MUSCLE STRENGTH IN CEREBRAL PALSY

Diane L. Damiano, PhD PT Dept. of Neurology/ Washington University St. Louis, Missouri USA

Outline
PART I: Scientific evidence for strengthening muscles in CP PART II: Augmenting strength through voluntary training and electrical stimulation
PART III: Effect of strength training on force production, motor function and participation

PART I: Scientific evidence for strengthening muscles in CP

NEVER STRENGTHEN SPASTICITY

HISTORY OF WEAKNESS IN CP

Recognized for decades

Cerebral palsy = weakness from the brain diplegia, hemiplegia, quadriplegia = location & no. of extremities that are weak

Strength & endurance training was an integral part of early treatment for CP (Phelps, 1950s; Berg 1970s)

WHY WAS STRENGTHENING ABANDONED?

Response not the same as in those with polio

Strengthening would increase spasticity and tightness in persons who were already stiff Individuals with CP cannot isolate muscles to do strengthening Problem is in the brain, not the muscles

CLINICAL OBSERVATIONS

Like other children who are weak, those with CP deteriorate in function during growth
Physical educators & athletes with CP strengthened for years with positive results Strengthening post-SDR had positive effects

Research Evidence

What do we know?
1. Children with CP are weak

Dominant Side
8 7 6 5 4 3 2 1 0
HFL HFS HE ABD ADD KF KE KE30 APFE APFF ADFF ADFF

Comparison Hemiplegia Diplegia

Non-Dominant Side
8 7 6 5 4 3 2 1 0
HFL HFS HE ABD ADD KF KE KE30 APFE APFF ADFF ADFF

Comparison Hemiplegia Diplegia

What do we know?
1. Children with CP are weak

2. Strength in CP is directly related to function

CORRELATIONS
MEAN STRENGTH
Velocity Stride Length Cadence GMFM Total EEI Double Support %

r
0.71 0.56 0.63 0.59 -0.54 -0.52

p
0.02 0.09 0.04 0.05 0.09 0.10

Strength by Ambulatory Level

5 4.5 4 3.5 3 2.5 2 1.5 1 0.5 0

hamstrings Quads 30

Level 1

Level 2

Level 3

CHILDREN WHO CANNOT WALK ARE BY DEFINITION WEAK

What do we know?
1. Children with CP are weak 2. Strength in CP is directly related to function

3. Children with CP can get stronger

4. Rate of strength increases similar to that for weakness not due to CNS lesions

QUADRICEPS STRENGTHENING Pre VS Post

5 4.5 4 3.5 3 2.5 2 1.5 1 0.5 0 PRE POST

48% 85%

FORCE/BW

140%

90

60

30

KNEE ANGLE

What do we know?
1. Children with CP are weak

2. Strength in CP is directly related to function

3. Children with CP can get stronger 4. Rate of strength increases similar to that for weakness not due to CNS lesions

5. Strengthening imparts direct functional benefits

Functional Changes after Strengthening

180 160 140 120 100 80 60 40 20 0 Velocity Cadence Fast Vel Fast Cadence %DS Fast GMFM 5 PRE POST

McLaughlin et al, 1998: RCT of SDR + PT vs. PT alone

RESULT: significant, equivalent gains across groups

The evidence suggestsin the Seattle studythe PT alone group underwent a regimen that included a stronger than customary emphasis on muscle strengthening
J.P. Lin, DMCN, 1998

What do we know?
1. Children with CP are weak

2. Strength in CP is directly related to function

3. Children with CP can get stronger 4. Rate of strength increases similar to that for weakness not due to CNS lesions 5. Strengthening imparts direct functional benefits

6. Strengthening does NOT increase spasticity!

STATE OF THE SCIENCE

Multiple studies in CP showing effectiveness of strengthening UE & LE using weights and isokinetics
Multiple studies also show effectiveness of strengthening in stroke and head injury after plateau in rehab

Recent Studies

Andersson et al, 2003 Dev Med Child Neurol

10 adults with CP (23-44yrs) Progressive strength training to improve walking 2X/week for 10 weeks Strength, gait speed, GMFM D & E, TUG improved No increase in spasticity

Blundell et al, 2003; Clin Rehab

8 children with CP (4-8yrs) Circuit training 2X/week after school for 4 weeks Strength > 47%; Lateral Step Test, sit to stand height, gait speed and stride improved

Where does the science of strengthening need to go now?

