2009prim haynes & franjoine1 children with ataxia margo prim haynes, ma, pt mary rose franjoine,...

2009 Prim Haynes & Franjoine 1

Children with Ataxia

• Margo Prim Haynes, MA, PT• Mary Rose Franjoine, PT, DPT, MS, PCS

• 2009


http://en.wikipedia.org/wiki/Cerebellum

Cerebellum


Role of Cerebellum• Integration of sensory perception,

coordination and motor control• Neural palthways from cerebellum:

– Link with motor cortex telling muscles to move

– Link with spinocerebellar track proving proprioceptive feedback on position of body in space

• Fine tunes motor movement (feedback)


General Comment• Children with ataxia have damage to

cerebellum• Cerebellum’s inputs & outputs connected

to motor cortex & brainstem are faulty• Specific systems vary with area of

cerebellum that is affected• Ataxia often seen in combination with

spasticity and athetosis

2009 Prim Haynes & Franjoine 62009 M R Franjoine & M P Haynes 6

NDT Enablement Classification Model of Health and Disability

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 -

Dim

ensi

ons

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


Video


Body Structure & Body Function• Cerebellum Damage • Damage to Structure:

– Interferes with Cerebellum ability to function• Controls execution of movement –

Corrects for deviations• Modulates muscle stiffness


Body Structure & Body Function

– Interferes with Cerebellum ability to function• Computes position of body segments • Involved in motor timing and

sequencing• Provides appropriate force during

rapid sequential movement.


Cognition Functions:• Cognitive challenges • Communicates

Impairments:• Cognitive challenges include processing

problems & motor planning• Communication concerns: articulation

issues


Cognition

Impairments:• Emotional inconsistencies

• Fearful of movement

• Perceived as shy and unsociable

– Bland affect


Neuromuscular System

Impaired Muscle Activation

• Co-activation from moderate to low (stiffness fluctuates from moderate to low) during task

• Oscillations of trunk, hands and tongue: small amplitude and large frequency


Neuromuscular System Impaired Muscle Activation• Latency in initiating, sustaining and

terminating postural muscle activity during tasks

• Impaired muscle synergies– Stereotyped patterns of movement

due to limited movement repertories


Neuromuscular System

Impairment of Timing and Sequencing

• Lack of coordination between agonist and antagonist muscles

– Overshoot- Dysmetria

– Latency response


Neuromuscular

Insufficient Force Generation (muscle strength)

• Postural Muscles

• Movement Muscles


Sensory System

• Sensory Processing Impairment fluctuates:

– Hypo-sensitive

– Hyper-sensitive

– Gravitational Insecurity

• Poor motor planning


Musculoskeletal System

Secondary Impairments

• Rib cage mobility may lead to upper respiratory problems

• Feet position in prontation may lead to foot problems






Impairments

A.Primary

B.Secondary






+ Domains -

Dim

ensi

ons



Posture and Movement

General Characteristics: Posture• Underlying postural tone low to

moderately low with fluctuations• Hyper mobile Joint Structure (elbows &

knees) for stability• Poor midline orientation =mild

asymmetry • Use visual fixes



General Characteristics: Posture• Alignment:

– Lock distal extremities into end ranges for stability

– Anterior or posterior position of pelvic for increased stability

• Wide BOS helps stabilize & lower COG so postural muscles do not have to work



General Movement Characteristics

• Moves with small amplitude phasic bursts of extension or flexion

• Initiates movement with cervical extension and upper body

• Prefer small amplitude small range movement (characteristic of fluctuating tone)



• Balance insufficient to prevent from falling

• As Speed ↑ see ↓ in accuracy and adaptability of movement

• Prefers sagittal plan movements


PronePostures:

• Not a position for function because of pull of gravity

Movement

• Initiates movement with phasic bursts


Pictures


Supine

Postures:

• Learns to function in this position because feels safe and close to surface

Movement

• Push off surface with cervical extension and upper body work (slight asymmetrical)


Pictures


SittingPosition

• Sitting is easier position to function

• Independent sitting (ring sit, long sit & W sit) with wide BOS

Movement

• Phasic bursts of head & neck extension before pushing with arms

• Prefer sagittal plan movements


Pictures


Mobility in QuadrupedPosture:• Alignment: arms internally rotated

elbows hyperextend, weight bearing on hand with wide BOS

Movement: • Bunny hop or creeps (small excursions)

=pelvis behind knees • Move in phasic bursts


Pictures


Kneeling

Posture

• Hips in increased flexion and abduction supporting the wide BOS (pelvis anterior or posterior)

Movement

• Stabilize with upper body to move


Pictures


Standing & Walking

Postures

• Often independent standers but prefer a support surface for Upper Extremities

• Uses wide BOS, knees hyper-extended or flexed to assist with stability

Movement

• Staggering movement

• Latency response interferes with reaction time


Pictures






Impairments

A.Primary

B.Secondary






+ Domains -

Dim

ensi

ons



Activities & Activities Limitation Locomotor Skills Ambulatory with or

without assistance

Communicates Communicates without assistance

Basic ADL’s Typically independent with ADL or needs occasional assistance






Impairments

A.Primary

B.Secondary






+ Domains -

Dim

ensi

ons



Participation

• Due to cognitive ability and motor ability often need assistance in school

• Need support to complete high school years and hold down a job

• May need a group living arrangement or live with family member in adult years


Treatment Strategies

• Alignment of BOS from wide to narrow for efficient activation

• “Awaken” postural system and wait for response

• Emphasize diagonal and rotational postures and movement


Treatment Comments

1. Gravitationally insecure

2. Does not enjoy movement

3. Stabilizes with eyes so remember this when treat in front of a mirror

4. Patience important


Video


Children with Ataxia

2009prim haynes & franjoine1 children with ataxia margo prim haynes, ma, pt mary rose franjoine,...

Documents

p haynes

ataxiamargo prim haynes

motor timing

motor cortex brainstem

motor controlneural

postural muscle activity

position of body segments

cerebellum ability