mcelroy, haynes, & franjoine 2009. a group of disorders impacting the development of movement...

McElroy, Haynes, & Franjoine 2009


A group of disorders impacting the development of movement and posture

Results in activity limitation

Accompanied by disturbances of:SensationCognitionCommunicationPerceptionPossibly behavior and/or seizure disorder

(Campbell S 2000, Miller F 2005, Bax M 2005 )


• Caused by a non-progressive defect or lesion occurring in an immature brain

• Insult occurs before or after birth

• Single or multiple locations

(Campbell S 2000, Miller F 2005, Bax M 2005 )


Spastic or Hypertonic CPHemiplegiaDiplegiaQuadriplegia

• Ataxia• Athetosis• Hypotonia

McElroy, Haynes, & Franjoine 2009M R Franjoine & M P Haynes

Dimension Functional Domain Disability Domain

A. Body structure & functions

Structural & functional integrity

Impairments

A.Primary

B.Secondary

B. Motor functions Effective posture & movement

Ineffective posture & movement

C. Individual functions

Functional activities Functional activity limitations

D. Social functions Participation Participation restriction

+ Domains -

From: NDT Approach Theoretical Foundations & Principles of Clinical Practice, Table 2.1 pg. 82


Spastic Diplegia 41.5

Spastic Hemiplegia 36.4%

Dyskinesia or Athetosis 10%

Spastic Quadriplegia 7.3%

Ataxia 5%

(Campbell S 2000)


Lesion(s) impacts: the motor cortex

and/or white matter projections to and from cortical sensorimotor areas of the brain

Causes:Unknown prenatal conditionAsphyxiaPrematurity

Intracranial bleeds, infection, medical conditions(Campbell S 2000)


Body Structure and FunctionsBody Structure and Functions

CognitionNeuromuscular SystemSensory SystemMusculoskeletal SystemRegulatoryGastrointestinalCardiopulmonaryIntegumentary


VARIABLE CHILD BY CHILD

Dependent on:• Lesion(s)• Secondary effects of various systems• Seizures• Access to environment


What is muscle “tone”?• Amount of tension in a resting muscle• Resists being lengthened• Has neural components• Has mechanical and elastic components;

muscle and connective tissue

(Lundy-Ekman 2002, Kandel 2000)


What is Hypertonicity?• Abnormally high resting tension• An abnormally high resistance to being

lengthened• Still has both neural and mechanical

components• The tonic component of hypertonus

(Crenna 1998, Lundy-Ekman 2002, Kandel 2000)


What is Spasticity?Resistance to rapid muscle stretchVelocity dependentThe phasic component of hypertonusOften associated with:

Upper Motor Neuron Syndrome (UMNS)Hyperactive deep tendon reflexesClonus (Kandel 2000)


Excessive Co-activation

Impaired Muscle Synergies

Impaired Muscle Activation

Inability to Initiate, Sustain, Terminate


Co-activationSimultaneous activation of agonists and antagonists at a joint influencing movement in the same plane

Normally used to increase joint stability or for proximal stability to support precise distal movements

Allows for graded movement


Excessive Co-activationDecreases movement speed

Limits flexibility of movement responses

Increased energy costs and fatigue


Muscle SynergiesA group of muscles working together across multiple joints and organized to act as a functional unitSimplifies the work of the CNSStrengthens with repetition


Impaired Muscle SynergiesBased on limited movement repertoiresDifficult to vary or adapt to meet the requirements of different tasksProduces stereotypical movement patterns


Impaired Muscle SynergiesMovements are limited in amount and frequencyMovements tend to be in more limited ranges


Initiate, Sustain, Terminate

Quick response of muscles to the decision to move

Easily maintain posture against gravity

Relax muscles

Quick response of muscles to the decision to cease movement


Difficulty with Initiate, Sustain, Terminate

Delay between desire to activate and ability to initiate muscular movement (latency)

Difficulty holding against gravity…especially postural muscles

Can’t turn off muscles in time


• Impaired Motor Execution

Impaired Modulation and Scaling of Forces

Impaired Timing and Sequencing

Excessive overflow of Intra-Interlimb contractions


Modulation and Scaling of Forces

Controlled acceleration or deceleration

Using the proper amount of force

Constant balancing of agonists and antagonists during movement


Impaired Modulation and Scaling of Forces

Inability to slow down as they approach a target

Reduces accuracy (overshoots)

