CEREBRAL PALSY

DEFINITION

Cerebral Palsy is a STATIC encephalopathy and may

be defined as a non-progressive disorder of posture

and movement, often associated with epilepsy and

abnormality of speech, vision and intellect resulting

from a defrct or lesion of the developing brain.

MAGNITUDE OF THE PROBLEM

• Cerebral palsy is the most common cause of chronic motor

disability that begins in childhood.

• It is difficult to estimate the precise magnitude of the problem

since milder cases are likely to be missed.

• It is the most common childhood physical disability

and affects 2 to 2.5 children per 1,000 born in the

United States.

• The exact incidence and prevalence from our country

is not known.

ETIOLOGY

Idiopathic (no known cause of damage to brain during pregnancy) - is the

most common cause.

CP could be classified on the basis of underlying cause of cerebral palsy-

Prenatal causes

Perinatal causes

Postnatal causes

PRENATAL CAUSES

PREMATURITY

LBW

MATERNAL EPILEPSY

TORCH INFECTIONS

BLEEDING IN THE 3RD TRIMESTER

INCOMPETENT CERVIX

SEVERE TOXEMIA, ECLAMPSIA

HYPERTHYROIDISM

DRUG ABUSE

TRAUMA

MULTIPLE PREGNANCIES

PLACENTAL INSUFFICIENCY

PERINATAL CAUSES

PROLONGED AND DIFFICULT LABOR

PROM

PRESENTATION ANOMALIES

VAGINAL BLEEDING AT THE TIME OF ADMISSION FOR

LABOR

BRADYCARDIA

HYPOXIA

PREMATURITY

KERNICTERUS

SEPSIS

CNS INFECTIONS

POSTNATAL

CNS INFECTIONS (MENINGITIS, ENCEPHALITIS)HYPOXIASEIZURESCOAGULOPATHIESNEONATAL HYPERBILIRUBINEMIAHead trauma from shaken baby syndrome (child abuse)

during the 1ST year of lifeLead poisoning during the 1st two years of life

PHYSIOLOGICAL CLASSIFICATION

Physiological classification

This is determined on the basis of the nature of the

movement disorder, muscle tone, and anatomic

distribution.

A single patient may have more than one

type. Spastic CP is the most common, accounting for

about two thirds of cases.

Pyramidal (or spastic) CP: This type is characterized

by neurologic signs of upper motor neuron damage

with increased "clasp knife" muscle tone, increased

deep tendon reflexes, pathologic reflexes, and spastic

weakness.

Extrapyramidal (nonspastic or dyskinetic) CP: This

type is characterized by prominent involuntary

movements or fluctuating muscle tone, with

choreoathetosis as the most common subtype.

Distribution is usually symmetric among the four limbs.

• Hypotonic CP: This type manifests as generalized

muscle hypotonia that persists with normal or increased

deep tendon reflexes. Many patients with this condition

develop cerebellar deficits of incoordination and ataxia,

and about one third of patients have severe retardation.

• Ataxic CP: Primarily cerebellar signs are seen.

• Mixed types

TOPOGRAPHICAL CLASSIFICATION

This is based on which limbs are involved.

• Monoplegia – Only one limb is involved.

• Paraplegia – Both the legs are involved

• Hemiplegia – Both upper and lower limbs are

involved on one side

• Triplegia – Three limbs are involved

• Quadriplegia – all the four limbs are involved

• Diplegia – The lower limbs are more involved

than the upper limbs.

GROSS MOTOR FUNCTIONAL CLASSIFICATION SYSTEM (GMFCS)

EARLY SIGNS OF CEREBRAL PALSY

Paucity of movement or excessive

& disorganized movements

Stereotyped behaviour

Persistent tone abnormalities

Feeding problems

Hyperextension of head & neck

Consistent fisting of hands

beyond 2 months

Delayed social smile

Reduced growth of head size

Early sign of hand preference in

the 1st yr of life

Persistent moro’s reflex beyond

6months of age

ETIOLOGY

SPASTIC DIPLEGIA

• Prematurity

• ischemia

• infection

• hemorrhages

• LBW

Neuropathology:

• periventricular leucomalacia

SPASTIC QUADRIPLEGIA

• Ischaemia

• Infections

• genetic/developmental

• birth asphyxia / trauma

Neuropathology :

