Cerebral Palsy Pt 1

Question 1

definition of cerebral palsy

Answer 1

• Group of clinical symptoms or a syndrome characterized by a disorder of movement and posture due to a static lesion or defect in an immature brain
• Most common non-progressive movement disorder in children

Question 2

risk factors associated with development of CP

Answer 2

History taking should include screening questions related to the mother’s health, pregnancy, birth and post-birth period. Risk factors for CP may be at the maternal or infant level.

Question 3

maternal risk factors

Answer 3

• Thyroid condition
• Infection during pregnancy
• Pre-eclampsia
• Multiple pregnancy (i.e. twins or greater)

Question 4

infant risk factors

Answer 4

• Prematurity
• Intrauterine growth restriction
• Low birth weight
• Acute intrapartum hypoxic event
• Moderate to severe encephalopathy
• Neonatal seizures
• Neonatal infection
• Hypoglycaemia
• Jaundice

Question 5

additional risk factors identified in children born at term

Answer 5

• Placental abnormalities
• Birth defects
• Meconium aspiration
• Instrumental/emergency caesarean delivery
• Birth asphyxia
• Respiratory distress syndrome

Question 6

postnatal risk factors

Answer 6

• Head injuries: TBI’s
• Infections: meningitis, encephalitis
• Brain hemorrhage: IVH
• Asphyxia: HIE, cardiac arrest, near drowning

Question 7

small percentage of children acquiring CP after one month of age

Answer 7

generally a result of stroke, which may occur spontaneously or from complications associated with another condition or medical intervention

Question 8

how CP is classified

Answer 8

According to muscle tone/movement patterns (MOTOR TYPE)

according to distribution in body (TOPOGRAPHY)

Question 9

spastic

Answer 9

Spasticity is a velocity-dependent resistance to stretch by the muscles. It is characterized by an excessive stiffness in the muscles when the child attempts to move or maintain a posture against gravity.

Question 10

dyskinetic

Answer 10

Dyskinesia/hyperkinesia refers to an increase in muscular activity that can result in excessive abnormal movements, excessive normal movements, or a combination of both. Dyskinetic/hyperkinetic CP is characterized by abnormalities of tone and various movement disorders including dystonia, athetosis and chorea.

Question 11

ataxic

Answer 11

Ataxia is characterized by shaky movements and affects a person’s coordination and balance. It is the least common form of CP - less effect on muscle tone, but great impact on balance and coordination. Exhibit difficulty with balance, coordination, and maintenance of stable alignment of head, trunk, shoulders, and pelvis.

Question 12

mixed

Answer 12

child shows components of high and low muscle tone; examples:
CP where more than one motor type is present, for example spasticity and dystonia.
Usually one motor type will be dominant.

Question 13

monoplegia

Answer 13

involves single extremity; upper or lower

Question 14

hemiplegia

Answer 14

involves one side of body

Question 15

dipelgia

Answer 15

total body is affected with LE’s/trunk more affected than UE’s/face

Question 16

quadriplegia

Answer 16

total body affected including face, neck, and trunk with equal involvement – typically seen in dyskinetic and ataxic types

Question 17

triplegia

Answer 17

involves three extremities

Question 18

dystonia

Answer 18

characterized by sustained or intermittent muscle contractions causing repetitive or twisting movements

Question 19

athetosis

Answer 19

characterized by uncontrolled, slow and writhing movements that prevent maintenance of a stable posture. It involves continuous smooth movements that appear random and are not composed of recognizable sub-movements or movement fragments. Athetosis is made worse by attempts to move however it may also be present at rest. Athetosis is distinguished from dystonia by the lack of sustained postures, and from chorea by the lack of identifiable movement fragments

Question 20

chorea

Answer 20

ongoing random-appearing sequence of one or more discrete involuntary movements or movement fragments.

Chorea is distinguished from dystonia due to the apparently random, unpredictable, and continuously ongoing nature of the movements, compared with the more predictable and stereotyped movements or postures of dystonia. The movements of chorea often appear more rapid than those associated with dystonia

Question 21

primary impairments

Answer 21

• Primary impairments are those that are immediate and a direct result of the cortical lesion in the brain.
• The nervous system damage that causes cerebral palsy can occur before or during birth or before or during birth or before a child’s second year, the time when myelination of the child’s sensory and motor tracts and CNS structures rapidly occurs.
• As a “non-progressive condition”, the original defect or lesion occurring the in the CNS typically does not worsen or change over time.
• However, because the lesion occurs in immature brain structures, the progression of the child’s motor development may appear to change (secondary impairments)
• Growth can be severely impacted in children with CP. Separate growth tables are available to track overall growth for both boys and girls with CP