Particular difficulties grading grip


Timing and Sequencing


Impaired Timing and Sequencing

Unable to turn on and off muscles or patterns of muscles at the appropriate times

i.e. agonist and antagonist coordinationi.e. the hamstrings during gait

Incorrect sequence of activation for a task


Overflow of Intra-Interlimb contractions

When learning a new skill

With increased effort

Within a limb or elsewhere in the body

Decreases as proficiency is gained

Can be actively overridden


Excessive overflow of Intra-Interlimb contractions

Bobaths described “associated movements”i.e. while grasping with one arm, will posture with the otheri.e. when flexing the hip, the ankle dorsiflexes

Occur at times similar to typical but with a generally lower threshold

Decreases the capacity for isolated control during effort


Impaired Force Generation

Strength: the ability to contract a muscle to a sufficient degree to impact the task

Can be masked by tone and spasticityPrimary—impaired input from motor pathwaysSecondary– atrophy and resultant fiber type and connective tissue changes

Postural

Movement system


Anticipatory Postural Control

TYPICAL: Postural set prior to initiating a task or movement

ATYPICAL: Posture is not linked to movementFail to anticipate postural needs prior to a movement or taskFail to generate adequate proximal posture for distal function


Poverty of Movement

TYPICAL:Large variety of movement repertoires to chose fromCan easily adapt and modify repertoires for the taskMovements are fluid, flexible, and complex

ATYPICAL:Movement repertoires are limited in number“Stereotypic”Repertoires are difficult to change Adapt poorly to various tasks


Fractionated or Dissociated Movements

TYPICAL:“Isolated movement” or “dissociated movement”

ATYPICAL:Difficulty isolating movement

Segment to segmentInter-limbIntra-limbLimbs from trunk


Vision

Vestibular

Somatosensory


Varies greatly

Cortical blindness to refractory errors

Strabismus (eyes not properly aligned)

Esotropia (the eye turns in)Exotropia (the eye turns out)

Nystagmus

Visual tracking problemsField cuts


Ghasia, Brunstrom, Gordon & Tychsen, 2008

GMFCS levels I and IISimilar to typically developing childrenStrabismusAmblyopia (lazy eye)

GMFCS levels III to VMore severe deficitsDeficits not observed in typically developing children i.e. dyskinetic strabismus and Cerebral Visual Impairment


Difficult to separate from vision and postural controlMore impact seen in SQ than SD


Clearly atypical yet difficult to truly assess

PropioceptionKinesthetic awareness2 point discriminationStereognosis


“ the ability of the nervous system to perceive, interpret, modulate, and organize sensory input for use in generating or adapting motor responses… (Miller & Lane 2000)


Considerable secondary impairmentsBone:

Boney deformities 2° atypical muscle pullDecreased bone density of long bones (FX)


Considerable secondary impairments

Dislocations: 2° to atypical muscle pull and atypical bone shape formationImpacts many joints from jaw to foot

Scoliosis and rib cage deformities


Considerable secondary impairmentsMuscle:

Shortening and contractureFiber type shiftWeakness

Connective tissue: Increased stiffness due to atypical matrix within muscleOver-lengthening or shortening of tendons





Impairments

A.Primary

B.Secondary






+ Domains -



Motor FunctionsMotor Functions

Effective Posture and Movement

Ineffective Posture and Movement


• Alignment of body segments• COM over BOS• Weight shift• Quality of movement (fast, slow)

“Observable conditions that are neither functional limitations nor system impairments” (Howle 2002)


Little variation

Influenced by limited joint range

Tone influenced by position in space

High tone extremities, low tone trunk


Uses increased tone to gain stability

Eyes adjust to posture rather than posture being driven by vision


Limited amount

Limited variety

Stiffen extremities to attain postural stability

Poor dissociation


Trunk moves with either flexors or extensors

Most active movement is in sagittal plane

Difficulty organizing movements in relation to the BOS

Uses eyes and mouth to increase postural stability


PostureDifficult position

Pulled into gravityFlexor tone is biased (typical)Lack of joint range

MovementAsymmetrical neck hyperextension OR

Not enough antigravity strength to lift head


PosturePreferred position

StableHave the possibility of viewing the worldExtensors are biased (typical)

MovementSometimes can kick reciprocallyPush with neck hyperextension asymmetrically and may arch with body


PostureVery unstable positionNarrow baseStabilization efforts create non-functional alignment of hips, spine, neck, and head

MovementToo unstable to seek movementIncreased full body stiffness when movement is initiated


PostureRarely can hold without assistance

MovementOften can’t move at allMay pull with both arms—”combat crawl”Occasional child may “bunny hop”


