☺Components of cerebral palsy

• Abnormal control of motor function
• Nonprogressive disorder of movement and/or posture
• Damage to Immature Brain

☺ Detection of CP

• Most patients with cerebral palsy are simply hypotonic and physically and developmentally delayed during the first year of life.
• Frequently, the child is older than 1 year of age before he is referred to a specialist because the child appears to be developmentally delayed and the only physical sign of cerebral palsy may be persistent infantile reflexes.
• Probably the greatest predictor of cerebral palsy in the first year of life is an abnormal perinatal history.

☺ Average age at detection

• Spastic diplegia 8 to 10 mths
• Hemiplegia 20 to 24 mths
• Athetosis 24 mths

Etiology of CP

• In most cases of cerebral palsy only risk factors can be identified, and not specific causes. Only approximately 10 to 15% of patients in one large group had documented perinatal hypoxia or other problems
• 60 to 65% of afflicted children were born at full term
• 10% of cerebral palsy patients weigh less than 1,500 grams at birth
• The risk of having cerebral palsy is 90 per 1,000 for premature SGA child compared with 3 per 1,000 if weighing more than 2,500 grams and appropriate for gestational age

☺ Risk Factors for CP

Classification

☺ Neuropathic

• Spastic: Hyperreflexia and Hyperre
  flexia, Weakness, Loss of muscle,control Dexterity, Interference with balance, Fatigability, Simultaneous contracting of antagonistic muscles

• Athetoid: Purposeless writhing movements which become intensified when the child is frightened or excited.Associated with Kernicterus
• Dystonia; Increased general muscle tone, Distorted postures, Abnormal positions that are induced by voluntary movements
• Ataxic: Disturbance of coordinated movement, most noticed when walking, Cerebellar dysfunction, Intention tremor
• Hypotonic: Transient Phase,The brain lesion is present but masked by lack of myelination of the pathways that will carry its abnormal messages,Mixed

☺ Anatomical types

• Diplegia:

  • Lower limb > Upper limb

  • Periventricular haemorrahage

  • Hypoxia

• Hemiplegia:

  • Upper limb > Lower limb
  • Focal brain lesion: Trauma, vascular, infection
  • Homonymous hemianopia
  • Asteriognosis
  • Epilepsy

• Whole body (quadriplegia)

  • Global involvement with mental retardation
  • The usual cause is severe hypoxia.
  • Initially presenting as a floppy babyBulbar dysfunction ( drooling, dysarthria, and dysphagia)
  • Seizures
• Monoplegia
• Double hemiplegia
• Triplegia

History

☺Purpose

History will provide knowledge about

• Etiology/ Risk factors
• Milestones
• Ability/ disability
• Associated problems
• Treatment
• Family’s resourses

☺ Presentations

• Follow up of “at risk” infants, such as those born prematurely
• Delayed motor milestones, particularly learning to sit, stand and walk
• Asymmetric movement patterns, for example, strong hand preference early in life
• Abnormalities of muscle tone particularly spasticity or hypotonia
• Management problems, for example, severe feeding difficulties and unexplained irritability. Many other conditions present with these features.

☺ Key points in history

Examination

☺ Neurological examination

• Skull circumferance ( Hydrocephalus/ microcephaly)
• Mental status
• Cranial nerves
• Vision/ Hearing/ speech
• Motor System
• Muscle strength and selective control
• Muscle tone
• Reflexes and sensory function

☺Degree of deformity or muscle contracture

• Range of motion
• Deformity (linear, angular, and torsional deformation of the spine and long bones, and fixed hand or foot deformities)
• Balance, equilibrium, and standing or walking postures

☺ Functional Examination

• Dynamic examination evaluate the head control, sitting balance, the ability to crawl, the ability to pull up to stand, standing posture and balance, and the ability to walk.
• Observational gait assessment is imperative in those who can walk.

☺Orthopaedic Evaluation of the lower limb

These tests are described in standard orthopaedic examination text books

☺ Measurements in spasticity

Tardieu Scale

This test is performed with patient in the supine position, with head in midline.

Measurements take place at 3 velocities (V1, V2, and V3). Responses are recorded at each velocity as X/Y, with X indicating the 0 to 5 rating, and Y indicating the degree of angle at which the muscle reaction occurs. By moving the limb at different velocities, the response to stretch can be more easily gauged since the stretch reflex responds differently to velocity.

Velocities:

V1: As slow as possible, slower than the natural drop of the limb segment under gravity

V2: Speed of limb segment falling under gravity

V3: As fast as possible, faster than the rate of the natural drop of the limb segment under gravity

Angles

R1, the angle of catch following a fast velocity stretch at either V2 or V3; and R2, the passive range of movement achieved following a slow velocity stretch at V1

Scoring:

0 No resistance throughout the course of the passive movement

1 Slight resistance throughout the course of passive movement, no clear

catch at a precise angle

2 Clear catch at a precise angle, interrupting the passive movement, followed

by release

3 Fatigable clonus with less than 10 seconds when maintaining the pressure

and appearing at the precise angle

4 Unfatigable clonus with more than 10 seconds when maintaining the

pressure and appearing at a precise angle

5 Joint is immovable

Modified Ashworth Scale

0 No increase in muscle tone

1 Slight increase in muscle tone, manifested by a catch and release

or by minimal resistance at the end range of motion when the part is

moved in flexion or extension/abduction or adduction, etc.

