CEREBRAL PALSY

Definition:

A Persistent but not unchanging disorder ofposture and movement, caused by damage to thedeveloping nervous system, before or during birthor in early months of infancy

- World Commission of Cerebral Palsy.

It is an umbrella term encompassing a group of non-progressive non-contagious motor conditions that cause physical disability in human development, chiefly in the various areas of body movement.

Cerebral refers to the cerebrum, which is the affected area of the brain (although the disorder most likely involves connections between the cortex and other parts of the brain such as the cerebellum), and palsy refers to disorder of movement.

Prenatal (Constitutes for 85%)

Perinatal (constitutes for 15%)

Post natal (constitutes for 15%)

AETIO - PATHOGENISIS

Prenatal causes:

Infection (Rubella, Toxoplasmosis, Cytomegalovirus, Herpes, AIDS)

Prematurity

Hypertension

Diabetes mellitus

Malnutrition

Radiations

Medicines

Seizures

Trauma

Perinatal causes:

Asphyxia

Infection

Disproportion of fetal head size and birth canal

Breech presentation

Cord around the neck

Post natal

Trauma

Infection

Seizures

Sudden respiratory distress syndrome

Hyperbilirubinaemia

By type of movement

Spastic - too much muscle tone

Athetoid - no muscle control

Hypotonic - decreased muscle tone

Ataxic - balance and coordination problems

Mixed - mixture of two or more of the above

Classification

By involved body parts

Hemiplegia - one arm and one leg on the same side of the body

Diplegia - predominantly both legs (arms also involved)

Quadriplegia - all four extremities

Monoplegia any one extremities

An insult to the immature brain (any time before birth up to 3 years)

The cerebral insult alters muscle tone, muscle stretch reflexes, primitive reflexes, and postural reactions.

Other associated symptoms may be involved secondary to the neurologic insult (e.g. mental retardation, vision and hearing problems, seizures)

Pathophysiology

The predominant features for all kinds of cerebral palsy are

Altered or abnormal tone

Abnormal quality of movement

Problems in coordinating the posture and movement

Sensory motor problems

Clinical features and findings

Physical attributes of different types of cerebral palsy:

The spastic type (pyramidal cerebral palsy)

Patients have signs of upper motor neuron involvement, including hyper reflexia, clonus, extensor Babinski response, persistent primitive reflexes, and overflow reflexes (crossed adductor).

Cognitive impairment is present in approximately 30% of spastic diplegic patients, but most patients with spastic quadriplegia have some cognitive impairment.

The dyskinetic type (extrapyramidal cerebral palsy)

Extrapyramidal movement patterns, abnormal regulation of tone, abnormal postural control, and coordination deficits.

Athetosis, chorea, and choreoathetoid or dystonic movements can be seen.

Patients often have pseudobulbar involvement, with dysarthria, swallowing difficulties, drooling, oromotordifficulties, and abnormal speech patterns.

hypotonic at birth, with abnormal movement patterns emerging at 1-3 years.

The arms are usually more involved than the legs.

Abnormal movement patterns may increase with stress or purposeful activity.

Muscle tone is usually normal during sleep.

Intelligence is nearly normal in patients with Athetoid cerebral palsy.

A high incidence of sensorineural hearing loss is reported.

Spastic diplegia

Period of hypotonia followed by extensor spasticity in the lower extremities, with little or no functional limitation of the upper extremities.

Patients have a delay in developing gross motor skills.

Spastic muscle imbalance often causes persistence of infantile coxa valga and femoral anteversion.

A scissoring gait (ie, hips flexed and adducted, knees flexed with valgus stress, equinus ankles) is observed.

Hemiplegia

weak hip flexion and ankle dorsiflexion, an overactive posterior tibialis muscle, hip hiking/circumduction, supinated foot in stance,

upper extremity posturing (that is, often held with the shoulder adducted, elbow flexed, forearm pronated, wrist flexed, hand clenched in a fist with the thumb in the palm),

impaired sensation, impaired 2-point discrimination, and/or impaired position sense.

cognitive impairment is found in some

Associated problems

Neurological problems:

Mental retardation

Learning disabilities

Attention deficithyperactivity disorder

Seizure disorder (epilepsy)

Visual impairment

Swallowing difficulties

Speech impairment (dysarthria)

Hearing loss

Orthopedic problems:

Scoliosis

Hip dislocation

Contractures of joints

Discrepancy in leg length

Secondary defects:

Communication disorder

Drooling

Poor nutrition

Depression

Fragile bones and frequent fractures

Cavities

Constipation

Diagnosis:

Neuroimaging studies can help to evaluate brain damage and to identify persons who are at risk for cerebral palsy.

Data to support a definitive diagnosis of cerebral palsy are lacking.

Computed tomography (CT) scanning provides information to help in diagnosing congenital malformations & intracranial haemorrhages

Neonatal ultrasonography provides information about the ventricular system, basal ganglia, and corpus callosum, as well as diagnostic information on intraventricular haemorrhage and hypoxic-ischemic injury to the periventricular white matter.

Magnetic resonance imaging (MRI) is most useful after 2-3 weeks of life. MRI is the best study for assessing white matter disease in an older child.

Evoked potentials are used to evaluate the anatomic pathways of the auditory and visual systems.

A normal brain imaging study does not mean that the child does not have cerebral palsy, since the diagnosis is always based only on physical exam findings.

Other tests:

Hearing and vision screens may be helpful.

Electroencephalography is useful in evaluating severe hypoxic-ischemic injury.

Assessment of Cerebral Palsy

Medical Management & Management of associated problems

Surgical management

MANAGEMENT

APGAR scoring

For identifying high risk infants.

A score is given for each sign at one minute and five minutes after the birth.

If there are problems with the baby an additional score is given at 10 minutes.

A score of 7-10 is considered normal,

While 4-7 might require some resuscitative measures, and

A baby with APGARS of 3 and below requires immediate resuscitation.

CRITERIA

P osturing / Poor muscle control and strength

O ropharyngeal problems

S trabismus/ Squint

T one (hyper-, hypotonia)

E volutional maldevelopment

R eflexes (e.g. increased deep tendon)*Abnormalities 4/6 strongly point to CP

P

O

S

T

E

R

A. General management- Proper nutrition and personal care

B. Pharmacologic

Botox, Intrathecal, Baclofen

- control muscle spasms and seizures,Benzodiazepines act on brain chemistry to relax muscles.

Glycopyrrolate -control drooling

Pamidronate -may help with osteoporosis.

C. Surgery

-To loosen joints,

-Relieve muscle tightness,

- Straightening of different twists or unusual

curvatures of leg muscles

- Improve the ability to sit, stand, and walk.

Selective posterior rhizotomyIn some cases nerves need to be severed to decrease muscle tension of inappropriate contractions.

D. Physical Aids Orthosis, braces and splints

Positioning devices

Walkers, special scooters, wheelchairs

E. Special Education

F. Rehabilitation Services- Speech andoccupational therapies

G. Family Services -Professional support

H. Other Treatment

- Therapeutic electrical stimulation,- Acupuncture,- Hyperbaric therapy - Massage Therapy- Exercise therapy