PosturePlaced in standingBase too narrow--adducted legs and

plantar-flexed ankles

MovementStiffens whole body to gain stabilityMay support weight stiffly and then both “give”Some may have reciprocal movements of legs





Impairments

A.Primary

B.Secondary






+ Domains -



LOCOMOTOR SKILLS

• Weightbearing assisted transfers will improve quality of life• Motorized mobility if possible

COMMUNICATION • Establish early and simple communication• Think AT

BASIC ADL’S • Fully dependent in most skills• Full time assistance• Respite care for families





Impairments

A.Primary

B.Secondary






+ Domains -



Inclusion in family activities and outings difficultSpecial classroom or mainstreamed with full-time paraprofessionalParticipation is often limited due to:

Lack of interaction and communication with othersWheelchair accessTransportation that accommodates WC


Address decreased ROMLengthen

Incorporate into your activitiesAccompany with activation

and/orCompensate

Work upright whenever possible


Help establish an appropriate BOSUsually need to widen

ALIGNMENT!In relation to BOSSegment to segmentDecrease asymmetries

Activate the trunk to free the extremitiesBalanced flexors and extensors in the trunkWork in the frontal and transverse planes


Keep them moving!May need large ranges to know where they are

BUTThey can only control small ranges themselves

RepetitionMotor learningStrength


Empower these children!!!

Treat age appropriately despite motor abilities

Treat upright whenever possibleForget the developmental sequence!!!

Attend to ALL the systems…Many impact their health and quality of lifeHave a long term perspective


Upper extremities show more variability than LEs

UE posturing may increase in unstable situations

Uses UEs for support in upright instead of trunk


LEs: Hip adduction, internal rotation, knee flexion, ankle plantar-flexion

LEs influenced by limited joint range


Active children

Pull themselves around with their arms

Poor dissociation of LEs from trunk and from each other


Most active movement is in sagittal plane

Quadruped and walking progression is achieved by moving the COM outside the BOS

Walking speed is achieved by using LE spasticity instead of strength


PostureTends to stay on elbows until going to 4sLack of joint range at hips increases anterior tilt of pelvis and stresses T-L joint

MovementLateral weightshifts are limitedPulls with arms to move forwardPushes with arms and keeps legs stiff to roll


PosturePlays with handsLEs are stiff in hip flexion/adduction, internal rotation, knee flexion, ankle plantar-flexion

MovementReciprocal kicking, poorly gradedVery mild children may have hands to knee and hands to feet play Move into and out of position using UEs


PostureLike to sitNarrow base is unstable so they seek “W” sitSupport with UEs

MovementGet into and out of sitting in the sagittal planeIn “W” sitting, use both hands for play


PostureHips flexed, anterior tilt to pelvis, lumbar lordosisUEs either used for support or posture to increase trunk stabilityAnkles often dorsiflex

MovementUse this as a transition position to extend both legs and attain standingStay in sagittal plane, lateral weight shifts difficult


PostureDifficulty controlling midrange hip and knee ranges so “sits” on heels

Movement“Bunny Hop”Moves both UEs together then both LEs together


PostureBase narrow--adducted legs and plantar-flexed ankles

Supports on UEs

Often “sinks” to foot flat during quiet standing

Can’t stand still


MovementReciprocal movements of LEs—often abruptDifficulty dissociating LEs from each other

LE posturing may increase during gait

Difficulty with lateral weight-shift so often use trunk





Impairments

A.Primary

B.Secondary






+ Domains -



LOCOMOTOR SKILLS

• Usually walk (with & without assistive devices)• WC may be necessary for energy conservation

COMMUNICATION • Usually communicate without difficulty

BASIC ADL’S • Many become independent





Impairments

A.Primary

B.Secondary






+ Domains -



Function well as a member of the family though may difficulty keeping up with siblingsSpecial classroom or mainstreamed. Participation is often limited due to:

FatigueSpeed and balanceCrutch, Walker, and WC accessCommunity accessabiltyAcceptance of peers


Address decreased ROMLengthen

Incorporate into your activitiesAccompany with activation

and/orCompensate

Help establish an appropriate BOSUsually need to widen


ALIGNMENT!In relation to BOSSegment to segmentDecrease asymmetries

Get the trunk moving over the hipBalanced flexors and extensors in the trunkWork in the frontal and transverse planes


Keep the LEs dissociated from each other

Work for midrange control and eccentric control

RepetitionMotor learningStrength


Protect their hands and other joints

Remember biomechanics when they get on their feet

Increased function increases risk of deformities

Have a long term perspective

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