Children with Hypertonia

What is Cerebral Palsy (CP)? A group of disorders impacting the development of movement and posture Results in activity limitation Accompanied by disturbances of: Sensation Cognition Communication Perception Possibly behavior and/or seizure disorder (Campbell S 2000, Miller F 2005, Bax M 2005 )

What is Cerebral Palsy (CP)? 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 )

Categories of CP Spastic or Hypertonic CP Ataxia Athetosis Hypotonia Hemiplegia Diplegia Quadriplegia Ataxia Athetosis Hypotonia

NDT Enablement Classification Model of Health and Disability + Domains - Dimension Functional Domain Disability Domain A. Body structure & functions Structural & functional integrity Impairments Primary Secondary B. Motor functions Effective posture & movement Ineffective posture & movement C. Individual functions Functional activities Functional activity limitations D. Social functions Participation Participation restriction Dimensions From: NDT Approach Theoretical Foundations & Principles of Clinical Practice, Table 2.1 pg. 82 M R Franjoine & M P Haynes

NDT Enablement Classification Model of Health and Disability + Domains - Dimension Functional Domain Disability Domain A. Body structure & functions Structural & functional integrity Impairments Primary Secondary B. Motor functions Effective posture & movement Ineffective posture & movement C. Individual functions Functional activities Functional activity limitations D. Social functions Participation Participation restriction Dimensions From: NDT Approach Theoretical Foundations & Principles of Clinical Practice, Table 2.1 pg. 82 M R Franjoine & M P Haynes

Body Structure and Functions Spastic Diplegia 41.5 Spastic Hemiplegia 36.4% Dyskinesia or Athetosis 10% Spastic Quadriplegia 7.3% Ataxia 5% (Campbell S 2000)

Body Structure and Functions Lesion(s) impacts: the motor cortex and/or white matter projections to and from cortical sensorimotor areas of the brain Causes: Unknown prenatal condition Asphyxia Prematurity Intracranial bleeds, infection, medical conditions (Campbell S 2000)

Body Structure and Functions Cognition Neuromuscular System Sensory System Musculoskeletal System Regulatory Gastrointestinal Cardiopulmonary Integumentary

VARIABLE CHILD BY CHILD Cognition VARIABLE CHILD BY CHILD Dependent on: Lesion(s) Secondary effects of various systems Seizures Access to environment

What is muscle “tone”? Neuromuscular System 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? Neuromuscular System 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)

Neuromuscular System What is Spasticity? Resistance to rapid muscle stretch Velocity dependent The phasic component of hypertonus Often associated with: Upper Motor Neuron Syndrome (UMNS) Hyperactive deep tendon reflexes Clonus (Kandel 2000)

Neuromuscular System --Primary Impairments Impaired Muscle Activation Excessive Co-activation Impaired Muscle Synergies Inability to Initiate, Sustain, Terminate

Typical Co-activation Simultaneous 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-activation Atypical Excessive Co-activation Decreases movement speed Limits flexibility of movement responses Increased energy costs and fatigue

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Typical Muscle Synergies A group of muscles working together across multiple joints and organized to act as a functional unit Simplifies the work of the CNS Strengthens with repetition

Atypical Impaired Muscle Synergies Based on limited movement repertoires Difficult to vary or adapt to meet the requirements of different tasks Produces stereotypical movement patterns

Atypical Impaired Muscle Synergies Movements are limited in amount and frequency Movements tend to be in more limited ranges

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Typical 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

Atypical 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

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Neuromuscular System Impaired Motor Execution Impaired Modulation and Scaling of Forces Impaired Timing and Sequencing Excessive overflow of Intra-Interlimb contractions

Typical Modulation and Scaling of Forces Controlled acceleration or deceleration Using the proper amount of force Constant balancing of agonists and antagonists during movement

Atypical Impaired Modulation and Scaling of Forces Inability to slow down as they approach a target Reduces accuracy (overshoots) Particular difficulties grading grip

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Typical Timing and Sequencing

Atypical Impaired Timing and Sequencing Unable to turn on and off muscles or patterns of muscles at the appropriate times i.e. agonist and antagonist coordination i.e. the hamstrings during gait Incorrect sequence of activation for a task

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Typical 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

Atypical Excessive overflow of Intra-Interlimb contractions Bobaths described “associated movements” i.e. while grasping with one arm, will posture with the other i.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

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Neuromuscular System Impaired Force Generation Strength: the ability to contract a muscle to a sufficient degree to impact the task Can be masked by tone and spasticity Primary—impaired input from motor pathways Secondary– atrophy and resultant fiber type and connective tissue changes Postural Movement system

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Neuromuscular System Anticipatory Postural Control TYPICAL: ATYPICAL: Postural set prior to initiating a task or movement ATYPICAL: Posture is not linked to movement Fail to anticipate postural needs prior to a movement or task Fail to generate adequate proximal posture for distal function

