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Physical Activity in Neuromuscular Disease

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Presented at NIDRR Rehabilitation Research and Training ... n = 26 (MMD, HMSN-1, Limb Girdle Dystrophy, FSHD, BMD, SMA III. Community heart rate monitoring ... – PowerPoint PPT presentation

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Title: Physical Activity in Neuromuscular Disease


1
Physical Activity in Neuromuscular Disease
Craig M. McDonald, MD, Assoc. Professor Department
s of PMR and Pediatrics UC Davis School of
Medicine cmmcdonald_at_ucdavis.edu Director RRTC
in Neuromuscular Diseases, UCDMC Neuromuscular
Disease Clinic, and UCDMC Pediatric
Rehabilitation Program Presented at NIDRR
Rehabilitation Research and Training Center in
Neuromuscular Diseases Roundtable 2001 Role of
Physical Activity and Exercise Training in
Progressive Neuromuscular Diseases. Sept. 30,
Oct. 1-3, 2001, San Diego, CA.
2
Hereditary and Acquired Neuromuscular Diseases
  • Definition Peripheral disorders of the nervous
    system affecting anterior horn cells, peripheral
    nerves, neuromuscular junction, and muscle.
  • More than 300 neuromuscular diseases (NMD over
    250 distinct genes)
  • Prevalence of the most common NMD is
    approximately 400,000
  • Overall prevalence is greater than 4 million in
    the U.S.

3
Enhance Quality of Life in Persons with
Neuromuscular Disease
4
Quality of Life According to Consumers with NMD
  • Comprehensive Quality of Life Survey of target
    population (n 811) reported problems that
    reduce quality of life
  • Weakness
  • Difficulty getting exercise
  • Fatigue / poor endurance
  • Problems with weight
  • Respiratory / sleep problems
  • Pain

5
Limitations in Physical Activity in NMD from
SF-36 (n 811)
Recalculated from physical activity data from
the SF-36 survey conducted by Abresch et al., Am
J Hospice and Palliative Care 200219(1)39-48.
6
Physical Activity in NMD
  • Impact of reduced physical activity on health and
    fitness
  • Methodologic issues relating to assessment of
    physical activity
  • Determinants of physical activity (in DMD)

7
Goals of Physical Activity Exercise in NMD
  • Increased cardiopulmonary endurance
  • Increased lean mass, decreased fat mass
  • Increased muscle strength endurance
  • Improved flexibility
  • Improved health outcomes
  • Psychosocial benefits

8
Benefits of Physical Activity in the Able-Bodied
  • Prevention and control of
  • Coronary artery disease
  • Hypertension
  • NIDDM
  • Osteoporosis
  • Obesity
  • Mental health

9
Persons with NMD are Sedentary and Deconditioned
  • Lower work capacity / peak VO2
  • Reduced resting energy expenditure
  • Body composition changes
  • Decreased muscle strength and endurance

10
Age-Related Changes Observed in Sedentary Subjects
  • Greater
  • age-related decline in VO2 max
  • decrease in muscle mass (sarcopenia)
  • increase in body fat
  • decrease in strength
  • reduction in walking speed in those with weak
    knee extension

11
Reduced Exercise Performance in NMD Disuse or
Disease?
Reduced functional muscle mass
(Disease) Reduced physical activity Disuse
atrophy To the extent that reduced exercise
performance is due to the effects of detraining
from a sedentary existence, endurance exercise
may be helpful in reversing the negative effects
of the deconditioned state. (Neufer 1989)
12
Objective Assessment of Physical Activity
Current Methods
  • Diary/self-report
  • Doubly labeled water (total energy expenditure)
  • Standard pedometry
  • Accelerometers
  • Long-term heart rate monitoring (with HR-VO2
    calibration)

13
Goals for the Quantitative Assessment of
Physical Activity
  • When is a subject active/sedentary during a day ?
  • What is the magnitude of peak activity?
  • What is the average total daily physical activity
    over extended sampling time?
  • What is the mean proportion of time spent at
    defined physical activity intensities?
  • Unobtrusive monitoring in community.