 Need

better understanding of why persons with spasticity are weak

Primary inability of agonist to produce force Constraint by antagonist (spasticity, cocontraction, muscle imbalance) Muscle may be at sub-optimal length to produce force due to contractures Reduced activity level Changes in muscle properties (stiffness, atrophy) Patient may have poor selective control of muscle

For higher functioning children with CP, cocontraction and spasticity have a minimal effect on force production: in children with CP who had 50% less quad strength, cocontraction magnitude accounted for <10% (Ikeda et al, 1998) no relationship between spasticity and strength in spastic diplegia (Engsberg et al, 2000)
These relationships may be very different in children with greater neurological involvement!

TYPES OF CONTRACTIONS

ISOMETRIC: muscle contracts while entire M-T unit does not change length CONCENTRIC: M-T unit shortens while muscles contracting ECCENTRIC: M-T unit is lengthening; sarcomeres are stretched while trying to contract and generates a greater amount of tension

Things we do that weaken muscles

Botulinum toxin injections Intrathecal baclofen Lengthen tendons Braces Not providing opportunities or equipment to promote mobility

 Is

there a window of opportunity when strengthening would be more effective?

Spasticity reduction (BTX A & B, SDR, ITB) & MT Surgery all > length & < antagonist restraint, and should make strengthening more effective Strengthening may augment and prolong effects of these interventions

STRENGTHENING TO MAINTAIN MUSCLE LENGTH STRATEGIES: a. strengthen agonist b. strengthen spastic antagonist (Shortland,2002)

MUSCLE MODELING DATA

Muscles change their action (moment arm) depending on the position of a joint (Delp et al, 1999)

Hypothesis

Lengthening hamstrings, adductors may not be optimal for crouched gait Unlikely to correct rotational component May cause > pelvic tilt, >hip flexor tightness, knee hyperextension May not correct pattern if weak ALTERNATIVE: strengthen hip and knee extensors particularly at end range

Where does electrical stimulation fit into the strengthening picture?

PART II: Augmenting strength through voluntary activation and electrical stimulation

GENERAL PRINCIPLES OF STRENGTH TESTING AND TRAINING IN CP

DEFINITIONS

STRENGTH: Maximum isometric force produced in a single contraction of unrestricted duration Single value that is the cumulative result of multiple factors from the level of the sarcomere to the arrangement of muscles around a joint MUSCLE IMBALANCE = distortion in the physiological relationship of muscle forces across a joint

STRENGTH TESTING
Functional

tests & MMT can be used to estimate strength

Can be fooled by functional tests (substitutions) can have 40% loss and still get NORMAL grade difficult to gauge in children of different age/size

For

determining loads or measuring change more quantification necessary!

Improving Reliability of Strength Testing

Standardize test positions & keep the same across sessions Standardize stabilization Make sure they understand the task Use a make vs. break test Collect 3 trials, take mean of highest 2 Motivate patient to exert maximum

Strength Training Programs

LOAD Frequency Intensity Structure Tempo Duration/Maintenance

STRENGTH TRAINING = method of conditioning using resistance to increase muscular strength by various methods (a.k.a. resistance or weight training) WEIGHT LIFTING = ballistic, explosive maneuvers involving a weighted barbell which is lifted from the ground overhead
POWER LIFTING = non-ballistic maneuvers involving weighted barbell (bench press, squat lift, deadlift)

LOAD

Essential element !! Must be intense ( 80-90 % MVC) Dose response relationship Typically use low # of repetitions; muscle must be allowed to rest & recover

(Law of diminishing returns or More is not necessarily better can overtrain!)

Determining Load

One Repetition Maximum contraindicated in children!!!!

Suggested formula for isotonic program: (isometric maximum X 2) X %LOAD = optimal weight

# of reps until performance deteriorates is a good estimate of optimal load

NOTE: Form/control should always be emphasized over load Manual and isokinetic resistance encourage maximum loads

TYPES OF RESISTANCE

ISOTONIC: fixed load that stresses parts of range differently (free weights, machines)
ELASTIC: Increases with length as band is stretched ISOKINETIC: constant velocity with accommodating resistance throughout range (Cybex, Kincom, Biodex)

FORCE

isotonic
isokinetic

isometric
Elastic Bands JOINT ANGLE

KNEE CURVE IN CP VS NORMAL

NORMAL TORQUE

CP

30

60

90

JOINT ANGLE

What is optimal program?

ACSM states that no one optimal protocol exists, but a general recommendation for children and adolescents is:

1-3 sets of 6-15 reps performed 23X/week on non-consecutive days

General Guidelines
Goal Reps & LOAD Sets
8-20/5+ 60-80% 1-3/10+ 90-100%*

TEMPO
Slow w/ control & rest Moderate & sustained Fast; with rest

Strengthening 3-8/3-5 80-100% Endurance Power

* can b ead justed in

Other Aspects of Program

Frequency
Recommended 2-3X week

Intensity
Depends on person & goals

Can overdo

Duration/Maintenance use or lose

Special Considerations in Spasticity

Not all that special same principles apply! May need to test and train in synergies if isolated control is poor May need to start more slowly in less active or more fragile persons

Which muscles should be strengthened?