• PVL

• multicystic malformation

HEMIPLEGIA• Thrombophilic disorders, • infections, PVH

• Neuropathology: stroke in utero or neonatal

EXTRA PYRAMIDAL• Kernicterus,• mitochondrial, genetic/

metabolic

• neuropathology: basal ganglia, putamen ,globus pallidus , thalamus

SPASTIC HEMIPLEGIA

SPASTIC HEMIPLEGIA

• Involvement of the arm and leg on one side of the body

• Upper extremity more severly involved

• Constitutes 20% of cases with spastic CP

• Manifests from 4th month – 2nd yr of life

• Incomplete Moro’s or Premature development of

handedness could be early symptom

• M/C form of CP

• Functional prognosis good as compared to other forms

• Underdevelopment of affected side is common

• Will crawl with affected arm tucked under the trunk and pushing self with the sound arm

• Cortical thumb

DIPLEGIA

DIPLEGIA

• Patients with diplegia have motor abnormalities in all four extremities, with the lower extremities more affected than the upper.

• The close proximity of the lower extremity tracts to the ventricles most likely explains the more frequent involvement of the lower extremities with periventricular lesions.

• The child uses the arms in a normal

reciprocal fashion but tends to drag

the legs behind more as a rudder

(commando crawl) rather than using

the normal four-limbed crawling

movement.

• If the spasticity is severe, application

of a diaper is difficult because of the

excessive adduction of the hips.

QUADRIPLEGIA

SPASTIC QUADRIPLEGIA

• Spastic quadriplegia is the most severe form of CP because of marked

motor impairment of all extremities and the high association with

mental retardation and seizures.

• In quadriplegia, all four extremities are equally involved, and many

patients have significant cognitive deficiencies that make care more

difficult.

• Head and neck control are usually present, which helps with

communication, education, and seating.

ATHETOID CP

• Athetoid cerebral palsy is less common than spastic cerebral palsy.

• This type is most likely to be associated with birth asphyxia.

• It is caused by an injury to the extrapyramidal tracts

and is characterized by dyskinetic, purposeless

movements that may be exacerbated by

environmental stimulation.

• The clinical picture varies based on the level of

excitement of the patient. In pure athetoid cerebral

palsy, joint contractures are uncommon.

• Affected infants are characteristically hypotonic with poor head

control and marked head lag and develop increased variable tone

with rigidity and dystonia over several years.

• Feeding may be difficult, and tongue thrust and drooling may be

prominent.

• Speech is typically affected because the oropharyngeal muscles are

involved.

• Seizures are uncommon, and intellect is preserved in many patients.

DYSTONIC CP

• The trunk muscles and the proximal portion of the limb

muscles are predominantly affected.

• Movements may be slow and persistent.

• Abnormal movements invoving the head and neck area.

ATONIC CP

• Hypotonicity of lower limb muscles.

• Arms may have near normal strength and coordination.

• Often associated with slow attainment of motor

milestones.

MIXED CP

• Manifestations of both spastic and extrapyramidal types.

• Often an ataxic component is present.

• Have mild to moderate degree of choreoathetosis.

DIAGNOSTIC CRITERIA FOR CEREBRAL PALSY

Cerebral palsy can be diagnosed using Levine (POSTER) criteria. POSTER stands for – P - Posturing/abnormal movements O - Oropharyngeal problems (e.g., tongue thrusts, swallowing

abnormalities) S - Strabismus T - Tone (hyper- or hypotonia) E - Evolutional maldevelopment (primitive reflexes persist or

protective equilibrium reflexes fail to develop R - Reflexes (increased deep tendon reflexes/persistent

Babinski's reflex)

• In a child with cerebral palsy motor deficit is always present.

• The usual presenting complaint is that child is not reaching motor milestones at the appropriate age.

• Medical history establishes that the child is not losing function.

• This history, combined with a neurologic examination establishing that motor deficit is due to a cerebral abnormality, establishes the diagnosis of cerebral palsy.

• Serial examinations may be necessary if the history

is unreliable.

• Other causes of neonatal hypotonia include muscular

dystrophies, spinal muscular atrophy, Down's

syndrome, spinal cord injuries.

Laboratory tests are not necessary to establish the

diagnosis. Workup is helpful for assessment of recurrence

risk, implementation of prevention programs, and medico

legal purposes.

Metabolic and genetic testing should be considered if on

follow-up the child has -

• Evidence of deterioration or episodes of metabolic

decompensation

• No etiology determined by neuroimaging

• Family history of childhood neurologic disorder associated

with cerebral palsy

• Developmental malformation on neuroimaging

• An EEG should be obtained when a child with cerebral

palsy has a history suggestive of seizures.

• Children with cerebral palsy should be screened for

ophthalmologic and hearing impairments, speech and

language disorders.

• Nutrition, growth, and swallowing function should be

monitored.

