Week 9.1 - Cerebral Palsy & Assessment Infants & Children

Cerebral Palsy (CP)

A group of permanent disorders of the development of movement and posture, causing activity limitations that are attributed to NON-PROGRESSIVE disturbances that occurred in the developing fetal or infant brain.

In what cases will children with CP have more comorbidities?

In more severe cases

CP is the most common motor disorder in childhood. How many does it effect in 1st world and 2nd world countries?

1st world : 1.4-1.8 / 1000 live births - 1 in 500 births

2ns world: 2.95-3.4/ 1000

Why is there a need for a new definition of CP?

Need to reflect that CP is a LIFE LONG CONDITION , not just in childhood.

Current definitions being just about kids leads to gaps in research, funding, and care for many adults with CP

Cerebral Palsy is an early onset _______ neurodevelopmental condition ?

Lifelong

How do the limitations in activity due to impaired development of movement and posture manifest?

spasticity

dystonia

choreoathetosis and/or ataxia

Why does maldevelopment occur in CP?

dysplasia of or injury to the fetal or infant brain that is not degenerative. Although manifestations may change over time.

The phenotype of CP is complex and ______, with each person experiencing a unique presentation

heterogeneous

What secondary condition often means you work with a CP child until they are ~ 21 years ?

Severe pelvic obliquity (tilting) and scoliosis

Potential causes of CP? (6)

Genetic abnormalities : 1 in 10 children had a genetic variant or likely genetic variant for their CP

17.7 percent had variants of uncertain significance that may be linked with CP after further research

More common:

Damage to white matter :Periventricular leukomalacia (PVL). Tiny holes in white matter

Common in premise and presents as spastic diplegic and hemiplegic

Abnormal brain development - cerebral dysgenesis - caused by infections, trauma, maternal fever

Bleeding - intracranial hemorrhage (fetal stroke)

Severe lack of oxygen (pre-post natal) : perinatal asphyxia/ hypoxic ischemic encephalopathy (HIE) common in traumatic births

Acquired: Bacterial Menigitis, viral encephalitis, stroke, abusive/head trauma

Risk Factors for CP?

Premature birth

Low BW

Mulitple births (twins/triplets)

Blood type incompatibility between mother and baby (develops antibodies)

Exposure to toxins

What birth term and weight are at the highest risk for CP?

Born before 28 weeks

VLBW: 1000-1499g

How early can a CP diagnoses be made?

Why is early diagnosis important and should be standard of care

Before 6 months corrected age

Early diagnosis should be standard of care becuase contemporary early interventions optimize neuroplasticity and functional outcomes.

What are the most accurate ways to diagnose CP before 5 months? and After 5 months?

Before 5 months: Corrected age: MRI

General Movements Assessment

Hammersmith Infant Neurological Examination (HINE)

After 5 months: Corrected Age: MRI

Hammersmith Infant Neurological Examination (HINE)

Development Assessment of Young children are recommended

What are the 3 ways to classify CP?

Motor Types (Spastic, Dyskinetic, Ataxia, Combined)

Parts of Body (Quadriplegia, Diplegia, Hemiplegia)

Gross Motor Skills (GMFCS)

What is more common type of motor CP? How much of total motor CP does it account for? How does it present ?What is the cause?

Spastic CP

80-90%

Muscles appear stiff and tight

Happens due to Motor Cortex Damage

What is spasticity?

velocity- dependent resistance of a muscle to stretch

What can result from progression of spasticity ? How might this be apparent in gait?

Contractures

Contractures in knees and feet = little to no movement and compensations in other part of the body and altered gait pattern

Besides spastic, what are the other types of Motor CP? Explain them

Dyskinetic 6% :

Involuntary movements such as dystonia, athetosis and/or chorea

Arises from damage to the Basal Ganglia

Ataxic 5%:

Characterised by shaky movements. Affects balance and sense of positioning in space.

Arises from damage to the cerebellum

Mixed types:

Common to have 2 motor types e.g. spasticity and dystonia

Dystonia

What can increase dystonia and cause pt to get stuck?

