Children with Ataxia

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

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

3. 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) Prim Haynes & Franjoine

4. 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 Prim Haynes & Franjoine

5. Prim Haynes & Franjoine

6. NDT Enablement Classification Model of Health and Disability + Domains - Dimensions From: NDT Approach Theoretical Foundations & Principles of Clinical Practice, Table 2.1 pg. 82 2009 Prim Haynes & Franjoine M R Franjoine & M P Haynes 6

7. Video Prim Haynes & Franjoine

8. 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 Prim Haynes & Franjoine

9. 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. Prim Haynes & Franjoine

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

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

12. 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 Prim Haynes & Franjoine

13. 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 Prim Haynes & Franjoine

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

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

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

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

18. NDT Enablement Classification Model of Health and Disability + Domains - Dimensions From: NDT Approach Theoretical Foundations & Principles of Clinical Practice, Table 2.1 pg. 82 2009 Prim Haynes & Franjoine M R Franjoine & M P Haynes 18

19. 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 Prim Haynes & Franjoine

20. 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 Prim Haynes & Franjoine

21. 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) Prim Haynes & Franjoine

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

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

24. Pictures Prim Haynes & Franjoine

25. 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) Prim Haynes & Franjoine

26. Pictures Prim Haynes & Franjoine

27. 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 Prim Haynes & Franjoine

28. Pictures Prim Haynes & Franjoine

29. 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 Prim Haynes & Franjoine

30. Pictures Prim Haynes & Franjoine

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

32. Pictures Prim Haynes & Franjoine

33. 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 Prim Haynes & Franjoine

34. Pictures Prim Haynes & Franjoine

35. NDT Enablement Classification Model of Health and Disability + Domains - Dimensions From: NDT Approach Theoretical Foundations & Principles of Clinical Practice, Table 2.1 pg. 82 2009 Prim Haynes & Franjoine M R Franjoine & M P Haynes 35

36. Activities & Activities Limitation Prim Haynes & Franjoine

37. NDT Enablement Classification Model of Health and Disability + Domains - Dimensions From: NDT Approach Theoretical Foundations & Principles of Clinical Practice, Table 2.1 pg. 82 2009 Prim Haynes & Franjoine M R Franjoine & M P Haynes 37

38. 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 Prim Haynes & Franjoine

39. 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 Prim Haynes & Franjoine

40. Treatment Comments • Gravitationally insecure • Does not enjoy movement • Stabilizes with eyes so remember this when treat in front of a mirror • Patience important Prim Haynes & Franjoine

41. Video Prim Haynes & Franjoine

42. Children with Ataxia Prim Haynes & Franjoine