• Neuroimaging is recommended if the etiology has not

been established previously; for example, by

perinatal imaging.

• MRI, when available, is preferred to CT scanning

because of higher yield in suggesting an etiology, and

timing of the insult leading to cerebral palsy.

What other conditions are associated with cerebral palsy?

Neurological problems associated with cerebral palsy include:

• Seizures and epilepsy ( As many as half of all children with CP have one or more seizures.)

• Behavior problems

• Mental retardation ( Mental impairment is more common among those with spastic quadriplegia than in those with other types of cerebral palsy.)

• Learning disabilities

• Hearing loss

• Attention deficit–hyperactivity disorder

• Speech impairment

• Swallowing difficulties

• Hydrocephalus

Visual impairment As many as 75 to 90 percent of children with CP may suffer from-

• strabismus,

• Amblyopia (“lazy eye”),

• optic atrophy

• nystagmus

• visual field defects

• refractive errors

The secondary effects of cerebral palsy include:

• Poor growth

• Sleep disorders

• Poor nutrition

• Upper airway obstruction

• Aspiration pneumonia

• Communication disorder

• Gastroesophageal reflux

• Tooth decay and gum disease

• Hernia

• Constipation

• Bladder control problems

• Drooling

• Spinal deformities and osteoarthritis (Spinal deformities can make sitting, standing, and walking difficult and cause chronic back pain. Pressure on and misalignment of the joints may result in osteoporosis)

• Contractures.  Muscles can become painfully fixed into abnormal positions, called contractures, which can increase muscle spasticity and joint deformities in people with CP.

• Frequent fractures

MANAGEMENT

A LARGE AND EXTENSIVE CARE TEAMSURROUNDS THE FAMILY WITH A CHILD WHO HAS CEREBRAL PALSY.

• These care providers are roughly organized around the educational system, primary medical care provider, the cerebral palsy specialized medical team, and community support services.

Significant overlap and good communication provide the best resources to the child and the family.

• Because of the heterogeneous nature of cerebral palsy, it is difficult to make generalized statements regarding treatment, and it is best to have an individualized approach to each patient and his or her needs.

Four basic treatment principles exist -

• The first is that although the central nervous system injury, by definition, is nonprogressive, the deformities caused by abnormal muscle forces and contractures are progressive.

• The second, which can be a source of frustration, is that the treatments currently available correct the secondary deformities only and not the primary problem, which is the brain injury.

• The third is that the deformities typically become worse during times of rapid growth. For some patients, it may be beneficial to delay surgery until after a significant growth spurt to decrease the risk of recurrence.

• The fourth is that operative or nonoperative treatment should be done to minimize the impact it has on the patient's socialization and education.

HOME MODIFICATIONS

There are various ways to modify the home environment so that your child will have more independence and things will be easier on parents too. Example-

• Replace doorknobs with lever door handles.• Replace entrance steps with ramps.• Install a hinged arm support for help with toileting.• Rearrange furniture in order to remove obstructions.

CHOOSING APPROPRIATE SEATING

Strollers, feeding chairs, tumble form sets, headrests,

seating supports, and corner chairs—all of these are

forms of adaptive seating that may benefit children with

cerebral palsy.

PRESSURE MANAGEMENT AWARENESS

For children who use wheelchairs, the need to change

positions or relieve pressure, particularly on their

buttocks or ischial tuberosities, is critical. If is not

relieved, skin breakdown or pressure sores can occur.

If the child is not standing between 18 and 24 months, it is

necessary to start a child standing even when she does not

have adequate head or upper body control to stand alone.

Standing is important because

it allows the child to do some

weight bearing through the

legs, which in turn helps make

the bones stronger and

stimulates the development of

motor coordination and head

control.

WALKERS AND GAIT TRAINERS

• There are no absolute indicators for initiating the use of a walker.

• If child is able to hold up head independently, sitting in a chair with minimal support, and being able to stand and accept weight through the legs are a few of the basics that are needed.

REHABILITATION TECHNIQUES

Neurodevelopmental Therapy

• These specialized techniques encourage active use of

appropriate muscles and diminish involvement of muscles

not necessary for the completion of a task.

• Improvements in efficiency can include decreased

energy used during a task, decreased work required

of the muscles during a task, and habituation of new

patterns of movement.

• Occupational, speech, and physical therapists as well

as educators can use NDT.

STRENGTHENING EXERCISES

Muscle strength is related to motor performance and should

be an integral part of a rehabilitation program that addresses

other impairments which inhibit motor performance in this

population, such as muscle–tendon shortening, spasticity,

and coordination deficits.

