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Activity in Cerebral Palsy: How it helps muscles (& brains!)

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Presentation on theme: "Activity in Cerebral Palsy: How it helps muscles (& brains!)"— Presentation transcript:

1 Activity in Cerebral Palsy: How it helps muscles (& brains!)
Diane L. Damiano, PhD PT National Institutes of Health Bethesda MD USA

2 TAKE HOME MESSAGE Activity, Activity…….

3 Activity and Cerebral Palsy
3/22/2017 Activity and Cerebral Palsy Those with CP have one of the most sedentary lifestyles among pediatric disabilities (Longmuir & Bar-Or 2000) Van den Berg-Emons et al (1995) estimated that ‘average’ child with CP would need to exercise 2.5 hours/day to reach activity levels of peers CP Planning meeting Boston

4 Step Counts in CP by GMFCS LEVEL vs. Peers (Bjornson et al 2007)
3/22/2017 Step Counts in CP by GMFCS LEVEL vs. Peers (Bjornson et al 2007) CP Planning meeting Boston

5 Outline Discuss generalized effects of activity on muscle structure & function and motor outcomes (optimizing physical rehabilitation) Neurobiology of activity: potential role of activity-based protocols for promoting neural recovery and restoration of function

6 3/22/2017 Muscles now known to be one of the most ‘plastic’ tissues in the body “Muscles respond in a fairly stereotypical manner to the amount and type of activity imposed upon them” Lieber et al, 2004 CP Planning meeting Boston

7 Muscle Myths Previously thought that fiber types and # of fibers determined genetically and could not change (marathon runners & sprinters born, not made) Rehabilitation of those with CP and other CNS disorders failed to include muscle strengthening or other intense training paradigms because it would > spasticity.

8 How Do Muscles Adapt? (Harridge, Exp Physiol, Review 2007)
3/22/2017 How Do Muscles Adapt? (Harridge, Exp Physiol, Review 2007) Two basic mechanisms at the level of the muscle fiber (cell): Change in mm size Primarily by increase/decrease in fiber diameter Mediated by satellite cells that repair or grow muscles (or replace themselves) Change in size directly related to maximal force output (In extreme cases (elite bodybuilders) & perhaps normal development (Sjostrom, 1992) the number of fibers may increase) Change in protein isoform (MHC) composition affects maximal shortening velocity (faster if > Type II) CP Planning meeting Boston

9 How can muscle adaptations be indiced?
3/22/2017 How can muscle adaptations be indiced? Decrease mm size Immobilization Decrease activity level (contractile activity) Weightlessness Increase mm size: Placing loads on muscles, e.g. progressive resistance exercise (PRE) Change protein isoform (MHC) composition High or low frequency electrical stimulation or high intensity (speed) voluntary training Denervation CP Planning meeting Boston 9

10 3/22/2017 Muscle plasticity in adult & developing skeletal mm : changes in MHC composition induced by inactivity & fast-type activity in Type I fibers Schiaffino et al. Physiology 22: CP Planning meeting Boston

11 What happens to muscles in CP?
3/22/2017 From infancy on (& perhaps before) children w/ CP do not move as much as those w/o CP & move differently Muscles cells are not mature at birth; therefore in CP, muscles may fail to develop properly from outset If muscles are not used, they become progressively weaker– then it becomes even harder to move To what extent is this preventable or reversible? CP Planning meeting Boston

12 What CP Care Environment Does to Muscles
3/22/2017 Many treatments in CP weaken muscles: Muscle-tendon lengthenings: <force-generation capability (Delp & Zajac, 1992) Orthoses: can cause atrophy of calf mms Botulinum toxin: paralyzes one mm at a joint to allow > stretch & enhance opposite mm function ITB: depresses involuntary & voluntary muscle activity PT: casting, splinting, restrictive garments, prior emphasis on movement quality vs. quantity, ban on strengthening can limit muscle development CP Planning meeting Boston

13 Strength in CP vs. Non-CP: Dominant Side
3/22/2017 (Wiley & Damiano, DMCN 1998) CP Planning meeting Boston

14 3/22/2017 Non-Dominant Side CP Planning meeting Boston

15 Strength by GMFCS Level
3/22/2017 CP Planning meeting Boston

16 3/22/2017 Muscle Strengthening Multiple reviews in CP & other conditions showing that strength is predictably increased (Dodd, Tayl0r & Damiano 2002; Taylor, Dodd & Damiano 2006) Changes in gait speed & other aspects of functioning noted often but not consistently Depends on ‘dose’ and ‘duration’. Must be done properly & for sufficient time to achieve benefits Must be maintained across lifespan CP Planning meeting Boston




20 Muscle Fatigue in CP Fatigue is cited as main cause for decline or cessation in walking in CP (Bottos 2004) Cardio-respiratory endurance lower in CP No reports on voluntary muscle fatigue in CP We hypothesized that those with CP would be more fatigable than age-matched peers, and that endurance would worsen with level of involvement