Question 22

secondary impairments

Answer 22

• in systems or organs over time due to the effects of one or more of the primary impairments
  • For example, a child with cerebral palsy may have a primary impairment of hypertonia and a muscle imbalance across a joint. This abnormal muscle tone may cause poor alignment across a joint, further muscle weakness, and eventually a contracture in the joint.
  • The resulting muscle contractures, poor body alignment, and poor ability to initiate movement would be considered secondary impairments.
  • This is important to understand since the diagnosis of cerebral palsy means a child has a static non-progressive lesion in the brain. Although the initial brain injury is unchanging, the results or the secondary impairments are not static and change over time with body growth and attempts to move against gravity.
  • Children with cerebral palsy may continue to rely on automatic movement pattern because they are unable to direct their muscles to move successfully in more typical motor patterns.
  • The atypical patterns used to play or complete functional activities may become repetitive and fixed. The repetition of the atypical movement patterns prevent children with cerebral palsy from gaining independent voluntary control of their own movements and can lead to diminished strength and musculoskeletal problems.
  • The combination of impaired muscle co-activation and the use of reflexively controlled postures may lead to future contractures in the muscle, tendon, and ligamentous tissues, causing the tissues to become permanently shortened. Bone deformities and alterations of typical posture or spinal and joint alignment may also occur.

Question 23

diagnosis/indicators highly predictive of CP

Answer 23

• No definitive diagnostic test; difficult to get medical diagnosis under 1 year of age
• Indicators highly predictive of CP
• Prematurity
• Low birth weight
• Hypertonus at birth or still present at 4 months
• Delayed motor milestones at 4 months
• Abnormal findings on MRI or HUS (head ultra sound)

Question 24

key prognostic message - life long disability

Answer 24

CP is a life-long disability
• Disability increases with age
• Aging occurs earlier
• Rehabilitation planning must consider adulthood

Question 25

key prognostic message - normal life expectancy

Answer 25

Almost all children with CP will have a normal life expectancy
• 5-10% of children with CP will die in childhood
• The risk of premature death increases with co-occurring epilepsy, intellectual disability and severe physical impairment
• Presence of severe dysphagia increases the risk of premature death

Question 26

key prognostic message - presence of associated conditions affect outcomes

Answer 26

CP is almost always accompanied by associated conditions

• Screening for the presence of associated conditions should be routine and associated conditions managed if present

Question 27

key prognostic message - severity predictions

Answer 27

Under 2 years of age, severity predictions are incorrect 42% of the time - voluntary movement is still developing and hypertonia may still be evolving with myelination
• MRI may provide some information for predicting function - unilateral injuries usually result in milder presentations; periventricular white-matter lesions generally result in milder motor impairments (ie, usually ambulatory) but not always, whereas brain malformations, cortical, subcortical, and basal ganglia lesions generally result in more severe motor impairments (ie, non-ambulatory)

Question 28

using MRI to predict prognosis

Answer 28

Do not rely on MRI alone for predicting prognosis in children with CP
• Consider the likely cause of CP and the findings from MRI (if performed) when discussing prognosis with the child or young person and their parents or caregivers
• Combine information obtained about risk factors, neuroimaging and assessment of motor function **(especially the Hammersmith Infant Neurological Examination - HINE) to assist prediction of severity and therefore prognosis

Question 29

characteristics of children with SPASTIC CP

Answer 29

Area of CNS damage: primary motor cortex, corticospinal tracts

Sub-classifications: monoplegia, hemiplegia, diplegia, or quadriplegia\

Muscle tone: hypertonic
UE’s- scapular retraction, shoulder add/ext/IR
LE’s- hip ext/add/IR, knee ext, ankle PF

Range and quality of movement: movement limited to midrange; labored and in synergistic patterns

Righting/Equilibrium/Protective extension: delayed or absent; pull into COG to increase postural stability

Respiratory Problems: decreased thoracic mobility, decreased respiration to support phonation

Speech problems: Dysarthria, decreased dissociation of tongue/lip/jaw with poor lip closure

Feeding Problems: abnormally strong gag reflex, tongue thrust, bite reflex, and rooting reflex

Deformities: ***at risk for contractures, hip dislocation

Other signs: associated reactions, exaggerated positive support reaction, clonus, increased DTR’s

Personality: postural insecure with fear of falling, may be afraid of loud noises,, most remain dependent, withdrawn and passive

