1. 2009Prim Haynes & Franjoine1 Children with Ataxia Margo Prim Haynes, MA, PT Mary Rose Franjoine, PT, DPT, MS, PCS 2009

2. Prim Haynes & Franjoine2 http://en.wikipedia.org/wiki/Cerebellum Cerebellum

3. 2009Prim Haynes & Franjoine3 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)

4. 2009Prim Haynes & Franjoine4 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

5. 2009Prim Haynes & Franjoine5

6. 2009Prim Haynes & Franjoine62009M R Franjoine & M P Haynes6 NDT Enablement Classification Model of Health and Disability DimensionFunctional DomainDisability Domain A. Body structure & functions Structural & functional integrity Impairments A.Primary B.Secondary B. Motor functionsEffective posture & movement Ineffective posture & movement C. Individual functions Functional activitiesFunctional activity limitations D. Social functionsParticipationParticipation restriction + Domains - Dimensions From: NDT Approach Theoretical Foundations & Principles of Clinical Practice, Table 2.1 pg. 82

7. 2009Prim Haynes & Franjoine7 Video

8. 2009Prim Haynes & Franjoine8 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

9. 2009Prim Haynes & Franjoine9 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.

10. 2009Prim Haynes & Franjoine10 Cognition Functions: Cognitive challenges Communicates Impairments: Cognitive challenges include processing problems & motor planning Communication concerns: articulation issues

11. 2009Prim Haynes & Franjoine11 Cognition Impairments: Emotional inconsistencies Fearful of movement Perceived as shy and unsociable –Bland affect

12. 2009Prim Haynes & Franjoine12 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

13. 2009Prim Haynes & Franjoine13 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

14. 2009Prim Haynes & Franjoine14 Neuromuscular System Impairment of Timing and Sequencing Lack of coordination between agonist and antagonist muscles –Overshoot- Dysmetria –Latency response

15. 2009Prim Haynes & Franjoine15 Neuromuscular Insufficient Force Generation (muscle strength) Postural Muscles Movement Muscles

16. 2009Prim Haynes & Franjoine16 Sensory System Sensory Processing Impairment fluctuates: –Hypo-sensitive –Hyper-sensitive –Gravitational Insecurity Poor motor planning

17. 2009Prim Haynes & Franjoine17 Musculoskeletal System Secondary Impairments Rib cage mobility may lead to upper respiratory problems Feet position in prontation may lead to foot problems

18. 2009Prim Haynes & Franjoine182009M R Franjoine & M P Haynes18 NDT Enablement Classification Model of Health and Disability DimensionFunctional DomainDisability Domain A. Body structure & functions Structural & functional integrity Impairments A.Primary B.Secondary B. Motor functionsEffective posture & movement Ineffective posture & movement C. Individual functions Functional activitiesFunctional activity limitations D. Social functionsParticipationParticipation restriction + Domains - Dimensions From: NDT Approach Theoretical Foundations & Principles of Clinical Practice, Table 2.1 pg. 82

19. 2009Prim Haynes & Franjoine19 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

20. 2009Prim Haynes & Franjoine20 Posture and Movement 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

21. 2009Prim Haynes & Franjoine21 Posture and Movement 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)

22. 2009Prim Haynes & Franjoine22 Posture and Movement Balance insufficient to prevent from falling As Speed ↑ see ↓ in accuracy and adaptability of movement Prefers sagittal plan movements

23. 2009Prim Haynes & Franjoine23 Prone Postures: Not a position for function because of pull of gravity Movement Initiates movement with phasic bursts

24. 2009Prim Haynes & Franjoine24 Pictures

25. 2009Prim Haynes & Franjoine25 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)

26. 2009Prim Haynes & Franjoine26 Pictures

27. 2009Prim Haynes & Franjoine27 Sitting Position 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

28. 2009Prim Haynes & Franjoine28 Pictures

29. 2009Prim Haynes & Franjoine29 Mobility in Quadruped Posture: 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

30. 2009Prim Haynes & Franjoine30 Pictures

31. 2009Prim Haynes & Franjoine31 Kneeling Posture Hips in increased flexion and abduction supporting the wide BOS (pelvis anterior or posterior) Movement Stabilize with upper body to move

32. 2009Prim Haynes & Franjoine32 Pictures

33. 2009Prim Haynes & Franjoine33 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

34. 2009Prim Haynes & Franjoine34 Pictures

35. 2009Prim Haynes & Franjoine352009M R Franjoine & M P Haynes35 NDT Enablement Classification Model of Health and Disability DimensionFunctional DomainDisability Domain A. Body structure & functions Structural & functional integrity Impairments A.Primary B.Secondary B. Motor functionsEffective posture & movement Ineffective posture & movement C. Individual functions Functional activitiesFunctional activity limitations D. Social functionsParticipationParticipation restriction + Domains - Dimensions From: NDT Approach Theoretical Foundations & Principles of Clinical Practice, Table 2.1 pg. 82

36. 2009Prim Haynes & Franjoine36 Activities & Activities Limitation Locomotor SkillsAmbulatory with or without assistance CommunicatesCommunicates without assistance Basic ADL’sTypically independent with ADL or needs occasional assistance

37. 2009Prim Haynes & Franjoine372009M R Franjoine & M P Haynes37 NDT Enablement Classification Model of Health and Disability DimensionFunctional DomainDisability Domain A. Body structure & functions Structural & functional integrity Impairments A.Primary B.Secondary B. Motor functionsEffective posture & movement Ineffective posture & movement C. Individual functions Functional activitiesFunctional activity limitations D. Social functionsParticipationParticipation restriction + Domains - Dimensions From: NDT Approach Theoretical Foundations & Principles of Clinical Practice, Table 2.1 pg. 82

38. 2009Prim Haynes & Franjoine38 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

39. 2009Prim Haynes & Franjoine39 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

40. 2009Prim Haynes & Franjoine40 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

41. 2009Prim Haynes & Franjoine41 Video

42. 2009Prim Haynes & Franjoine42 Children with Ataxia