14
Physical Activity Assessment Heart Rate
Monitoring
15
Physical Activity in Slowly Progressive NMD
(McCrory 1998)
  • n 26 (MMD, HMSN-1, Limb Girdle Dystrophy, FSHD,
    BMD, SMA III
  • Community heart rate monitoring
  • TEE REE ACTEE

16
Physical Activity in Slowly Progressive NMD
(McCrory 1998)
17
Physical Activity in Slowly Progressive NMD
(McCrory 1998)
  • NMD subjects reported spending less time
    exercising than controls
  • NMD exercise at light level, controls at
    moderate level
  • Body fat in NMD subjects inversely related to
    minutes active and fat-free mass

18
Step Activity Monitor (PRS, Seattle, WA)
19
Step Activity Monitor (PRS, Seattle, WA)
20
Accuracy of the SAM
21
Step Activity Monitor (PRS, Seattle, WA)
22
(No Transcript)
23
Total Daily Steps in Able-Bodied Control Children
24
Step Activity Able-Bodied Obese vs. Controls
(children ages 6-10)
25
Determinants of Physical Activity
  • Medical Rehabilitation Model (NCMRR)
  • Pathophysiology
  • Impairment
  • Functional limitation
  • Disability
  • Societal limitation

26
Natural History of Duchenne Muscular Dystrophy
27
DMD/BMD Gene
XP 21 locus 2.4 million base pairs Exons 1 -
79 Hotspots Exons 3 - 19
Exons 42 - 60
28
Pathophysiology
Defined Interruption of or interference with
normal physiological and developmental processes
or structures. Example Absence of dystrophin
leads to progressive loss of functional muscle
fibers in DMD.
29
Dystrophin Complex
30
Dystrophin
31
Pathophysiology of Duchenne Muscular
Dystrophy
Gene abnormality at xp 21 Absence of
dystrophin Susceptibility of sarcolemmal
membrane to mechanical injury Muscle fiber
injury and degeneration Cycles of degeneration
and regeneration Cell death (replacement by fat
connective tissue)
32
Muscular Dystrophy Muscle Biopsy Findings
  • Histology
  • Normal fibers
  • Hypertrophy of fibers
  • Degeneration of fibers
  • Atrophy of fibers
  • Regeneration of fibers
  • Connective tissue/fatty infiltration

33
Muscle Biopsy 3-yr-old boy, normal fiber
34
Muscle Biopsy 3-yr-old, DMD fiber
35
Muscle Biopsy 8-yr-old, DMD fiber
36
Muscle Biopsy 19-yr-old, DMD fiber, postmortem
37
Biceps Muscle Biopsy 19-yr-old, DMD fiber,
postmortem
38
Gastrocnemius Muscle 19-yr-old, DMD fiber,
postmortem
39
DEXA
40
DEXA Scan of 17-yr-old Male with DMD
  • Validity measure of body composition
  • Test-retest reliability 0.998
  • Applicable to severe NMD with myogenic atrophy
  • Scoliosis and contractures

41
Body Composition in DMD
42
Mean Lean Tissue Mass in DMD
43
Body Fat and Physical Activity in DMD
  • Correlation between body fat by DEXA and
    total steps per day by SAM -0.61.

44
Impairment
  • Defined Loss or abnormality of cognitive,
    emotional, physiological, or anatomical structure
    or function, including all losses or
    abnormalities, not just those attributable to the
    initial pathophysiology.
  • Example Decreased strength in DMD due to loss
    of muscle fiber, impaired contractility, and/or
    disuse weakness.