1 lengthen spastic agonist; strengthen antagonist,e.g. hip ext, quads, ankle DFs 2 concentrate on power producers for task, e.g. hip flexors and ankle PFs for gait 3 consider absolute and relative strength across joint e.g. hip flex/ext; ham/quad ratios

Other clinical/ research questions:

Is strength training more effective if done in functional positions?

Must be sure target mms are sufficiently loaded Large joint impact forces a concern in CP

How can the positive effects of strengthening best be maintained?

is it safe for children and adults of all ages to lift weights?

Is weight training SAFE?

Is safer than most other forms of exercise & may decrease sport injuries by strengthening ligaments, tendons, bones
Can be harmful if done improperly children must be supervised by an adult & exercise specialist should be involved in program design NO evidence (in 1109 children who trained at national level) of serious injury or growth plate damage from weight training

WAYS TO STRENGTHEN

What type of strength training program is best?

ANSWER: It depends on goal, e.g.

Weakness at end ranges (ISOTONIC, ELASTIC) Weakness same throughout range (ISOKINETIC) Fast movements most difficult (ISOKINETIC,ES) Eccentric weakness (ISOKINETIC) Cannot activate mm to strengthen (ES) Minimize joint stress (AQUATICS)

ELECTRICAL STIMULATION

TREATMENT GOALS FOR ES

(in addition to strengthening) Inhibit Spasticity Stimulate agonist; RI to spastic antagonist (e.g. before PT) Stimulate spastic mm (? Fatigue or autogenic inhibition) Increase Range of Motion Must produce Fair grade contraction; can stimulate in alternating motions or contract-relax for agonist Slow ramp (< clonus or stretch response) and multiple repetitions

Motor Control/Learning Sensory level ES may provide visual & proprioceptive feedback during simulated task Contraction level for kinesthetic feedback; EMG-assist devices to augment mvmt triggered voluntarily

Evidence for TES in CP

Pape (JPO 1993) & Steinbok (DCMN 1997)

reported improved gait & motor function

No evidence of > strength or muscle growth Dali et al (DMCN 2002) no improvement in DB RCT in 57 children in CP from Sommerfelt et al (DCMN 2001). 12 children with CP had no change from TES Hazlewood (DMCN) said TES effective, but was not TES (NMES to 10 children at low levels of contraction). Only ROM increased

Evidence for NMES in CP

Comeaux et. al. (Ped PT; 1997) NMES on 14 children w/ CP improved ankle motion and heelstrike in gait Wright & Granat (DMCN, 2000) 12 children with CP w/ wrist extensor ES + exercise had > hand function Carmick : 4 case studies recommending electric stimulation for children with CP (1993,1993,1995,1997)
Generated >interest in using NMES Dramatic increases in function that persisted No statistics; no consistent protocol Mechanism unclear but does NOT appear to be strengthening (sub-threshold) Must use caution and judgment when generalizing

Electrical Stimulation Parameters Waveform

Bursted (2-10 khz frequency) Pulsed fine for small mms, but cause discomfort in large mms at higher intensities & rates Biphasic - prevents skin lesions Monophasic Symmetric Asymmetric more comfortable for large mms

Electrical Stimulation Parameters

Frequency

Range = 30-50 pps; achieve balance between tetanic contraction (twitches need to summate) & fatigue Lower frequencies used in TES (for type I fibers)

Pulse Width

Range typically between 50-1000us (less for portable devices) Less is reportedly more comfortable but get less stimulation if frequency held constant

Electrical Stimulation Parameters

Electrode size Depends on mm size - stimulate whole mm but avoid spread to other mm >comfort (bigger electrode diffuses current (+); goes deeper (-) ON/OFF Cycle Adjusted to minimize fatigue; generally 1:1 1:5 Muscle fatigue and overwork weakness a concern Ramping Slower is more comfortable, less clonus, better control May be shortened or eliminated during FES

ON

OF F

VOLUNTARY vs. ELECTRICAL ACTIVATION

VOLUNTARY
Recruitment Asynchronous Type I first Close to MVC Must be present Can target mm groups often requires clinic/gym

ELECTRICAL
Synchronous Type II first Low % MVC effective Not needed Can target specific mms Can be done at home

