Cerebral Palsy

                        

Cerebral Palsy

Cerebral palsy (CP) is a static encephalopathy

Definition - a nonprogressive disorder of posture and movement often associated with epilepsy and abnormalities of speech, vision, and intellect resulting from a defect or lesion of the developing brain.

prevalence of 2/1,000 population.

The condition was first described almost 150yr ago by Little, an orthopedic surgeon. The primary causes included birth trauma, asphyxia, and prematurity. During the past 2–3 decades, considerable advances have been made in obstetric and neonatal care, but there has been no change in the incidence of CP.

EPIDEMIOLOGY AND ETIOLOGY.

Prevalence of CP is 4/1,000 live births.

High in babies with increased risk for developing asphyxia during the perinatal period. One reason is intrapartum asphyxia.

Other - Intrauterine exposure to maternal infection (e.g., chorioamnionitis, inflammation of placental membranes, umbilical cord inflammation, foul-smelling amniotic fluid, maternal sepsis, temperature greater than 38°C during labor, and urinary tract infection) is associated with a significant increase in the risk of CP in normal birthweight infants.

High in low birth weight infants, particularly those weighing less than 1,000g at birth, primarily because of intracerebral hemorrhage and periventricular leukomalacia.

CLINICAL MANIFESTATIONS.

CP may be classified into

1. physiologic,
2. topographic,
3. etiologic categories
4. functional capacity

physiologic classification = the major motor abnormality,

topographic taxonomy = the involved extremities.

CP is also associated with developmental disabilities, =

1. mental retardation,
2. epilepsy,
3. visual,
4. hearing,
5. speech,
6. cognitive,
7. behavioral abnormalities.

Types of CP

Spastic hemiplegia

           

1. Decreased spontaneous movements on the affected side and show hand preference at a very early age.
2. The arm is often more involved than the leg and difficulty in hand manipulation is obvious by 1yr of age.
3. Walking is usually delayed until 18–24 mo, and a circumductive gait is apparent.
4. Examination of the extremities may show growth arrest, particularly in the hand and thumbnail, especially if the contra lateral parietal lobe is abnormal, because this area of the brain influences extremity growth.
5. Spasticity is apparent in the affected extremities, particularly the ankle, causing an equinovarus deformity of the foot. An affected child often walks on tiptoes because of the increased tone, and the affected upper extremity assumes a dystonic posture when the child runs.
6. Ankle clonus and a Babinski sign may be present, the deep tendon reflexes are increased, and weakness of the hand and foot dorsiflexors is evident. About one third of patients with spastic hemiplegia have a seizure disorder that usually develops during the first year or two, and approximately 25% have cognitive abnormalities including mental retardation.
7. A CT scan or MRI may show an atrophic cerebral hemisphere with a dilated lateral ventricle contralateral to the side of the affected extremities. Intrauterine thromboembolism with focal cerebral infarction may be one cause; CT or MRI at birth in infants with focal seizures often demonstrates the area of infarction. Spastic hemiplegia is more common than spastic diplegia in low birthweight infants.

Spastic diplegia

           

1. Bilateral Spasticity of the legs. The first indication of spastic diplegia is often noted when an affected infant begins to crawl. 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.
2. If the Spasticity is severe, application of a diaper is difficult owing to excessive adduction of the hips.
3. Examination of the child reveals Spasticity in the legs with brisk reflexes, ankle clonus, and a bilateral Babinski sign. When the child is suspended by the axillae, a scissoring posture of the lower extremities is maintained.
4. Walking is significantly delayed; the feet are held in a position of equinovarus; and the child walks on tiptoes.
5. Severe spastic diplegia is characterized by disuse atrophy and impaired growth of the lower extremities and by disproportionate growth with normal development of the upper torso.
6. The prognosis for normal intellectual development is excellent for these patients, and the likelihood of seizures is minimal.
7. The most common neuropathologic finding is periventricular leukomalacia, particularly in the area where fibers innervating the legs course through the internal capsule.

Spastic quadriplegia

           

1. The most severe form of CP because of marked motor impairment of all extremities and the high association with mental retardation and seizures.
2. Swallowing difficulties are common owing to supranuclear bulbar palsies, and they often lead to aspiration pneumonia.
3. At autopsy, the central white matter is disrupted by areas of necrotic degeneration that may coalesce into cystic cavities.
4. Neurologic examination shows increased tone and spasticity in all extremities, decreased spontaneous movements, brisk reflexes, and plantar extensor responses.
5. Flexion contractures of the knees and elbows are often present by late childhood.
6. Associated developmental disabilities, including speech and visual abnormalities, are particularly prevalent in this group of children.
7. Children with spastic quadriparesis often have evidence of athetosis and may be classified as having mixed CP.

Athetoid CP

1. Rare, especially since the advent of aggressive management of hyperbilirubinemia and the prevention of kernicterus.
2. Affected infants are characteristically hypotonic and have poor head control and marked head lag.
3. Feeding may be difficult, and tongue thrust and drooling may be prominent. The athetoid movements may not become evident until 1yr of age and tend to coincide with hypermyelination of the basal ganglia, a phenomenon called status marmoratus. Speech is typically affected owing to involvement of the oropharyngeal muscles.
4. Sentences are slurred, and voice modulation is impaired. Generally, upper motor neuron signs are not present, seizures are uncommon, and intellect is preserved in most patients.