45
Quantitative Strength Testing in DMD
46
Quantitative Strength Testing in DMD
47
Strength per Lean Tissue Ratio in DMD
48
Impairment Predicts Functional Limitation
  • Correlation between knee extension strength and
    walking speed in DMD 0.70.
  • Walking speed predicts time to wheelchair
    (McDonald, 1995).

49
Functional Limitation Leads to Further
Impairment
  • Transition to sedentary existence in wheelchair
    leads to contractures in DMD.
  • Short periods of bed rest result in significant
    loss of strength and function in DMD.

50
Ankle Plantarflexion Contractures in DMD
51
Knee Contractures in DMD
52
Hip Flexion Contractures in DMD
53
Static Positioning Leads to Contractures in DMD
54
Elbow Flexion Contractures in DMD
55
Static Positioning Leads to Contractures in DMD
56
Impairments that Reduce Physical Activity in DMD
  • Weakness (due to dystrophinopathy and disuse)
  • Fatigue
  • Cardiopulmonary involvement
  • Contractures
  • Excessive weight gain

57
Weight Management in Neuromuscular Disease
  • Obesity (sedentary)
  • Cacchexia (end-stage)

58
Weight Gain in DMD
59
Functional Limitation
  • Defined Restriction or lack of ability to
    perform an action in the manner or within a range
    consistent with the purpose of an organ or organ
    system.
  • Example Decreased velocity of ambulation,
    decreased distance achieved per unit time,
    metabolically inefficient ambulation in DMD.

60
Velocity of Locomotion in DMD
61
Percent Individuals Who Walked Distance in Ten
Minutes
62
Energy Cost of Locomotion with COSMED K4b2
Validity Test-retest reliability
0.94 Functionally relevant measure of economy of
actual locomotion
63
Energy Expenditure During Wheelchair Propulsion
64
Economy of Movement Able-Bodied Obese vs.
Controls
65
O2 Cost During Walking and Sprinting
66
Walking O2 Cost vs. Velocity
67
Heart Rate in DMD During 100-Meter Sprint
68
Sprinting VO2 vs. Distance Traveled 100-meter
Sprint
69
VO2 per Gram Lean (DEXA)
70
Sprinting O2 Cost vs. Velocity
71
Disability
  • Defined Inability or limitation in performing
    tasks, activities, and roles to levels expected
    within physical and social contexts.
  • Example Inability to exercise, decreased daily
    physical activity in the community in DMD.

72
Step Activity Monitor (24 Hour) Able-Bodied
Control
73
Step Activity Monitor (24 Hour) Duchenne
Muscular Dystrophy
74
Step Activity in DMD vs. Controls
75
Quantitative Physical Activity in DMD
76
Societal Limitation
  • Defined Restriction, attributable to social
    policy or barriers (structural or attitudinal)
    which limits fulfillment of roles or denies
    access to services and opportunities that are
    associated with full participation in society.
  • Example Teenager in power wheelchair with DMD
    does not participate in PE adaptive PE not
    available or is poorly developed.

77
Future Needs Scientifically Based
Recommendations Concerning Optimal Exercise
Guidelines
  • Disease-specific recommendations relating to
    types of exercise that can be safely performed.

78
Future Needs Scientifically Based
Recommendations Concerning Optimal Exercise
Guidelines
  • Disease-specific recommendations relating to
    types of exercise that can be safely performed.
  • Recommendations regarding the minimum frequency,
    amount, and duration of exercise required for
    beneficial health effects.

79
Future Needs Scientifically Based
Recommendations Concerning Optimal Exercise
Guidelines
  • Disease-specific recommendations relating to
    types of exercise that can be safely performed.
  • Recommendations regarding the minimum frequency,
    amount, and duration of exercise required for
    beneficial health effects.
  • Development of novel approaches to enhance levels
    of physical activity in persons with varied
    severities of impairment due to NMD.
  • This work was supported by the National
    Institute on Disability and Rehabilitation
    Research Grant HB133B980008 and a grant from the
    National Institutes of Health RO1 HD35714.
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