Intensity Isolated Control

Setting

Russian Stimulation proposed by Kots, 1974

Supra-maximal intensity stimulation using bursted current rather than pulsed to minimize discomfort Claimed strength gains exceeded those that can be achieved voluntarily & lead to increased interest in electrical stimulation Protocols for enhancing performance in athletes have filtered down in to rehabilitation

Claims unconfirmed by follow-up studies although replication difficult because of equipment differences and inadequate description

TES (Threshold Electrical Stimulation)

low level, barely perceptible sensation, no muscle contraction night time use (6X/week) to grow muscle and prevent atrophy 2 recent studies have not upheld previous claims of functional gains

FES (Functional Electrical Stimulation)

Muscle made to contract during functional task, e.g. Restoration of function in SCI Can augment gait events or other motor tasks in CP Surface or implanted

NMES (Neuromuscular Electrical Stimulation)

NMES = electrical stimulation of skeletal mms through motor nerves (preferably at the motor point) to assist in the treatment of postural or movement disorders Multiple protocols depending on goal of treatment Used counter-intuitively in CP at sub-threshold level during tasks to obtain carry over in the absence of stimulation (Carmick)

INTENSITY OF STIMULATION

Intensity
Dose-response clearly shown in literature
Weaker muscles need less current

High intensities needed for stronger, larger mm BUT pulsed current hard to tolerate
Alter parameters (use asymmetric waveform, slower rampup & <frequency) Gradual build up of tolerance Allow movement during activation Change current to BURSTED Implant electrodes (risks of surgery, infection, discomfort)

HOW INTENSE IS IT?

QUANTIFYING INTENSITY
DIAL SETTING NOT ACCURATE ENOUGH:Despite same setting, intensity will change with electrode placement & wear Amount of movement can be a simple gauge

Patient must be instructed to relax and not to resist stimulation

Need at least F+ for PROM; probably more for strengthening Measure MVC (if possible)

CAN BE EASILY QUANTIFIED WITH DYNAMOMETER!

Measure Peak Force during stimulation

Can compare MVC to norms Determine % MVC stimulation produces

SUMMARY

Potential for surface ES to increase strength is limited by patient tolerability in CP (only small and/or weak mms likely to benefit)
Implanted electrodes, better machines, combining with weight training may increase usefulness in CP Do not say you are strengthening unless you are certain that you are! Choice of strengthening method should be based first on effectiveness & short/ long term safety with ease, affordability, availability, time and enjoyment other important considerations

PART III: Effect of strength training on force production, motor function and participation

ISOTONIC EXAMPLE - TIM

10 yo w/ spastic diplegia

SDR - age 6;
no OS; consistent PT Ambulates w/ posterior walker & RAFOs WC for long excursions FAMILY GOAL: improve walking ability and ease

Pre-Training Strength Values

Muscles Hip Flexors Hip Extensors Hip Abductors Hip Adductors Quadriceps at 90 Quadriceps at 30 Hamstrings Gastrocsoleus Tibialis Anterior Right 21 85 32 64 11 2 29 29 23 Left 30 103 32 69 32 10 35 27 28

TRAINING PROGRAM
LOAD

Free ankle weight 80-90% max Progressive resistance (every 2 weeks) 3X/wk for 6 weeks 4 sets of 5 reps per mm per session Slow in both directions (concentric and eccentric)

FREQUENCY & INTENSITY

TEMPO

Hamstrings and hip flexor stretches; 5 minute walk as warm-up Strength training to hip flexors and knee extensors bilaterally:

Hip flexors performed in supported high marching Quadriceps done in sitting with encouragement to bring to end of range, held for 5 seconds, then lowered slowly

Muscles

Right

Left

CHANGE R / L

Hip Flexors Hip Extensors Hip Abductors Hip Adductors Quadriceps at 90 Quadriceps at 30 Hamstrings Gastrocsoleus Tibialis Anterior

21 85 32 64 11 2 29 29 23

30 103 32 69 32 10 35 27 28

55 / 58

39 / 60 25 / 50

Gait Parameters
Velocity Stride Length Cadence % Double Support Pelvic Rotation Hip Flex/Ext Knee Flex/Ext Ankle DF/PF

PRE 0.23 0.57 46 52 26 48 57 25 61

POST 0.31 0.66 55 39 14 57 65 26 69

GMFM TOTAL

Follow-up

Add hip abductor strengthening Treadmill training or stairmaster for endurance training & generalizability Has begun UE strengthening Explore recreational opportunities so he can utilize strength gains