DIAGNOSIS.

1. history
2. physical examination
3. Exclude progressive disorder of the CNS, including degenerative diseases, spinal cord tumor, or muscular dystrophy.
4. Electroencephalogram (EEG) and CT scan - to determine the location and extent of structural lesions or associated congenital malformations.
5. Tests of hearing and visual function.
6. Multidisciplinary approach is most helpful in the assessment and treatment of such children.

TREATMENT.

A team of

1. physicians from specialties
2. occupational and physical therapists,
3. speech pathologist,
4. social worker,
5. educator,
6. developmental psychologist  are needed.

Measures to be taken – 1.handling, 2.exercise, 3.equipments, 4. Soft tissue surgery,
5. Spinal cord surgery, 6. Motorized wheel, 7. Communication, 8.behavior, 9.mental retardation, 10.eye, 11.UTI, 12.drugs

1. Parents should be taught how to handle their child in daily activities such as feeding, carrying, dressing, bathing, and playing in ways that limit the effects of abnormal muscle tone.
   
   
2. They also need to be instructed in the supervision of a series of exercises designed to prevent the development of contractures, especially a tight Achilles tendon. There is no proof that physical or occupational therapy prevents development of CP in infants at risk or that it corrects the neurologic deficit, but ample evidence shows that therapy optimizes the development of an abnormal child.

3. Children with spastic diplegia are treated with equipment, such as walkers, poles, and standing frames.
   
   
4. If a patient has marked spasticity of the lower extremities or evidence of hip dislocation, consideration should be given to performing surgical soft tissue procedures that reduce muscle spasm around the hip girdle, including an adductor tenotomy or psoas transfer and release.
    
5. A rhizotomy procedure in which the roots of the spinal nerves are divided has produced considerable improvement in selected patients with severe spastic diplegia. A tight heel cord in a child with spastic hemiplegia may be treated surgically by tenotomy of the Achilles tendon.
   
   
6. Quadriplegia is managed with motorized wheelchairs, special feeding devices, modified typewriters, and customized seating arrangements.

7. Communication skills may be enhanced by the use of Bliss symbols, talking typewriters, and specially adapted computers including artificial intelligence computers to augment motor and language function.
   
   
8. Behavior problems interfere with the development of a child with CP; assistance of a psychologist or psychiatrist.
9. Learning and attention deficit disorders and mental retardation managed by a psychologist and educator.
10. Strabismus, nystagmus, and optic atrophy –consult with  ophthalmologist - in the initial assessment.
11. Lower urinary tract dysfunction should receive prompt assessment and treatment.
12. Several drugs have been used to treat Spasticity, including dantrolene sodium, the benzodiazepines, and baclofen.

            Intrathecal baclofen - used - in selected children with severe spasticity.

            Botulinum toxin - management of spasticity in specific muscle groups, - positive response in - patients studied.

            Patients with incapacitating athetosis occasionally respond to levodopa, and       children with dystonia may benefit from carbamazepine or trihexyphenidyl.

n><� n < � � class=MsoNormal style='tab-stops:10.2pt'> 

Palpation

Applying the palm of the hand to the chest

Thrills

increased precordial pulsation (apical in left ventricular hypertrophy and basal and right sided in right ventricular hypertrophy)

diastolic shock (in the pulmonary area in pulmonary hypertension)

The apex beat, normally in the fourth or fifth intercostal space within the mid-clavicular line

pulse wrist (radial) or inguinal region (femoral).

Sinus arrhythmia (increase in rate on inspiration with decrease on expiration)

bounding pulse

weak pulse

collapsing (

femoral pulses may be absent, or delayed

Percussion

right cardiac border does not extend beyond the right sternal edge

the upper border is at the level of the second intercostal space

determine cardiac size

Diminished or absent cardiac dullness is found in emphysema and pneumothorax.

Auscultaition

The ranges for heart rate in infancy and childhood are:

Newborn          70/120  

Infant               80/160              

Preschool child 75/120  

School child 70/110

Auscultate areas -

Mitral

Tricuspid

Pulmonary

Aortic

3^rd & 4^th left intercostal spaces,below left clavicle.

Auscultatory assessment

cardiac rhythm

heart sounds

murmurs.

Third heart sound

ejection click

intensity of heart sounds

Description of murmurs should include

1)   site,

2)   intensity (graded 0—6) with point of maximum intensity,

3)   timing (systolic: pan, early or late; or diastolic: early diastolic, mid-diastolic or presystolic,

4)   propagation (mitral systolic murmurs radiate to the left axilla, aortic systolic to the neck, aortic regurgitant down the left sternal edge) and

5)   variation with position. Coarctation of the aorta may produce a murmur audible over the back.

6)  Variation with respiration

venous hum

pericardial friction rub

to his ear.

other systems, e.g. by hepatic enlargement in cardiac failure.