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Neuromuscular System Poverty of Movement TYPICAL: ATYPICAL: Large variety of movement repertoires to chose from Can easily adapt and modify repertoires for the task Movements are fluid, flexible, and complex ATYPICAL: Movement repertoires are limited in number “Stereotypic” Repertoires are difficult to change Adapt poorly to various tasks

Neuromuscular System Fractionated or Dissociated Movements TYPICAL: “Isolated movement” or “dissociated movement” ATYPICAL: Difficulty isolating movement Segment to segment Inter-limb Intra-limb Limbs from trunk

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Sensory System Vision Vestibular Somatosensory

Vision 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 problems Field cuts

Vision Ghasia, Brunstrom, Gordon & Tychsen, 2008 GMFCS levels I and II Similar to typically developing children Strabismus Amblyopia (lazy eye) GMFCS levels III to V More severe deficits Deficits not observed in typically developing children i.e. dyskinetic strabismus and Cerebral Visual Impairment

Vestibular Difficult to separate from vision and postural control More impact seen in SQ than SD

Somatosensory Clearly atypical yet difficult to truly assess Propioception Kinesthetic awareness 2 point discrimination Stereognosis

Sensory Processing “ 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)

Musculoskeletal System Considerable secondary impairments Bone: Boney deformities 2° atypical muscle pull Decreased bone density of long bones (FX)

Musculoskeletal System Considerable secondary impairments Dislocations: 2° to atypical muscle pull and atypical bone shape formation Impacts many joints from jaw to foot Scoliosis and rib cage deformities

Musculoskeletal System Considerable secondary impairments Muscle: Shortening and contracture Fiber type shift Weakness Connective tissue: Increased stiffness due to atypical matrix within muscle Over-lengthening or shortening of tendons

NDT Enablement Classification Model of Health and Disability + Domains - Dimension Functional Domain Disability Domain A. Body structure & functions Structural & functional integrity Impairments Primary Secondary B. Motor functions Effective posture & movement Ineffective posture & movement C. Individual functions Functional activities Functional activity limitations D. Social functions Participation Participation restriction Dimensions From: NDT Approach Theoretical Foundations & Principles of Clinical Practice, Table 2.1 pg. 82 M R Franjoine & M P Haynes

Motor Functions Effective Posture and Movement Ineffective Posture and Movement

Posture and Movement “Observable conditions that are neither functional limitations nor system impairments” (Howle 2002) Alignment of body segments COM over BOS Weight shift Quality of movement (fast, slow)

The Child with Spastic Quadriplegia

General Characteristics of Posture in Children with SQ Little variation Influenced by limited joint range Tone influenced by position in space High tone extremities, low tone trunk

General Characteristics of Posture in Children with SQ Uses increased tone to gain stability Eyes adjust to posture rather than posture being driven by vision

General Characteristics of Movement in Children with SQ Limited amount Limited variety Stiffen extremities to attain postural stability Poor dissociation

General Characteristics of Movement in Children with SQ 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

Prone Posture Movement Difficult position Pulled into gravity Flexor tone is biased (typical) Lack of joint range Movement Asymmetrical neck hyperextension OR Not enough antigravity strength to lift head

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Supine Posture Movement Preferred position Stable Have the possibility of viewing the world Extensors are biased (typical) Movement Sometimes can kick reciprocally Push with neck hyperextension asymmetrically and may arch with body

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Sitting Posture Movement Very unstable position Narrow base Stabilization efforts create non-functional alignment of hips, spine, neck, and head Movement Too unstable to seek movement Increased full body stiffness when movement is initiated

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Quadruped Posture Movement Rarely can hold without assistance Often can’t move at all May pull with both arms—”combat crawl” Occasional child may “bunny hop”

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Standing and Walking Posture Movement Placed in standing Base too narrow--adducted legs and plantar-flexed ankles Movement Stiffens whole body to gain stability May support weight stiffly and then both “give” Some may have reciprocal movements of legs

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NDT Enablement Classification Model of Health and Disability + Domains - Dimension Functional Domain Disability Domain A. Body structure & functions Structural & functional integrity Impairments Primary Secondary B. Motor functions Effective posture & movement Ineffective posture & movement C. Individual functions Functional activities Functional activity limitations D. Social functions Participation Participation restriction Dimensions From: NDT Approach Theoretical Foundations & Principles of Clinical Practice, Table 2.1 pg. 82 M R Franjoine & M P Haynes

Activities and Activity Limitations 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

NDT Enablement Classification Model of Health and Disability + Domains - Dimension Functional Domain Disability Domain A. Body structure & functions Structural & functional integrity Impairments Primary Secondary B. Motor functions Effective posture & movement Ineffective posture & movement C. Individual functions Functional activities Functional activity limitations D. Social functions Participation Participation restriction Dimensions From: NDT Approach Theoretical Foundations & Principles of Clinical Practice, Table 2.1 pg. 82 M R Franjoine & M P Haynes