When is dystonia NOT present ?

movement disorder in which involuntary sustained or intermittent muscle contractions cause twisting and repetitive movement, abnormal postures, or both

Dystonia: increase and causing pt to be stuck with high stress and/or emotion

Dystonia does not occur during sleep

Athetosis

Slow continuous involuntary writhing movement that prevents maintained of a stable posture. More "dancelike" than dystonia

Chorea

Ongoing randomly appearing sequence of one or more discrete involuntary movements or movement fragments

Characterized by brief, abrupt, irregular, unpredictable movements.

Ataxia

lack of coordination

What is the most common mixed type of motor CP? What is the challenge with this?

Spasticity + dystonia

Makes it difficult to measure each

Explain the Parts of the Body classifications of CP (3) How common is each?

Spastic Hemiplegia/ unilateral: 39% * MOST COMMON

One side of the body (arm and leg) are affected

Spastic Diplegia/ Bilateral: 38%

Both legs are affect

Arms may be affected to lesser extent

Spastic Quadriplegia/Bilateral :23%

Both arms and leg are affected

Muscles for trunk, face and mouth are often also affected

Gross Motor Classification System (GMFCS)

What is it?

What is it based on?

What does is emphasize?

Standardized system to classify gross motor function

Based on observation of the child's self-initiated movement and need for hand-help mobility devices of wheeled mobility

Emphasis in on usual performance in home, school, and community

GMFCS Levels

Level 1 - indepent walking without limitations

Level 2 - Walks with limitations Use of hand rails

Level 3 - Handheld mobility devices

Level 4: Self mobility with limitations. May use powered mobility

Level 5: Transported in Manual Wheelchair

What was added to the GMFCS - Expanded and Revised?

Added ages 12-18

What are the age groups for GMFCS?

Under 2

2-

What process occurs when a child is classified with the GMFCS

Classification is not stable.

Will be reclassified after 2 years and will stay at this level moving forward

Is the GMFCS an outcome measure?

NO.

It is just a scale.

How does the GMFCS help us to optimize management of treatment for those with CP? (6)

Gives use an idea of what they will be like as a teenager

Enhances communication

Emphasizes abilities rather than limitations

Assists with realistic goal settin g

Assists with intervention planning

Facilitates resource allocation

What level of CP tend to have more behavioural problems?

Mild CP

What levels of CP is pain present?

ALL LEVELS

How many people with CP have hip displacement? What intervention does this indicate?

1 in 3 - this means hip surveillance x-rays every 6 or 12 months

Which GMFCS levels have hip displacement issues? Which levels are most common?

GMFCS level 1 (if they have Winter-Gage-Hicks) , 4 and 5

Levels 4 and 5 are most common (68-90%) : displacement or complete dislocation

What does hip displacement affect for CP patients?

Gross motor function

What is a Winter-Gage- Hicks Pattern (aka Type IV gait pattern) ?

What level of CP is this seen in?

What does this put them at risk for?

Unilateral involvement

Hip Flexion

Hip Adduction

Hip Internal Rotation

Knee Flexion

And are up on their toes

This is in children with hemiplegia & GMFCS level 1

It puts them at risk for hip displacement

The process of monitor and identifying critical early indicators of hip displacement

Hip Surveillance

Primary goal of hip surveillance

Ensure that the progressive hip displacement is detected early enough to enable timely referral for orthopaedic assessment and treatment

What causes hip displacement?

At birth, joint is fine

Over time, head of the femur displaces naturally due to spasticity around the muscle

What is the ideal position for hip surveillance imaging? Why is this important?

" AP PELVIS" X ray

Supine with pelvis horizontal, knees and toes up, neutral ADD/ABD

LOW BACK FLAT ON FLOOR (this can be achieved by putting blocks under the knees/legs

This position is needed to assess migration percentage (MP)

What is migration percentage (MP)? What MP is concerning?

Home much the head of the femur is displaced laterally to the acetabulum

MP > 30% = concerning

What is the man ROM you should be aware of in regards of hip displacement in CP?

reduced hip ABduction (PROM)

What are the 3 other key questions you want to ask to the parent/ child (as filled out on NS Health Form)

Is it more difficult to care for the child now?

Is there a decreased ability to WB through the hips?

Is there a deterioration in ability to walk?

considerations for the selection of outcome measures for children with cerebral palsy

What outcome measures are possible?

Whats the ICF and “F-Words” in Childhood Disability

What is the the Gross Motor Function Measure (GMFM 88 & 66)