ELECTRICAL STIMULATION TECHNIQUES

• Neuromuscular electrical stimulation (NMES)

• Transcutaneous electrical stimulation (TES)

HIPPOTHERAPY

The North American Riding for the

Handicapped Association (NARHA) has

defined hippotherapy as “The use of the

movement of a horse as a tool by

therapists to address impairments,

functional limitations and disabilities in

patients with neuro musculoskeletal

dysfunction.

AQUATIC THERAPY

• The relief of hypertonus in the spastic type of CP is one of the major advantages of aquatic therapy.

• When a body is immersed in warm water (92°to 96°F), its core temperature increases, causing reduction in gamma fiber activity, which in turn reduces muscle spindle activity, facilitating muscle relaxation and reducing spasticity, thus resulting in increased joint range of motion and consequently creating better postural alignment.

A great way to start gait

training, especially after

surgical procedures, is pool

walking. This means the pool

needs to have handles

available in the water at the

correct height.

M.O.V.E. (MOBILITY OPPORTUNITIES VIA EDUCATION) CURRICULUM

• It is an activity-based curriculum designed to teach individuals basic functional motor skills needed for adult life.

• The MOVE curriculum provides a framework for teaching the skills necessary for individuals with disabilities to gain greater physical independence.

• Physical and occupational therapies are useful for promoting mobility and the use of the upper extremities for activities of daily living.

• Speech language pathologists promote acquisition of a functional means of communications.

• 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.

MEDICAL MANAGEMENT

• Several oral medications have been used to reduce tone, including

diazepam, baclofen, dantrolene, tizanidine, and clonidine.

• Although they can decrease spasticity, their sedating side effects

are not well tolerated in Children.

• Intrathecal baclofen has been shown to reduce spasticity with

fewer side effects.

INTRATHECAL BACLOFEN PUMPS

• Use of intrathecal baclofen in the

pediatric patient having CP has

yielded as good a reduction in

tone as dorsal rhizotomy and does

not represent an ablative

procedure. This is important

because, unlike rhizotomies, it is

entirely reversible.

BACLOFEN

• GABA agonist -Acts on spinal cord neurons

• inhibits release of excitatory neurotransmitter at level of spinal cord

• Dose : 2 mg/kg/day, can be given intrathecally

• ADR : hypotonia of trunk muscles, dec alertness, dec appetite, vomiting, aggravates epilepsy

• Withdrawal: Rebound spasticity

BOTULINUM TOXIN A (BTA)• Blocks acetylcholine release, with a loss of motor end

plates

• Effect – 3 to 4 months

• Multiple site intramuscular injections for selected

muscle groups

• Effective only for dynamic spasticity

• Helps delay surgery beyond 8 yrs of age

• ADR : painful injection

• systemic effects- laryngeal weakness, bladder

incontinence

SURGICAL TECHNIQUES

Upper Extremity Procedures

1. Shoulder Adductor, Extension, and External Rotator Lengthening

2. Humeral Derotation Osteotomy (in patients with severe abduction external rotation contractures)

3. Elbow Flexion Contracture Release

4. Pronator Release or Transfer

5. Flexor Carpi Ulnaris Transfer for Wrist Flexion Deformity

6. Proximal Row Carpectomy and/or Wrist Fusion

7. Thumb Adductor Lengthening

A rhizotomy procedure in which the roots of the spinal nerves are divided produces considerable improvement in selected patients with severe spastic diplegia.

SPINAL PROCEDURES

1. Posterior Spinal Fusion with Unit Rod2. Anterior Spinal Release

• Hip Procedures- Iliopsoas Lengthening, Adductor and Iliopsoas Lengthening with Proximal Hamstring Lengthening.

• Knee Operative Procedures- Hamstring Lengthening

• Foot and Ankle Procedures- Gastrocnemius Lengthening, Tendon Achilles Lengthening

MANAGEMENT OF ASSOCIATED CONDITIONS

Strabismus is normally treated by correcting the visual

acuity in each eye, either with glasses or by patching. If a

significant strabismus remains even after these

therapies, then surgery is indicated.

Feeding –Problems: Tongue thrusting

Drooling of saliva Risk of aspiration

GERD

Mgt: Upright position during and after feeds Thickening of feeds Drugs - antacids, prokinetics, H2 blockers NGT feeds Antireflux surgery – fundoplication

For sialorrhea - glycopyrrolate

• Constipation –Risk of acquired megacolon

Increased fluid intake, varied diet, lactulose, enema

• Incontinence –Patient toilet training may be attempted according to stage of development