Participation Inclusion in family activities and outings difficult Special classroom or mainstreamed with full-time paraprofessional Participation is often limited due to: Lack of interaction and communication with others Wheelchair access Transportation that accommodates WC

Treatment Strategies Address decreased ROM Lengthen Incorporate into your activities Accompany with activation and/or Compensate Work upright whenever possible

Treatment Strategies Help establish an appropriate BOS ALIGNMENT! Usually need to widen ALIGNMENT! In relation to BOS Segment to segment Decrease asymmetries Activate the trunk to free the extremities Balanced flexors and extensors in the trunk Work in the frontal and transverse planes

Treatment Strategies Keep them moving! Repetition May need large ranges to know where they are BUT They can only control small ranges themselves Repetition Motor learning Strength

Treatment Comments Empower these children!!! Treat age appropriately despite motor abilities Treat upright whenever possible Forget the developmental sequence!!! Attend to ALL the systems…Many impact their health and quality of life Have a long term perspective

The Child with Spastic Diplegia

General Characteristics of Posture in Children with SD Upper extremities show more variability than LEs UE posturing may increase in unstable situations Uses UEs for support in upright instead of trunk

General Characteristics of Posture in Children with SD LEs: Hip adduction, internal rotation, knee flexion, ankle plantar-flexion LEs influenced by limited joint range

General Characteristics of Movement in Children with SD Active children Pull themselves around with their arms Poor dissociation of LEs from trunk and from each other

General Characteristics of Movement in Children with SD 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

Prone Posture Movement Tends to stay on elbows until going to 4s Lack of joint range at hips increases anterior tilt of pelvis and stresses T-L joint Movement Lateral weightshifts are limited Pulls with arms to move forward Pushes with arms and keeps legs stiff to roll

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Supine Posture Movement Plays with hands LEs are stiff in hip flexion/adduction, internal rotation, knee flexion, ankle plantar-flexion Movement Reciprocal kicking, poorly graded Very mild children may have hands to knee and hands to feet play Move into and out of position using UEs

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Sitting Posture Movement Like to sit Narrow base is unstable so they seek “W” sit Support with UEs Movement Get into and out of sitting in the sagittal plane In “W” sitting, use both hands for play

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Kneel Posture Movement Hips flexed, anterior tilt to pelvis, lumbar lordosis UEs either used for support or posture to increase trunk stability Ankles often dorsiflex Movement Use this as a transition position to extend both legs and attain standing Stay in sagittal plane, lateral weight shifts difficult

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Quadruped Posture Movement Difficulty controlling midrange hip and knee ranges so “sits” on heels Movement “Bunny Hop” Moves both UEs together then both LEs together

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Standing and Walking Posture Base narrow--adducted legs and plantar-flexed ankles Supports on UEs Often “sinks” to foot flat during quiet standing Can’t stand still

Standing and Walking Movement Reciprocal movements of LEs—often abrupt Difficulty dissociating LEs from each other LE posturing may increase during gait Difficulty with lateral weight-shift so often use trunk

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NDT Enablement Classification Model of Health and Disability + Domains - Dimension Functional Domain Disability Domain A. Body structure & functions Structural & functional integrity Impairments Primary Secondary B. Motor functions Effective posture & movement Ineffective posture & movement C. Individual functions Functional activities Functional activity limitations D. Social functions Participation Participation restriction Dimensions From: NDT Approach Theoretical Foundations & Principles of Clinical Practice, Table 2.1 pg. 82 M R Franjoine & M P Haynes

Activities and Activity Limitations 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

NDT Enablement Classification Model of Health and Disability + Domains - Dimension Functional Domain Disability Domain A. Body structure & functions Structural & functional integrity Impairments Primary Secondary B. Motor functions Effective posture & movement Ineffective posture & movement C. Individual functions Functional activities Functional activity limitations D. Social functions Participation Participation restriction Dimensions From: NDT Approach Theoretical Foundations & Principles of Clinical Practice, Table 2.1 pg. 82 M R Franjoine & M P Haynes

Participation Function well as a member of the family though may difficulty keeping up with siblings Special classroom or mainstreamed. Participation is often limited due to: Fatigue Speed and balance Crutch, Walker, and WC access Community accessabilty Acceptance of peers

Treatment Strategies Address decreased ROM Lengthen Incorporate into your activities Accompany with activation and/or Compensate Help establish an appropriate BOS Usually need to widen

Treatment Strategies ALIGNMENT! Get the trunk moving over the hip In relation to BOS Segment to segment Decrease asymmetries Get the trunk moving over the hip Balanced flexors and extensors in the trunk Work in the frontal and transverse planes

Treatment Strategies Keep the LEs dissociated from each other Work for midrange control and eccentric control Repetition Motor learning Strength

Treatment Comments 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