeabody Scales of Infant Development

GAIT Velocity, stride length, balanceOxygen costKinetics or kinematics on gait analysis

THERAPEUTIC MANAGEMENT

GOALS OF REHABILITATION (MOLNAR)

1. To decrease complications of CP.2. To enhance or improve acquisition of

new skills.

ADDITIONAL GOALS by ROTHERY and Colleagues:

1. Parent and caregiver education2. Decrease skeletal deformity3. Improve mobility

EARLY INTERVENTION

“ Early institution of physical therapy can decrease the impact of the brain injury in the development of CP”. – Kong

The rationale for early intervention is closely connected to concepts that stress the importance of the early years for normally developing children, and the role that environmental factors play in the development.

2 Main models for the delivery of Developmental Early Intervention:1. Direct Therapy Service Model2. Consultation Model

THERAPY APPROACHES

I. Integrated Therapy Programs in SchoolII. Caregiver Participation/ Home TherapyIII. Physical TherapyIV. Individual Therapy Programs

Speech Therapy Occupational Therapy

V. Conductive EducationVI. Constraint-induced or Forced Use

TherapyVII. FES and BiofeedbackVIII.TES

CONDUCTIVE EDUCATION

Intervention that melds educational theory and PT.

A mode of therapy that integrates rhythmic movement and activities in a group setting, with a class leader.

Improvement in functional skills such as toileting for children in conductive education programs versus those in regular school therapy.

CONSTRAINT-INDUCED OR FORCED USE THERAPY

Has promise and supports the theory of motor plasticity.

Restrains the sound limb to increase the use of the affected side.

Gains in the areas of motor performance were maintained up to 6 months post intensive training in one study.

THERAPEUTIC ELECTRICAL STIMULATION

Use of low-voltage, high frequency ES. Increase blood flow and improve

muscle growth and strength.

MEDICAL AND SURGICAL MANAGEMENT

MANAGEMENT OF HYPERTONIA

Oral Medicationsa. Baclofenb. Dantrolenec. Diazepam

Specific Medications for Dystonia:a. Trihexyphenidyl HClb. Levodopa-carbidopa (Sinemet)

Botox Injections Phenol Injection

Reappraisal of spasticity or hypertonia in a growing child with CP every 6 months is necessary.

BOTULINIUM TOXIN TYPE A (BOTOX) Effective in improving ROM and reducing tone. Also effective in improving motor control. Best results: Botox + Serial casting Indications for Use of Botox:

a. Calf injection for dynamic equinus persistent throughout the gait cycle

b. Hamstring injection for dynamic knee flexionc. Adductor injection for scissoringd. Diagnostic measures before surgerye. Management of focal limb dystoniaf. Analgesia for pain and spasm in the perioperative

periodg. In the UE, persistent thumb in palm, wrist posture

preventing hand use, and elbow flexion.

NEUROSURGICAL INTERVENTIONS

I. Selective Dorsal Rhizotomy (SDR)II. Intrathecal Baclofen (ITB) PumpIII. Stereotactic ablation of selected

thalamic nucleiIV. Chronic ES of the Cerebellum or

Posterior Columns Has shown promise in adults with

dystonia.

SELECTIVE DORSAL RHIZOTOMY (SDR)

Since 1980’s Reduces spasticity by interrupting the sensory

input in the dorsal horn. Ideal Candidates for SDR:

1. Premature child with spastic diplegia.2. Good balance3. Good selective motor skills4. Aged 4 or 5 years5. With minimal contactures6. Able to walk unassisted

Athetosis – contraindication for SDR Dystonia – can become more problematic post

SDR

Candidates for rhizotomy usually are already involved in an active physical therapy program.

Because the procedure involves intensive follow-up therapy, children who can understand and follow directions generally are ideal candidates.

Two groups of children who benefit from selective dorsal rhizotomy:1. Spastic Diplegics (Borderline

ambulators) The goals of surgery : better gait and

leg function2. Severe Spastic Quadriparetics

increase their independence by allowing them to sit for longer periods of time, use a potty seat, or power a wheelchair on their own.

INTRATHECAL BACLOFEN

In addition to spasticity reduction, it also has impact on dystonia.

Complications with ITB are as high as 50%.Improvements in technology

with catheters and pumps might reduce the mechanical risk.

Experience in management.Abrupt withdrawal in children

must be treated aggressively.

ORTHOPEDIC INTERVENTION

Nearly all children with CP develop an abnormality of physical form and/or function.

Children with CP should have regular orthopedic consultations.

If pain or discomfort is present, especially around the hip, surgical relief may be necessary.

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