1+ Slight increase in muscle tone, manifested by a catch, followed by

minimal resistance throughout the remainder (less than half) of the

ROM

2 More marked increase in muscle tone through most of the ROM, but

the affected part is easily moved

3 Considerable increase in muscle tone, passive movement is difficult

4 Affected part is rigid in flexion or extension (abduction ,adduction, etc.)

Associated Problems

• CNS:
• Mental retardation (30 to 65%) behavioral and emotional difficulties perceptual disorders
• Learning disorders
• Bulbar involvement
• Sensory deafness
• Visual difficulties (50%) perceptual problems, strabismus, nystagmus, and cortical blindness
• Gastrointestinal
• Constipation and fecal impaction
• Impaired swallowing, vomiting, esophageal reflux, and hiatal hernia
• Aspiration and the risk of severe pneumonia, epigastric pain, profound feeding problems, and poor nutrition
• Genitourinary
• Bladder dysfunction (28 %)
• Urinary incontinence (26 %)
• Urinary tract infections

Functional Assessment

☺Gross Motor Function Classification System (GMFCS)

Class Goal of treatment

1- Walks independently, speed, balance & coordination reduced

2- Walks without assistive devices but limitations in community Diminish energy expenditure, decrease level of support, improve appearance

3- Walks with assistive devices Improve gait, position for sitting, transfers, supported standing

4- Transported or uses powered mobility Decrease pain, improve sitting & standing

5- Severely limited, dependent on wheelchair Better positioning, decrease pain, improve hygiene

Gaits in cerebral palsy

Hemiplegic gait

Hemiplegic gait is characterised by pes equinus, genu recurvatum,

internal femoral rotation and hip adduction.

There are four types of hemiplegic gait

Type 1: There is weakness of the tibialis anterior and an adequate

gastrocnemius-soleus length.

Type 2: Gastrocnemius-soleus muscle is short in addition to tibialis anteriorweakness. The child compensates with knee hyperextension in midstance

Type 3: There is persistent knee flexion in stance phase and decreased knee motion in swing phase in addition to the above findings. This is defined as stiff knee gait.

Type 4: There is adduction and flexion of the hip in addition to the findings above  Bony deformities such as excessive internal femoral rotation and tibial torsion may also be seen

Diplegia gait patterns.

Scissoring

Scissoring is a frontal plane pathology also called crossing over.

Cause-hip adductor and/or medial hamstring spasticity.

Persistent femoral anteversion

The child walks with legs crossing one another. The hip is in flexion, adduction and internal rotation. The knees are turned inward.

Jump gait

most common sagittal plane pathology in young diplegic children. Almost all diplegic children begin walking with a jump knee gait pattern.

Jump gait is defined as excessive hip flexion, knee flexion and equinus in stance Cause- lower extremity flexor muscle spasticity.

The child walks with hips and knees in flexion and ankles in plantar flexion looking like an athlete getting ready to jump.

Crouch gait

The second most common sagittal plane pathology and it occurs in the older diplegic.

It is defined as excessive knee flexion throughout the stance phase with dorsiflexion of the ankle joint.

Causes-short or spastic hamstrings, hip flexor tightness and excessive ankle

dorsiflexion. Excessive ankle dorsiflexion may result from isolated triceps surae lengthening without addressing the spastic hamstrings.

Hamstring tightness causes crouch and a short step length when walking. When sitting, tight hamstrings pull the ischial tuberosities and tilt pelvis posteriorly causing kyphosis and sacral sitting.

Stiff knee

This is a sagittal plane pathology characterized by limited range of motion in the knee joint, especially a lack of flexion in swing

Cause- spasticity of rectus femoris muscle or unopposed rectus femoris function after hamstring lengthening.Compensatory movements of hip external rotation and circumduction are observed. The patient experiences difficulty going up steps. Step length is shortened, foot clearance is poor, shoes wear out rapidly.

Genu recurvatum

Genu recurvatum occurs in the stance phase of walking and is generally  associated with mild equinus caused by triceps surae spasticity, excessive spasticity in the quadriceps, and may be related to weakness of the hamstring muscles or contracture of the hip flexors.

Investigations

• Urine / plasma metabolic screen if a metabolic disorder is suspected
• TORCH titer if congenital infections suspected
• Chromosomal analysis if genetic disorder suspected
• MRI
  ? vascular lesion
  ? malformation
  ? periventricular leucomalacia

Botulinum toxin is a medication and a neurotoxic protein produced by the bacterium Clostridium botulinum.