Question 30

characteristics of children with DYSKINETIC CP

Answer 30

Area of CNS damage: Basal Ganglia

Sub-classifications: usually quadriplegic

Muscle tone: usually hypotonia at birth; athetosis may develop 1-4 years of age; large sudden fluctuations; usually seen with spastic component

Range and Quality of Movement: DEPENDS ON TYPE: (Athetoid, choreoathetoid, dystonic). Extremities may move through extreme ranges, lack of grading of antagonistic muscles (jerky); little midrange control; limited stability; lack of coordinated movements; involuntary movements may be exaggerated by volitional movements

Righting/Equilibrium/Protective Extension: absent or delayed; unreliable if present

Respiratory problems: decreased thoracic mobility, shallow breathing

Speech problems: May have dysarthria, volume of speech may go up/down with breath

Feeding problems: Decreased due to instability, poor coordination of
tongue/jaw/swallow, strong gag, tongue thrust and poor coordination of oral muscles for chewing/swallowing

Deformities: low risk to develop but potential if has a spastic component

Other signs: normal DTR’s

Personality: Emotionally unstable and immature, sudden fluctuations from happy to extreme anger and tantrums, less fearful of movement, easily frustrated

Question 31

characteristics of children with ATAXIC CP

Answer 31

Area of CNS damage: cerebellum

Sub-classifications: usually quadriplegic

Muscle tone: usually hypotonic but can fluctuate slightly, can have spastic or athetoid component

Range and Quality of Movement: poor coordination/balance, trouble with postural fixation an stabilizing proximal joints, clumsy, unable to direct or limit motion, intention tremors

Righting/Equilibrium /Protective Extension: usually developed but executed abnormally due to poor coordination, difficulty with balance

Oral Motor: speech is monotone and slow, decreased articulation, uses teeth to stabilize tongue or to hold cup to mouth when drinking

Deformities: low risk

Other signs: decreased DTR’s

Personality: no specific personality traits

Question 32

OT assessment

Answer 32

• Early identification of atypical postures typically seen in children diagnosed with cerebral palsy can minimize the use of compensatory and dysfunctional movements which could lead to secondary impairments and decreased functional abilities later in life.
• During therapy sessions, more mature and typical movement patterns can be facilitated by the therapist in order to make progress in typical developmental milestones.
• Clinical assessment data creates a “picture” of the child’s functioning and indicates his or her strengths and weaknesses.
• Observation of a child completing simple tasks such as putting on a shirt, transitioning from a chair to the floor, opening containers, or playing with age appropriate toys will help a therapist identify atypical postures and movements that may be limiting functional abilities.
• Thorough assessment data are essential when working as part of a service delivery team and selection of assessment measures may be based on several factors including the child’s age, the setting (ie. home health, school system, community), and the caregiver’s and child’s specific concerns about functional limitations.

Question 33

Gross Motor Function Classification System (GMFCS)

Answer 33

LEVEL I
Walks without Limitations. Performs gross motor skills like running and jumping but speed, balance, and coordination may be impaired.

LEVEL II
Walks with limitations. This includes on uneven surface, inclines, stairs, long distances or in crowds or confined spaces.

LEVEL III
Walks Using a Hand-Held Mobility Device. Walks on even surfaces, indoors and outdoors with assistive devices. Children may use manual wheelchair for long distances.

LEVEL IV
Self-Mobility with Limitations. Child may use powered mobility or require assistance from a caregiver. May walk short distances with a mobility device but relies primarily on wheeled mobility.

LEVEL V
Transported in a Manual Wheelchair. Child has no means of independent mobility and relies on caregiver for all transportation needs.

Question 34

prognosis for walking

Answer 34

Most children will walk - 60% will walk independently (GMFCS I-II), 10% will walk with a walking aid (GMFCS III) and 30% are wheelchair users (GMFCS IV-V)
• The more severe the child’s physical, functional or cognitive impairment, the greater the possibility of difficulties with walking
• If a child can sit at 2 years of age it is probable (but not certain) that they will be able to walk unaided by age 6
• If a child cannot sit but can roll at 2 years of age, there is a possibility that they may be able to walk unaided by age 6
• If a child cannot sit or roll at 2 years of age, they are unlikely to be able to walk unaided

Question 35

Manual Ability Classification System (MACS)

Answer 35

LEVEL I Handles objects easily and successfully.

LEVEL II Handles most objects but with somewhat reduced quality and/or speed of achievement.

LEVEL III Handles objects with difficulty; needs help to prepare and/or modify activities.

LEVEL IV Handles a limited selection of easily managed objects in adapted situations.

