Cerebral Palsy

CEREBRAL PALSY DEFINITION

It is a group of neurological disorders that result from non-progressive brain damage during early development and are characterized by abnormalities of movement and posture. The incidence is about 2 per 1000 live births, with the highest rates in premature babies and those of multiple births.

PATHOPHYSIOLOGY

This process involves microglial (brain macrophage) cell activation and cytokine release, which causes damage to a specific cell type in the developing brain called the oligodendrocyte.

The oligodendrocytes are a type of supportive brain cell that wraps around neurons to form the myelin sheath, which is essential for white matter development.

Intrauterine infections activate the fetal immune system, which produces cytokines (e.g., interferon- γ and TNF-α) that are toxic to premyelinating oligodendrocytes.

Infections also activate microglial cells, which release free radicals. Premyelinating oligodendrocytes have immature defenses against reactive oxygen species (e.g., low production of glutathione, an important antioxidant).

Cerebral palsy has associated problems such as perceptual problems, epilepsy, behavioral problems and learning difficulties. The main consequence or complications are development of neuromuscular incoordination, dystonia, weakness and spasticity.

Oro-facial motor incoordination may make speech and swallowing difficult and drooling is a frequent problem

  CAUSAL FACTORS (ETIOLOGY)

• maternal toxaemia which is associated with reduced incidence of germinal matrix hemorrhage in premature babies
• prematurity,
• Perinatal hypoxia is marked by oxygen loss and blood supply loss to the baby and generally occurs shortly before or during birth. It accounts for around 6% to 8% of all cerebral palsy cases.
• kernicterus –it is a type of brain damage that can result from high levels of bilirubin in a baby's blood
• postnatal brain infections or injury; birth injury

SIGNS AND SYMPTOMS OF CEREBRAL PALSY

• Delays in movements and motor skills, such as holding head up, rolling over, sitting, crawling, and walking
• Body parts that are too stiff or floppy
• Favoring one side of the body
• Scooting on the bottom instead of crawling
• Inability to stand, even with support

DIFFERENTIAL DIAGNOSIS

Diagnosis in infancy

During infancy, diagnosis may be difficulty to determine. A history of prenatal toxaemia, haemorrhage, premature birth, difficult labor, foetal distress or kernicterus should arouse suspicion.

A neonatal ultrasound scan of the head may identify intracerebral bleeding that would increase the likelihood of later problems.

 Early symptoms include difficulty in sucking and swallowing, with dribbling at the mouth.

    Cerebral palsy – early diagnosis

By 6 months these twin brothers had developed quite differently, the one being smaller and showing (1) lack of head and arm control, (2) lack of body control when helped to the sitting position and (3) inability to sit unaided.

SITTING POSTURE -The child may find it difficult or impossible to sit unsupported hypotonic trunk may slump into a kyphotic posture and others may always ‘fall’ to one side. In attempting to sit, the lower limbs may be thrust into extension. There may be an obvious scoliosis or pelvic obliquity.

STANDING POSTURE-In the typical case of a spastic diplegia, the child stands with hips flexed, adducted and internally rotated, the knees are also flexed and the feet are in equinus. With tight hamstrings.

Balance reactions are often poor and a gentle push that would force a normal child to take a step in the appropriate direction to maintain his or her balance may simply knock over a child with cerebral palsy.

DIAGNOSIS IN LATER CHILDHOOD ADULT

The motor disorders of cerebral palsy are often accompanied by disturbances of sensation, perception, cognition, communication, and behavior, by epilepsy and by secondary musculoskeletal problems. Knowledge of the spinal cord tracts gives an understanding of how muscle control and coordination is affected among children with cerebral palsy.  Tracts comprises of collection of nerve fibers that connects 2 masses of grey matter within the central nervous system (CNS).they are classified into 2; the descending Tracts (Motor pathways) and The Ascending Tracts (Sensory Pathways)

The descending tracts (motor pathways) consist of motor neurons that deliver efferent (Motor) impulses from the brain to the spinal cord. Functionally divided into 2 major groups; Pyramidal tracts and Extrapyramidal tracts.

The Pyramidal tracts are formed by axons originating from pre-motor cortex and primary motor cortex. They form a major pathway concerned with the voluntary control of the musculature of the body and face movement. The pyramidal tracts derive their name from the medullary pyramids of the medulla oblongata, which they pass through. Functionally, these tracts can be subdivided into two:

Corticospinal tracts – supplies the musculature of the body.

Corticobulbar tracts – supplies the musculature of the head and neck.