21 Methods Subjects: 18 w/ CP; 15 controls (ages 10-23y)
Fatigue Protocol: Biodex isokinetic dynamometer Consecutive, maximal, (concentric) reciprocal knee extension/flexion reps 35 repetitions at 60 deg/s Instructions: “Push all the way up as hard and fast as possible; pull down….” Verbal encouragement each repetition

22 3/22/2017 Methods Computed slope of the decline in torque (normalized to peak torque) in the quadriceps & hamstrings mms 0.2 0.4 0.6 0.8 1 5 10 15 20 25 30 35 REPETITIONS NPTKE (Nm) CP Planning meeting Boston

23 Results for the Quadriceps
3/22/2017 Results for the Quadriceps 0.2 0.4 0.6 0.8 1 5 10 15 20 25 30 35 CP Repetitions Normalized Peak Torque Controls Previously we reported………… The Control group was more fatigable than the group with CP, averaged across both muscle groups CP Planning meeting Boston

24 Correlation of Slope & GMFCS
II III Slope KE (N-m) N=10 n=5 n=10 0.0 0.2 0.4 0.6 0.8 1.0 n=3 Spearman (r) = -0.50, p = .035

3/22/2017 RESULTS Group w/ CP had greater endurance in their quadriceps than controls; hamstrings not different in CP Stackhouse et al evaluated fatigue with electrically elicited contractions: found quadriceps (but not triceps surae) to be less fatigable in CP We further found that the less functional (and weaker) they were, the greater their endurance tended to be HOW DO YOU EXPLAIN THIS? CP Planning meeting Boston

26 FATIGUE PARADOX 3/22/2017 Stronger individuals may fatigue more rapidly (inconsistent) Muscles in CP have predominance of Type I fibers (Rose 2001) The subjective complaint of fatigue is likely due to weakness. Individuals with CP are working at higher % of maximum, so this makes them feel more tired during a similar task - same thing happens in elderly Loss of strength with age increases fatigue even more Suggests that the most effective long term strategy to avoid fatigue is to maintain/increase strength to lessen relative effort CP Planning meeting Boston

27 In Vivo Evidence of Muscle Plasticity

3/22/2017 TRADTIONAL PT CONTROL MOS. OF FESCYCLING (Sadowsky, McDonald, Damiano et al) CP Planning meeting Boston

29 Introduction to Muscle Architecture
3/22/2017 Introduction to Muscle Architecture Fascicle geometry Fascicle Length (FL) Fascicle Angle (FA) FL = MT / sin (FA) (Shortland et al, 2002) Muscle size (2D) Muscle thickness (MT) (3D) Cross-sectional area (CSA) (3D) Muscle volume (3D) Muscle length RF MA is a strong determinant of a muscles force-generating properties CP Planning meeting Boston

30 RECTUS FEMORIS 3D US Longitudinal  RF Axial 

31 Relationship of muscle size to strength in CP
Ohata et al (2004, 2006) suggested that muscle thickness could be used as a surrogate measure of strength in CP, especially for those who are too young, too cognitively impaired or lack sufficient motor control.

32 MUSCLE THICKNESS IN ADULTS WITH CP (Ohata et al, Phys Ther 2006)
3/22/2017 MUSCLE THICKNESS IN ADULTS WITH CP (Ohata et al, Phys Ther 2006) BY STANDING ABILITY BY GMFCS LEVEL CP Planning meeting Boston

33 Muscle Ultrasound (US)
GE VOLUSON730 E: linear (2D) & volume (3D) probes PARTICIPANTS:18 w/CP (12 ambulators), 20 Controls; 11 measured before & after intense summer sports camp METHODS: Muscles Rectus Femoris (RF) Vastus lateralis (VL) Position: Supine with hips & knees in extension Measurements RF: 50% of ASIS to Patella VL: 50% of GT to lateral femoral condyle

34 Relationship of Muscle Thickness to Peak Torque
Control CP 10 20 30 40 50 100 150 200 250 300 10 20 30 40 60 80 100 VL MT (mm) r = 0.85** r = 0.70** ISOMETRIC PEAK TORQUE (N.m) *p < 0.05 **p < 0.01

35 Rectus Femoris Cross-Sectional Area in CP by GMFCS Level and vs
Rectus Femoris Cross-Sectional Area in CP by GMFCS Level and vs. Control 3/22/2017 GMFCS X Normalized Cross-Sectional Area : r = 0.50, p =.05 CP Planning meeting Boston

3/22/2017 RECTUS FEMORIS THICKNESS CONTROL (23kg) RFT=20.0 mm GMFCS II (21kg) RFT=13.3 mm GMFCS III (25.6kg) RFT=105 mm GMFCS IV (28.4kg) RFT=10.4 mm CP Planning meeting Boston