Botulinum toxin type A, is a therapeutic muscle-relaxing agent that reduces the rigidity of muscles or unwanted spasms in a specific muscle. Over the past ten years, the use of botox has shifted from wrinkle reduction to successfully treating muscle spasticity in children with cerebral palsy, making botox and cerebral palsy a hugely successful match.

How does Botulinum Toxin work?

Botulinum toxin is a neurotoxic protein produced by the bacterium Clostridium botulinum. It affects the neuromuscular synapse by inhibiting the release of acetylcholine. The action of Botulinum toxin lasts for 3-6 months. this is because new nerve ending sprout and in later stages the original neuromuscular junction recovers.

Clinical effect of Botulinum toxin starts in 1-3 days but maximal effect is evident at 1-2 weeks

Cerebral Palsy : what happens with the muscles?

In cerebral palsy, the brain los es the ability to moderate the activity of contracting muscles. Muscles that produce contraction are stronger than those that produce extension, Partially paralyzing the stronger muscles with botulinum toxin gives patients an opportunity to stretch and strengthen the weak muscles. The long-term goal of the two components of the treatment – injections and physical therapy – is to achieve b etter muscle strength balance which may lead to restoring normal function.

How does Botox help in Cerebral palsy ?

The marriage of botox and cerebral palsy may seem odd at first, but is actually quite practical. When prepared for therapeutic use, botox is injected in small amounts into the spastic or stiff muscles. It begins to take effect by blocking transmission between the nerves and the affected muscles. The injection stops the signal between the nerve and the muscle, relaxing the muscle and reducing stiffness. Botox only affects the muscles that are injected and once the muscles are relaxed, therapists are able to stretch the muscles and stimulate normal growth.

Botox injections can offer many benefits including ease in stretching, improvement in child’s range of motion, tolerance to wearing braces and developmental improve ments in crawling, standing, or gait changes.

Who respond the best to Botox?

Children under the age of six respond best to this type of cerebral palsy treatment, especially effective in children who have not developed fixed joint contractures. It is most effective when used in the early stages of spasticity while the child’s bones are still developing and before problems with bone development and deformity set in.

Precaution to be taken after Botox injection

Since this treatment changes muscle tone , your child may initially miss some of the support that they relied on from their increased tone. Therefore, until your child gets used to these changes, we recommend close supervision in situations where they may be at increased risk of falling. This might include going up and down stairs, walking in halls, or other busy areas. Other than this precaution, there are no restrictions following the injections and normal activities can be resumed...

What are the advantages of Botulinum toxin

· can be done without anesthesia

· easy to administer

· essentially painless

· effects wear off

· most side-effects are minor

· can be repeated in 6 months

Disadvantages of Botulinum toxin

· patient can develop resistance to BTX-A (antibodies, unknown) but it usually reverses itself over time

· expensive

· limited effect

· minor comp lications like headache, flu like symptoms

Role of physiotherapy

Physiotherapy, which involves the use of stretching, strengthening, casting,bracing, positioning and facilitation of movement, is an essential part of the treatment plan for children with cerebral palsy. These forms of treatments assist

children in developing muscular control, better balance and mobility.

Ability ofthe parent or carer to assist their child in becoming as physically independent as possible is enhanced by physiotherapy. A range of motion exercises, tailoredfor each child help improve strength, maintain muscle length and improve movement of joints. Exercises can help strengthen muscles and improve physical stamina, preventing the weakening and wasting of under used muscles. This further reduces the risk of developing fixed contractures.

Children who stand and walk may benefit from the provision of special foot splints and walking aids (called orthotic appliances or orthoses) which, as well as supporting joints to assist in walking, are also able to assist in avoiding fixed contractures by stretching overactive muscles.

For more information on role of Botulinum toxin in cerebral palsy email to doctor@ipodindia.org

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. CP is caused by damage to the motor control centers of the developing brain and can occur during pregnancy (about 75 percent), during childbirth (about 5 percent) or after birth (about 15 percent) up to about age three^. Further research is needed on adults with CP as the current literature is highly focused on the pediatric patient.

Cerebral palsy describes a group of permanent disorders of the development of movement and posture, causing activity limitation, that are attributed to nonprogressive disturbances that occurred in the developing fetal or infant brain. The motor disorders of cerebral palsy are often accompanied by disturbances of sensation, perception, cognition, communication, and behaviour, by epilepsy, and by secondary musculoskeletal problems.^[5]

There is no known cure for CP. Medical intervention is limited to the treatment and prevention of complications arising from CP’s effects. A 2003 study put the economic cost for CP sufferers in the US at $921,000 per case, including lost income.

In another study, the incidence in six countries surveyed was 2.12–2.45 per 1,000 live births^, indicating a slight rise in recent years. Improvements in neonatal nursing have helped reduce the number of babies who develop cerebral palsy, but the survival of babies with very low birth weights has increased, and these babies are more likely to have cerebral palsy.