In the most inferior (caudal) part of the medulla, the tract divides into two: The fibers within the lateral corticospinal tract decussate (cross over to the other side of the CNS). They control the voluntary muscles responsible for skilled movement of the upper and lower limb of the opposite side of the body. The anterior corticospinal tract remains ipsilateral, descending into the spinal cord, decussate and terminate in the ventral horn of the cervical and upper thoracic segmental levels. They control movement of axial muscles (muscles of the trunk) same side of the body.

Extrapyramidal Tracts

The extrapyramidal tracts originate in the brainstem, carrying motor fibers to the spinal cord. They are responsible for the involuntary and automatic control of all musculature, such as muscle tone, balance, posture and locomotion. Extrapyramidal tract injuries are commonly seen in degenerative diseases, encephalitis and tumors. They result in various types of disorders of involuntary movement.

Developmental milestones

Developmental milestones are behaviors or physical skills seen in infants and children as they grow and develop. Rolling over, crawling, walking, and talking are all considered milestones. The milestones are different for each age range. There is a normal range in which a child may reach each milestone. A parent is likely to notice when a child doesn’t roll over when he or she should, or when an infant is not enjoying a first step. When this occurs, it could mean the child is slow to develop, or it could be a sign of developmental delay. Developmental delay is an indicator that a child may have Cerebral Palsy. Babies with Cerebral Palsy are often slow to reach developmental motor milestones. These may include learning to roll over, sit, crawl, or walk.

In the event a child has developmental delays, a number of interventions will help a young person develop their skill set, and their confidence, into adulthood. If developmental delay is suspected, the ensuing evaluation is broken into two parts:

NEUROMUSCULAR EXAMINATION

Examination of the limbs shows the typical features of upper motor neuron or spastic paresis. Passive movements are resisted, the reflexes are exaggerated and there is a positive Babinski response. However, spasticity may obscure the fact that muscle power is actually weak.

By the end of the examination the clinician should have a clear idea of the muscle tone, muscle power and range of movement at each joint.

DEFORMITY ASSESSMENT

Deformity at one level may be markedly affected by the position of the joints above and below. E.g., ankle equines with the knee extended often disappears when the knee is flexed; thus one can differentiate between tightness in the soleus and tightness in the gastrocnemius muscle.

In the Silfverskiöld test, with the child lying supine on the examination couch, the knee is flexed to a right angle and the ankle is dorsiflexed; this tests soleus tightness.

The knee is then fully extended on the couch and ankle dorsiflexion is repeated; now it is mainly gastrocnemius tightness that is being tested.

MANAGEMENT OF CEREBRAL PALSY

There is no single ‘blueprint’ for the management of all patients with cerebral palsy; each patient and his or her family provides a different challenge.

GOAL SETTING

 (1) An ability to communicate with others;

 (2) An ability to cope with the activities of daily living (including personal hygiene); and

 (3) Independent mobility – which may mean a motorized wheelchair rather than walking. For the child who from an early age is recognized to be ‘non-walking’ realistic goals should be:

(1) A straight spine with a level pelvis;

(2) Located, mobile and painless hips that flex to 90 degrees (for comfortable sitting) and extend sufficiently to allow comfortable most of the effects are seen during the period of growth; after skeletal maturity the changes in muscle tendon length and joint contracture are much less progressive.

Bony deformity

Normal bone growth is influenced by muscle pull. Hence in children with persistent abnormal muscle pull sleeping and participation in standing/swivel transfers;

(3) Knees that are mobile enough for sitting, sleeping and transferring; and

(4) Plant grade feet that fit into shoes and rest on the footplates of the wheelchair comfortably. For all children good medical care is also essential as is access to good quality orthotic supports, walking aid

Physical therapy

Cerebral palsy affects motor function in several ways. There is a dependence on immature or primitive reflexes and a loss of selective muscle control.

 Physiotherapy attempts to reduce or prevent the problems arising from abnormal muscle tone, imbalance between opposing muscle groups and abnormal body balance mechanisms.

A range of regular movement exercises will prevent or reduce the degree of muscle/joint contracture.

Physiotherapy is considered to be most helpful in early childhood up to the age of 7 or 8 yrs postoperative physiotherapy is essential in order to maximize the effects of surgery and overcome the immediate pain, stiffness and weakness that follow surgery.

Positioning and splinting Care must be taken at all times to ensure that the child both sits and sleeps, works and eats in a good position and with good posture

The indications for surgery are:

(1) A spastic deformity which cannot be controlled by conservative measures;

(2) Fixed deformity that interferes with function;

 (3) Secondary complications such as bony deformities, dislocation of the hip and joint instability

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