3/22/2017 Does intense and prolonged physical activity > mm size in CP? (new evidence suggesting this is possible in as few as 3 weeks) CP Planning meeting Boston

3/22/2017 Over the past 40 years, considerable data have been accumulated on the beneficial physiological effects from physical activity We are now becoming aware what activity does for the brain (e.g. it decreases depression & slows cognitive decline in Alzheimer's) CP Planning meeting Boston 38

39 3/22/2017 PROMOTING ACTIVITY Activity should be done ‘early and often’; parents can have the largest effect on this in infancy In addition to physical changes, personality, cognitive & social development may also be affected by early activity (or lack thereof) CP Planning meeting Boston 39

40 Activty-Based Exercise Programs
Catch-22: Those with CP need intense exercise to improve motor function, but they lack the motor function to exercise intensely. Therapeutic approach: Use of devices that force or enable person to exercise beyond their voluntary capabilities Body-weight supported treadmill training Lokomat and other motor driven gait devices FES and motor-assisted cycles

41 RANDOMIZED TRIAL OF TREADMILL TRAINING IN INFANTS WITH DOWN SYNDROME (Ulrich DA, Ulrich BD, Angulo-Kinzler RM, Yun J 2001) 3/22/2017 Description: 30 infants with DS assigned to control or home treadmill training beginning at independent sitting. Followed until onset of independent walking. CP Planning meeting Boston

42 RESULTS 3/22/2017 CP Planning meeting Boston

43 Review of BWSTT in Pediatric Rehabilitation (Damiano & DeJong, 2008 in press)
Shown to be efficacious (RCT) in Down Syndrome to accelerate motor milestone acquisition; more intense training seems to increase activity levels at 2 years Pediatric SCI – prolonged training in a few individual cases with impressive anecdotal results in most (children can be taught to step even if they cannot move voluntarily) CNS impairments: 17 studies (no RCT) suggesting that this improves gait speed and GMFM D&E. No comparison to alternatives (e.g. over ground training)


45 Potential Benefits of Treadmill Training in CP
3/22/2017 Strengthen anti-gravity muscles (by adjusting BWS or adding weights) Increase gait speed (> belt speed) Improve gait symmetry (e.g. elongating shorter strides) Improve interlimb coordination (through appropriate sensory inputs + practice) Increase endurance aerobic training) Combinations of above CP Planning meeting Boston 45

46 Motor-Assisted Cycling
BWS treadmill training labor & cost intensive, difficult for therapist/ family External assistance needed for those who cannot cycle on their own due to paresis or lack of motor control (FES-cycles or new motor assist devices) Cycling can be performed in home with little or no assistance, trunk balance or WS Form of locomotion similar in phasing & frequency to walking (Ting, 2002) Evidence of shared neural circuitry & similar reflex modulation in walking & cycling (Brooke 1997)

47 Current Cycling Trial PARTICIPANTS: 10 children w/ CP, ages 5-17, GMFCS III/IV PROTOCOL: All perform 50RPM passive or active-assisted cycling 30 min/day for 5 days/week X 3 mos GOAL: improve lower extremity coordination PRIMARY OUTCOMES: Changes in ‘comfortable’ & ‘as fast as possible’ cadence, variability in cadence, EMG reciprocation vs. synchronization SECONDARY OUTCOMES: 1) changes in spasticity; 2) Changes in cortical activation in response to a sensory stimulation using fMRI – none able to be still enough

48 Case study from motor-assisted cycling study
3/22/2017 Case study from motor-assisted cycling study 5 ½ yo boy with spastic diplegia GMFCS III – ambulates w/ post walker Ashworth 3 (0-4) in quadriceps & hamstrings (strong catch in first half of motion) Had adapted cycle, but needed assist from parents to ride He was able to cycle with the device part of the time (no resistance) CP Planning meeting Boston 48

49 Cortical Plasticity The brain is also use-dependent
3/22/2017 Cortical Plasticity The brain is also use-dependent Dramatic changes in the PNS produce dramatic changes in brain (e.g. SCI; amputation) Spinal circuits can be accessed and trained – effects may be specific and localized Spinal circuits may be used to drive cortical changes that may be more generalized How do we help the brain recover? CP Planning meeting Boston 49

50 What type of activity does brain like?
3/22/2017 Intense (amount, speed, mm activation) Some imposed rhythm but variable (more neural control) Complex or interesting; solving problems Electrical stimulation (other sensory stimulation) Locomotor training (loading/ proprioceptive input) CP Planning meeting Boston 50

51 Conclusions 3/22/2017 Those with motor disorders need to be active their whole life to minimize negative plasticity in mm (e.g. atrophy) & optimize positive plasticity (e.g. >fiber size) Increasingly obvious that we have been under-rehabilitating people with CP & other motor disorders Potential for exercise and activity to restore neural function and connectivity just beginning to be realized, Muscle activation (electrical activity) appears necessary to drive cortical plasticity CP Planning meeting Boston


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