LEVEL V Does not handle objects and has severely limited ability to perform even simple actions. Requires total assistance.

Question 36

Communication Function Classification System (CFCS) - levels

Answer 36

LEVEL I Effective Sender and Receiver with unfamiliar and familiar partners.

LEVEL II Effective but slower paced Sender and/or Receiver with unfamiliar and/or
familiar partners.

LEVEL III Effective Sender and Receiver with familiar partners.

LEVEL IV Inconsistent Sender and/or Receiver with familiar partners.

LEVEL V Seldom Effective Sender and Receiver even with familiar partners.

Question 37

prognosis for talking

Answer 37

1 in 4 children with CP will be non-verbal so it is important to assess and augment speech early
• Around 1 in 2 children with CP have some difficulty with elements of communication
• Around 1 in 3 children have specific difficulties with speech and language
• Communicative frustration can lead to behavioral disorders
• The more severe the child’s physical, functional or cognitive impairment, the greater the likelihood of difficulties with speech and language
• Uncontrolled epilepsy may be associated with difficulties with all forms of communication, including speech
• A child with bilateral spastic, dyskinetic or ataxic CP is more likely to have difficulties with speech and language than a child with unilateral spastic CP

Question 38

Eating and Drinking Ability Classification System for Individuals with CP (EDACS) - levels

Answer 38

LEVEL I Eats and drinks safely and efficiently
LEVEL II Eats and drinks safely but with some limitations to efficiency
LEVEL III Eats and drinks with some limitations to safety; there may be limitations to efficiency
LEVEL IV Eats and drinks with significant limitations to safety
LEVEL V Unable to eat or drink safely, tube feeding may be considered to provide nutrition.

Question 39

Vision Classification Systems (VFCS)

Answer 39

LEVEL I Uses visual function easily and successfully in vision related activities
LEVEL II Uses visual function successfully but needs self-initiated compensatory strategies
LEVEL III Uses visual function but needs some adaptations
LEVEL IV Uses visual function in very adapted environments but performs just part of vision-related activities
LEVEL V Does not use visual function even in very adapted
environments

Question 40

VFCS limitation

Answer 40

raters might find it hard to identify the usual performance in daily activities, as opposed to the best or the worst one.

Question 41

Modified Ashworth Scale (MAS)

Answer 41

• measurement of spasticity
  • Completed in supine if possible (most accurate and the lowest score; any tension anywhere in the body will increase spasticity)
  • Because spasticity is “velocity dependent” (the faster the limb is moved, the more spasticity is encountered), the MAS is done moving the limb at the “speed of gravity.” This is defined as the same speed a non-spastic limb would naturally drop. (fast)
  • The test is done a maximum of three times for each joint. If it is done more than three times the short-term effect of a stretch impacts the score
  • The MAS is done prior to goniometric testing. Goniometric testing provides a stretch and the short-term effect of a stretch impacts the score.

Question 42

Positions used for MAS

Answer 42

Elbow. Start position: Elbow fully flexed, forearm neutral. Movement: Extend elbow from maximum possible flexion to maximum possible extension. (Triceps would be the same position, opposite direction.)

Wrist. Start position: Elbow as straight as possible, forearm pronated. Movement: Extend the patient’s wrist from maximum possible flexion to maximum possible extension.

Fingers. Start position: Elbow as straight as possible, forearm neutral. All fingers are done at once. Movement: Extend the patient’s fingers from maximum possible flexion to maximum possible extension.

Thumb. Start position: Elbow as straight as possible, forearm neutral, wrist in neutral. Movement: Extend the thumb from maximum possible flexion (thumb against index finger) to maximum possible extension (in anatomical position, “abducted”).

Hamstrings. Start position: Prone so that ankle falls beyond end of the plinth, hip in neutral rotation. Movement: Extend the patient’s knee from maximum possible flexion to maximum possible extension.

Quadriceps. Start position: Prone so that ankle falls beyond end of the plinth, hip in neutral rotation. Movement: Flex the patient’s knee from maximum possible extension to maximum possible flexion.

Gastrocnemius. Start position: Supine, ankle plantar flexed, hip in neutral rotation and flexion. Movement: Dorsiflex the patient’s ankle from maximum possible plantar flexion to maximum possible dorsiflexion not more than three consecutive times and rate the muscle tone.

Soleus. Start position: Supine, ankle plantar flexed, hip in neutral rotation and flexion and with the knee flexed to ~15°. Movement: Dorsiflex the patient’s ankle from maximum possible plantar flexion to maximum possible dorsiflexion.