ISOKINETIC RESULTS Quadriceps

0.12 0.1 0.08 0.06 0.04 0.02 0 Q30 Q60 Q120 QECC PRE POST

ELECTRICAL STIMULATION CASE JAY

Age: 24; DX: R hemi Functional mobility: Independent PROM ADF: L 12 R 10 AROM ADF: L 10 R -10 Current complaints: Tripping and falling (at least 2X / week)

Intervention: ES to R tibialis anterior Parameters: 40 pps; 10/7 on/off cycle; 30 min BID; intensity to tolerance; 12 weeks Outcomes: PROM: L 13 R 10 AROM: L 10 R 4 No falls for 4 weeks

ES Pilot Study Amanda

17yo girl with R hemiplegia (GMFM=96.5) R toe strike & stiff knee; reduced active & passive ROM in KE and ADF GOAL: normalize appearance and gait Insurance refused to pay for ES unit, so she wanted to participate in pilot study

PROGAM : ES to R TA & quadriceps 30 minutes 2X/day to tolerance X 12 wks Ultraflex brace used during stimulation No voluntary activity Measured isokinetic strength, ROM, 3-D Gait, Active ROM, GMFM, PODCI (health status questionnaire)

Results

Strength no appreciable change Gait: cadence > by 7 steps/min GMFM NC; Health status measure showed expectations not met, < happiness ROM:
Passive & active > at ankle; knee had NC DF >7 in stance; > 4 in swing; Mean KF > by 15 in stance

WEIGHT TRAINING + STIMULATION David

Age: 16 Functional Mobility: Loftstrand crutches using a swing through pattern w/ WC for long distances PSH: Hamstrings & tendoachilles lengthenings MMT Strength: Anterior Tibialis = Trace on R & L; Quadriceps = 2/5 on R & L PROM: ankle DF to 0; hamstrings and adductors tight Social: untrusting, uncooperative, and openly opposed to Physical Therapy

Therapy Program

High intensity electric stimulation to Quadriceps and Anterior Tibialis Parameters: 50 pps to tolerance: 1:5 on/off Strengthening exercises for Quadriceps (4 lbs) & Anterior Tibialis (3/4 lbs) Stretching: Hamstrings; adductors and ankles

Early Progressions

Increased weights weekly Increased amplitude of electric stimulation as much as tolerated Fell and injured Knee at 9 weeks and discontinued Electric Stimulation to the Quads Began functional training at 12 weeks Quads at 20lb and DF with R 11.5 & L 9lbs

Later Progressions

At 20 weeks, discontinued electric stimulation Began Treadmill Ambulation Functional training expanded to include upper body, trunk and balance Continued PRE on quads (44 lbs) and TA Met patient at school to design program for Weight room

Final Progressions

Now working out in weight training class 3X/week in addition to PT Manager of the High school football team and wearing letter jacket. At 24 weeks began attempting one loftstrand Bracing progressed from rigid AFO to PLS to shoe inserts during therapy Continued PRE At 51 weeks Patient using one cane

Can Strengthening Enhance Participation & Quality of Life?

AQUATIC STRENGTHENING
31yo

man w/ CP; hamstring & TA lengthening, VDRO 20 yr ago

Walks C/o

w/ lofstrands (pull to gait)

weakness & muscle pain in legs; lack of endurance and energy at end of day
(Thorpe & Reilly, 2000)

Program
10 weeks, 3Xweek for 45 minutes Stretching in water (15 minutes) Resistance exercises (20 min) for hip & knee flexors/extensors, ankle dorsiflexors Water walking (10 min) Hydro-tone boots/ water for resistance EEI, TUG, FRT, GMFM (D&E), gait velocity, ASPP, strength (HHD)

Results
457% in EEI (reported better endurance and energy) Could walk independently for 20 feet switched to reciprocal gait Gait velocity > by 3 m/min GMFM D from 49-77%; E from 58-86% Strength > 100% Self-perception increased

PedsQL RESULTS 2 WK SPORTS REHAB PROGRAM

8 yo male; GMFCS level IV Function Physical Emotional Social School PRE PARENT 62 50 45 60 POST PARENT 59 85 100 85 PRE CHILD 56 40 30 90 POST CHILD 76 60 90 90

PedsQL RESULTS 2 WK SPORTS REHAB PROGRAM

14 yo female; GMFCS level III Function Physical Emotional Social School PRE PARENT 84 60 60 45 POST PARENT 88 100 60 80 PRE CHILD 25 65 50 70 POST CHILD 91 100 80 80

FINAL CONCLUSIONS

Intense and regular physical activity important for everyone but especially those w/ mobility challenges
Strengthening is one aspect of conditioning to enhance physical functioning &

participation in those w/ CP